Neuro Flashcards
cervical radiculopathy, basal skull fracture
How does MND present on nerve conduction studies?
Normal motor conduction
What are adverse side effects of phenytoin?
Acute: dizziness, visual changes, slurred speech, seizures
Chronic: gingival hyperplasia, drowsiness, megaloblastic anaemia, peripheral neuropathy, lymphadenopathy
Teratogenic: associated with cleft palate and congenital heart disease
How do the pattern of signs present in GBS?
Flaccid weakness with hyporeflexia
What is the management of autonomic dysreflexia?
Remove stimulus (distended bowel/bladder) and treat lift-threatening complications e.g. bradycardia
reduced GCS, paralysis and bilateral pin point pupils suggests what?
Pontine haemorrhage
Patient with new onset stroke?
Admit to hospital for urgent CT head -> ?stroke assessment
What should be considered when starting someone on phenytoin?
Cardiac monitoring due to arrhythmogenic effects
What is a common side effect of triptans?
Tightness of the chest and throat
clonic movements travelling proximally suggests what?
Jacksonian march - frontal lobe epilepsy
What is the most common complication of meningitis?
Sensorineural hearing loss
What is the management of headache linked to valsalva manoeuvres?
Raised ICP until proven otherwise so needs CT
Management of seizures?
Rectal diazepam
What is used to treat idiopathic intracranial HTN?
- Weight loss
- Acetazolamide
Ipsilateral oculomotor palsy and contralateral weakness of the upper and lower extremity
Posterior cerebral artery
What is the mode of action of ondansetron?
Selective 5-HT3 receptor antagonist which acts in the medulla oblongata
Subdural vs extradural?
Subdural will have fluctuating consciousness
patients with dangerous mechanism of injury, including falling more than 1 metre or from a height of 5 stairs or more require what?
CT head within 8 hours
criteria for CT head within 1 hour?
GCS < 13 on initial assessment
GCS < 15 at 2 hours post-injury
suspected open or depressed skull fracture
any sign of basal skull fracture (haemotympanum, ‘panda’ eyes, cerebrospinal fluid leakage from the ear or nose, Battle’s sign).
post-traumatic seizure.
focal neurological deficit.
more than 1 episode of vomiting
CSF findings for SAH?
- Normal or raised opening pressure
- Xanthochromia
criteria for CT head within 8 hours?
age 65 years or older
any history of bleeding or clotting disorders including anticogulants
dangerous mechanism of injury (a pedestrian or cyclist struck by a motor vehicle, an occupant ejected from a motor vehicle or a fall from a height of greater than 1 metre or 5 stairs)
more than 30 minutes’ retrograde amnesia of events immediately before the head injury
Idiopathic vs drug induced Parkinsons
Idiopathic - asymmetrical symptoms
Drug induced - symmetrical
Seizures vs syncopal episodes
Syncopal episodes - rapid recovery and short post ictal period
Sudden onset vertigo and vomiting, facial paralysis and sensorineural deafness - which artery
Anterior inferior cerebellar artery
Progressive supranuclear palsy vs multiple system atrophy?
PSP will have an upward gaze impairment
What is a patient with alcoholism at risk of?
Subdural haematoma
pain, ophthalmoplegia, proptosis, trigeminal nerve lesion and Horner’s?
Cavernous sinus syndrome
Cerebral Herniation can cause what?
Third nerve palsy: eye down and out with dilated pupil
What are the signs of lateral medullar syndrome/PICA stroke?
- Ipsilateral facial pain and loss of temperature
- Contralateral loss of limb and torso pain/temperature
- Ipsilateral Horners syndrome
Which anticoagulant is used for prophylaxis post TIA?
Lifelong clopidogrel
What visual features will be present in a patient with IIH?
- Blurred vision
- Papilledema
- Enlarged blind spot
What imaging is used to diagnose carotid artery stenosis?
Duplex US
What drug should be avoided in patients with myasthenia gravis?
Bisoprolol
Ventriculomegaly without sulcal enlargement suggests what?
Normal pressure hydrocephalus
What is the response that can occur following raised ICP?
Cushings reflex - hypertension and bradycardia with wide pulse pressure
Which opiate should be used if standard neuropathic medications are not working?
Tramadol
Which Parkinsons medication is linked with impulse control disorders?
Dopamine agonists
Which anaesthetic agent would someone with MG be resistant to?
NMBDs e.g suxamethonium
reduced GCS, paralysis and bilateral pin point pupils suggests what?
Pontine haemorrhage
Fluent speech, comprehension intact but poor repetition?
Conduction dysphasia
Gait ataxia is caused by what?
Cerebellar vermis lesions
Which drugs are associated with IIH?
A - Vit A and metabolites
L - Lithium/Levothyroxine
O - OCP
S - Steroids
T - Tetracycline
C - Cimetidine
What is a very common early symptom of MS?
Lethargy
What is associated with autonomic dysreflexia?
Stroke
Which cranial nerves are affected in acoustic neuromas?
5,7,8
What is the sensory loss in syringomyelia?
Spinothalamic - pain and temperature
How long can cluster headaches last?
15 mins - 2 hours
Muscle wasting of hands, numbness and tinging with autonomic symptoms suggests what?
Thoracic outlet syndrome
Which anti-epileptic drug can cause weight gain?
Sodium valproate
What is paroxysmal hemicrania?
- Unilateral headache in the orbital/temporal region
- Similar to cluster headaches
- Responsive to indomethacin
Which anti epileptic medication can cause numbness of fingers and feet?
Phenytoin
Empty delta sign on venography suggests what?
Sagittal sinus thrombosis
Vision worse on going down the stairs?
4th nerve palsy
What can be done in patients with raised ICP?
Hyperventilation to reduce blood CO2 to induce cerebral vasoconstriction
What can be used to differentiate between a seizures and a pseudo seizure?
Prolactin
What is the eye examination signs for 3rd nerve palsy?
Ptosis + dilated pupil + absent light reflex with intact consensual constriction
Where is the most common cause of obstructive hydrocephalus?
Cerebral aqueduct stenosis
Which anti-epileptic drug causes weight loss, renal stones and cognitive and behaviour changes?
Topiramate
sudden and severe back pain, followed immediately by rapidly progressive bilateral flaccid limb weakness and loss of sensation, particularly for pain and temperature?
Spinal cord infarction
What is the management of stroke in patients <60 with severe symptoms and atleast 50% infarct of MCA on CT?
Decompressive hemicraniectomy
What is the most common cause of third nerve palsy?
Diabetes
What causes painful third nerve palsy?
Posterior communicating artery aneurysm
What is the gold standard investigation for venous sinus thrombosis?
MR venogram
What is the triad for lewy-body dementia?
fluctuating cognition, parkinsonism and visual hallucinations
unilateral wide-amplitude flinging movements, usually of the proximal limb post stroke?
Hemiballismus secondary to lesion in the contralateral sub thalamic nucleus
Causes of SAH?
PKD, Ehlers Danlos, aortic cortication
Other symptoms of SAH except headache?
Vomiting, collapse, coma, visual disturbance, focal neurology
What is Kernigs sign?
- Demonstrates meningeal irritation
- Hip and Knee bent to 90 degrees, pain causes when knee is straightened
Extradural vs subdural
Extradural - lens shaped/lemon/biconvex, subdural - crescent shaped
What is Todds palsy?
T temporary weakness following a seizure - usually of affected limb(s).
What are causes of seizures?
Uraemia, hypoglycaemia, hyponatraemia, hypernatraemia, hypocalcaemia
What airway adjunct should be used if someone is having a seizure?
Nasopharyngeal
Management of seizure?
- Roll the patient into the recovery position and move any items away from him that could cause harm
- Place a pillow under head
What is the management of bladder dysfunction/incontinence in someone with MS?
US KUB
Sudden onset vertigo and vomiting, ipsilateral facial paralysis and deafness
Anterior inferior cerebellar artery
What is Freidreich’s ataxia?
Autosomal recessive neuro condition with weakness, cerebellar signs and scoliosis
What signs would be present to indicate GBS?
LMN signs: hypotonia, paralysis, no reflexes
What is the most common cause of surgical third nerve palsy?
Posterior communication artery aneurysm
Damage to which structures can cause a homonymous hemianopia?
Unilateral damage to optic radiation or visual cortex
What are symptoms of raised ICP?
- Headache
- Vomiting
- Papilledema
- Seizures
- Reduced consciousness
Drugs to manage ICP?
- Mannitol
- Dexamethasone
What are causes of Horners syndrome?
- Pancoast tumour: non-small cell carcinoma
- Stroke
- Carotid artery dissection
Atrophy of the caudate nucleus and putamen?
Huntingtons
Dementia + Depression/Irritability + Involuntary movements?
Huntingtons
Acute cord compression in someone on anticoagulation should make you think what?
Epidural haematoma
What can present very similarly to carpal tunnel syndrome?
degenerative cervical myelopathy?
What are C/I to thrombolysis?
- Haemorrhagic stroke
- Active bleedig
- Major surgery within 14 days
- Pregnancy
- Previous intracranial haemorrhage
Where is the damage in spastic cerebral palsy?
UMN in the periventricular white matter
What is the management of myasthenia crisis?
IV Immuniglobulins / Plasmapheresis
What is the most common cause of viral meningitis?
Enteroviruses e.g. coxsackie B
Vision worse going down stairs?
CN4 - Trochlear
What is a common complication of SAH?
Hyponatraemia -> can cause SIADH
What should FEV1 increase by when testing for asthma with short acting bronchodilators?
12% or more
What genes are associated with Parkinsons?
SNCA, LRRK2, PINK1
What are some non motor symptoms of Parkinsons?
- Cognitive impairment
- Depression
- Hallucinations
- Constipation
- Sleep disorders
Investigations for TIA?
- CT/MRI
- Echo
- 24 hour ECG
- Carotid doppler
What are complications of GCA?
- Visual loss
- Aortic aneurysm
- Seizures
- Stroke
Which chromosome is affected in Huntingtons?
Chromosome 4
What is the pathophysiology of Huntingtons?
Expansion of CAG triple repeats in the huntingtin gene
What are Alzheimers symptoms?
- Confusion
- Apraxia
- Depression
- Hallucinations
What are the 2 drugs for Alzheimers?
Cholinesterase inhibitors - Donepezil
NMDA antagonists - Memantine
Third nerve palsy with decreasing consciousness and heamatoma?
Trans-tentorial herniation
What investigation could be done to investigate suspected trigeminal neuralgia?
MRI head -> assess for underlying cause e.g MS
If CT is done within 6 hours of suspected SAH and is normal….
Do not do LP - consider alternative diagnosis
weakness of foot dorsiflexion and foot eversion
common peroneal lesion
what causes action potential prolongation in both sensory and motor axons?
Carpal tunnel
How long should symptoms be present before being able to diagnose chronic fatigue syndrome?
3 months
burning and tingling sensation over the upper lateral area of the thigh with no motor deficits
Think merralgia paraesthetica
loss of sensation to the palmar and dorsal aspect of the 5th digit
Ulnar
What occular signs are seen in Wernickes?
- Nystagmus
- Opthalmoplegia: lateral rectus palsy
When should amoxicillin be given to cover for Listeria in meningitis?
Over 60s
Fever on alternating days?
Malaria
Falling from a tree and grabbing a branch whilst falling -> claw hand?
Klumpke’s palsy -> T1 nerve damage
What should be given in non falciparum malaria?
Primaquine
Why is primaquine used in non-falciparum malaria?
Destroy liver hypnozoites and prevent relapse
Which MND has the worst prognosis?
Progressive bulbar palsy
lymphocytic CSF with high protein and low glucose
TB meningitis
What are options to reduce relapses in MS?
- Natalizumab
What are the 2 first line drugs for spasticity in MS?
Baclofen and Gabapentin
What are complications of spinal cord compression?
- Aspiration
- Pressure sores
- Depression
- Pneumonia
- Autonomic dysfunction
Epilepsy DVLA rules
First unprovoked/isolated seizure - 6 months
Established epilepsy - seizure free for 12 months
What causes short headaches before an SAH?
Small leaks from the aneurysm
What kind of signs would MS give?
UMN
What autoimmune conditions are associated with myasthenia gravis?
- Graves
- Hashimotos
- RA
What is the key feature of MG?
Fatigability - muscles become progressively weaker during periods of activity and improve after rest
What are the antibodies of MG?
- Anti-acetylcholine receptors
- anti muscle-specific tyrosine kinase antibodies
What is the Tensilon test?
IV edrophonium which reduces muscle weakness temporarily
What is the main complication with MG?
Acute respiratory failure due to weakness of the muscles of ventilation.
CT head showing temporal lobe changes
Herpes encephalitis
Sensory loss posterolateral aspect of leg and lateral aspect of foot
S1
Which organisms can cause GBS?/
- Campylobacter
- CMV
- Hepatitis
- Mycoplasma pneumoniae
- EBV
- HIV
What are types of CNS tumours?
Glioma - most common
Meningioma
Glioblastoma
What is the treatment for Lewy-Body dementia?
- Same as Alzheimers
- Donepezil/Rivastigmine
What criteria is used for MS diagnosis?
McDonald
Cushings reflex
- HTN
- Bradycardia
- Irregular breathing
Signs of expanding EDH?
- N+V
- Slurred speech
- Seizures
- Reduced GCS
What is coning?
Herniation of cerebellar tonsils through foramen magnum causing compression of brainstem
Management of ?brain abscess on CT?
IV Abx
MRI to rule out brain mets
Drainage if confirmed abscess
Meningitis complications?
Septic shock
Hearing loss
Seizures
Intellectual impairment
Abscess
Memantine side effect?
Constipation
CSF antibodies against NMDA?
Autoimmune encephalitis
What test should be done before starting acetylcholinesterase inhibitors?
ECG - can prolong QT
cerebral venous sinus thrombosis treatment?
LMWH
A hyperdense artery sign on CT can be a sign of what?
Ischaemic stroke
Effacement of the cerebral ventricles and loss of grey-white matter differentiation
Raised ICP
Hyperdense material in the cerebral sulci and basal cisterns
SAH
What is cortical basal degeneration?
Parkinson’s triad + spontaneous activity of an affected limb
What is internuclear opthalmoplegia?
Lesion in medial longitudinal fasciculus causing impaired adduction in affected side and nystagmus in opposite eye
What are some factors with worsen prognosis of MS?
- Older
- Male
- Many MRI lesions
- Early relapses
What is myotonic dystrophy?
- 20s year old with cataracts, muscle wasting of hands, slow relaxing grip
Management of lumbar spine stenosis?
- Pain management, physio
- Surgery only for severe cases e.g. laminectomy
Most common cause of spontaneous intracerebral haemorrhage?
HTN
REM sleep disturbances is a feature of what?
Lewy body dementia
What is decorticate?
Abnormal flexion in response to pain
India ink stain is used to test for what?
Cryptococcus -> Fungal meningitis
HIV patient with meningitis signs?
Fungal -> Cryptococcus
Excessive nitrous oxide inhalation can cause what?
Subacute degeneration of spinal cord
Vertigo, nystagmus, dysphagia, Horners?
PICA
CAG triple repeat with anticipation through spermatogenesis?
Huntingtons
Bell’s palsy?
acute unilateral facial nerve weakness or paralysis of rapid onset (<72hrs) and unknown causes
What may contribute to the development of Bell’s palsy (but mainly unknown cause)?
HSV, varicella zoster, autoimmunity
What age is Bell’s palsy most common?
15-45yrs
Cx of Bell’s palsy?
eye injury, facial pain, dry mouth, intolerance to loud noises, abnormal facial muscle contraction during voluntary movements, psychological Cx
Diagnosis of Bell’s palsy?
clinical, when no other condition is found to be causing facial weakness or paralysis
Symptoms of Bell’s palsy?
- rapid onset (<72hrs)
- unilateral facial muscle weakness invl upper AND lower parts of face
- reduction in movement on affected side
- drooping of eyebrows, corner of mouth and loss of nasolabial fold
- ear & postauricular pain on affected side
- difficulty chewing, dry mouth, changes in taste
- incomplete eye closure, dry eye, eye pain, or XS tearing
- numbness/tingling of cheek +/- mouth
- speech articulation problems, drooling
- hyperacusis
Mx of Bell’s palsy?
Present <72hrs of onset= prednisolone 50mg daily 10d
- keep affected eye lubricated= eye drops & artificial tears in day, at night- oitnment and tape eye closed
- specialist advice= ? antiviral + corticosteroid (may be recommended)
Urgent referral for Bell’s palsy if it may be caused by what?
- UMN cause
- cancer
- trauma
- acute or severe local infection
Bell’s palsy- when to refer to specialist?
- atypical features
- no improvement >3w after Tx
- new neurologic or worsen symptoms
- ocular symptoms= pain, irritation, itch
- incomplete recovery >3m of onset
Atypical features of Bell’s palsy that require referral for exclusion of an alternative diagnosis?
- gradual progression
- > 6m; ipsilateral hearing loss; pain within facial nerve distribution
- parotid mass
- previous stroke, brain tumour, trauma, recent infection, skin ca of head or face
- systemic illness or fever
- dysphagia, diplopia, vestibular abnormalities
- eye brow spared
- bilateral
- recurrent
- uneven distribution of weakness
Examination for Bell’s palsy?
complete head and neck exam
1) cranial nerve
2) otoscopy, Weber’s & Rinne’s
3) parotid gland
4) skin of head, face, cheek, oral cavity, mastoid: swelling, rashes
5) eyes & periocular complex
SHOULD be unremarkable except uniformly distributed one-sided facial palsy
Differential diagnosis for Bell’s palsy?
- infection= HSV, Lyme disease, otitis media, mastoiditis, cholesteatoma, Ramsay Hunt, meningitis, encephalitis, HIV, syphilis, glandular fever
- stroke
- brain tumour
- trauma
- facial nerve tumour, parotid tumour
- skin ca
- diabetes
- MS
- Guillain-Barre
- sarcoidosis
- arteriovenous malformation
Ramsay Hunt vs Bell’s palsy?
RH= pain followed by vesicular rash on pinna, or in ear canal or pharynx; sensorineural hearing loss; facial nerve palsy
Bell’s= uniformly distributed one-sided facial palsy
Stroke vs Bell’s palsy?
Stroke= FOREHEAD SPARED- can raise eyebrows on affected side
Bell’s= can’t raise eyebrows, palsy includes forehead
Is the facial palsy in stroke- where forehead spared, a UMN or LMN palsy?
UMN
What group of people is Bell’s palsy more common in?
pregnant women
Is Bell’s palsy a UMN or LMN facial nerve palsy?
LMN= forehead affected
Why is eye care eg. artificial tears and eye lubricants important in Bell’s palsy?
prevent exposure keratopathy
Follow up in Bell’s palsy?
if paralysis shows no sign of improvement after 3w, refer urgent to ENT
long standing weakness eg. months= may refer to plastic surgery
Prognosis of Bell’s palsy?
most make full recovery in 3-4m
untreated= 15% pts have permanent moderate to severe weakness
Causes of brain abscess?
extension of sepsis from middle ear or sinuses, trauma or surgery to scalp, penetrating head injury, embolic events from endocarditis
CP of brain abscess?
depends on site eg. if in critical areas like motor cortex will present earlier
- raised ICP= nausea, papilloedema, seizures
- headache= dull, persistent
- fever= may be absent
- focal neurology eg. oculomotor palsy or abducens palsy secondary to raised ICP
Ix for brain abscess?
CT head
Mx for brain abscess?
- surgery= craniotomy, abscess cavity debrided (may reform as head is closed following drainage)
- also IV Abx= IV 3rd gen cephalosporin + metronidazole
- ICP Mx= dexamethasone
IV Abx for brain abscess?
IV 3rd gen cephalosporin + metronidazole
Cerebral palsy?
term for group of permanent movement and posture disorders of the developing fetal or infant brain which limit activity
Cause of cerebral palsy?
acquired pathology with the developing brain during the prenatal, neonatal or infant period
The impaired movement associated with cerebral palsy results from what?
centrally-mediated abnormal muscle tone which leads (most commonly) to spasticity
Features of cerebral palsy?
- abnormal muscle tone leading to spasticity
- disorders of sensation, perception, cognition, communication and behaviour
- may incl. MSK problems, neurogenic bladder, GORD, XS salivation and feeding & swallowing difficulties
RFs for cerebral palsy?
- prematurity
- multiple gestation, maternal infections eg. chorioamnioitis
- lower birth weight
Possible early motor features suggestive of cerebral palsy?
- unusual fidgety movements or asymmetry or paucity of movement
- abnorm tone: hypotonia, spasticity or dystonia
- abnorm motor development: late head control, rolling and crawling
- feeding difficulties
Most common delayed motor milestones in children with cerebral palsy (corrected for gestational age)?
- not sitting by 8m
- not walking by 18m
- asymmetry of hand function before 1yr (dominant hand)
Children at increased risk of cerebral palsy should receive what?
enhanced MDT developmental follow up to age of 2yrs
Cerebral palsy suspected?
referral to child development service for MDT assessment
Who is involved in the Mx for confirmed cerebral palsy?
MDT specialist Mx:
- peads, nurse, physio, occupational, SALT, dietician, psychology
- orthopaedic surgery (& post-surgery rehab), rehav medicine, specialist neurology service, social care, learning disability services, mental health services
Common medical issues in pts with cerebral palsy that may need Mx?
nutrition problems, pain, mental health, constipation, GORD, resp problems
When does spasticity in cerebral palsy typically present?
after 1st yr when child attempts activities
What is spasticity in cerebral palsy characterised as?
hypertonia, abnormal deep tendon reflexes (increased), presence of clonus
progressive contractures or deformities can follow by age of 5yrs esp during periods of rapid growth
When to suspect a different diagnosis if already suspect cerebral palsy?
- absence of CP RFs
- FHx of progressive neuro disorder
- loss of already attained cognitive or developmental abilities
- development of unexpected focal neuro signs
Differential diagnosis for cerebral palsy?
- brain tumour
- dystonia
- muscular dystrophy
- myelodysplasia
- spinal muscular atrophy
- spinal stenosis/tethered cord
Cerebral palsy if defined as a disorder of movement and posture due to a non-progressive lesion of the….
motor pathways in the developing brain
Most common cause of major motor impairment?
cerebral palsy
Antenatal (80%), intrapartum (10%) and postnatal (10%) causes of cerebral palsy?
antenatal= cerebral malformation and congenital infection (rubella, toxoplasmosis, CMV)
intrapartum= birth asphyxia/trauma
postnatal= intraventricular haemorrhage, meningitis, head-trauma
Is cerebral palsy progressive?
no
Main possible mainfestations of cerebral palsy (4)?
- abnormal tone early infancy
- delayed motor milestones
- abnormal gait
- feeding difficulties
Children with cerebral palsy often have associated non-motor problems such as…
- learning difficulties (60%)
- epilepsy (30%)
- squints (30%)
- hearing impairment (20%)
Classification of cerebral palsy?
1) Spastic (70%)
2) Dyskinetic
3) Ataxic
4) Mixed
Spastic cerebral palsy subtypes?
hemiplegia, diplegia, quadriplegia
Spastic cerebral palsy?
subtypes= hemi, di or quadriplegia
increased tone resulting from damage to UMN
Dyskinetic cerebral palsy?
caused by damage to basal ganglia and substantia nigra
athetoid movements and oro-motor problems
Ataxic cerebral palsy?
caused by damage to cerebellum with typical cerebellar signs
Subtype of cerebral palsy: cerebellar signs?
ataxic
increased tone resulting from damage to UMN?
spastic cerebral palsy
Subtype of cerebral palsy: athetoid movements and oro-motor problems?
dyskinetic
Tx that can be used in cerebral palsy (Mx includes MDT approach)?
spasticity= oral diazepam, oral and intrathecal balofen, botulinum toxin type A, orthopaedic surgery and selective dorsal rhizotomy
analgesia
anticonvulsants
Chronic fatigue syndrome?
diagnosed after at least 3m of disabling fatigue affecting mental and physical function more than 50% of the time in the absence of other disease that may explain symptoms
Epidemiology of chronic fatigue syndrome?
females more common
past psychiatric history NOT a risk factors
Features of chronic fatigue syndrome?
- FATIGUE
- sleep problems= insomnia, hypersomnia, unrefreshing sleep, disturbed sleep-wake cycle
- muscle +/or joint pains
- painful lymph nodes without enlargement
- sore throat
- cognitive dysfunction= difficulty thinking, inability to concentrate, impaired short term memory, diff word finding
- general malaise/flu like symptoms
- dizziness
- nausea
- palpitations
What makes symptoms worse in chronic fatigue syndrome?
physical or mental exertion
Ix and Diagnosis for chronic fatigue syndrome?
Ix= screening blood to exclude other pathology eg. FBC, U&E, LFT, glucose, TFT, ESR, CRP, Ca, CK, ferritin, coeliac screening, urinalysis
Diagnosis= symptoms persist for 3m
Chronic fatigue syndrome (CFS) has a better prognosis in who?
children
Mx for chronic fatigue syndrome?
- refer to specialist CFS if diagnostic criteria met
- energy Mx= self-management strategy to keep activities within energy limit with support from healthcare proffessional
- Physical activity and exercise= only if specialist advised, if pt feels ready to progress activity beyond current ADLs
- CBT: supportive not curative
ME vs CFS?
Myalgic Encephalomyelitis (ME) and Chronic Fatigue Syndrome (CFS) are overlapping conditions often termed ME/CFS due to similar symptoms like severe fatigue, post-exertional malaise (PEM), and cognitive impairment.
ME historically focuses on neurological and muscular symptoms, implying inflammation in the brain and spinal cord, while CFS centers on prolonged, unexplained fatigue and broader systemic symptoms. Both conditions can follow viral infections and have no single known cause, with diagnosis based on symptom clusters rather than specific tests.
Dementia?
progressive irreversible clinical syndrome with range of cognitive and behavioural symptoms incl. memory loss, problems with reasoning and communication, change in personality, reduction in pts ability to carry out daily activities
How severe is decline in cognition in dementia?
extensive, often affects multiple domains of intellectual functioning
RFs for dementia?
ageing
mild cognitive impairment
genetics
Parkinson’s
cerebrovascular disease
CVD
Modifiable RFs for dementia?
smoking, DM, lack of physical activity, obesity
Mild cognitive impairment?
cognitive impairment that does not fulfil the diagnostic criteria for dementia
Common subtypes of dementia?
- Alzheimers (50-75%)= often co-exists with other forms eg. vascular
- Vascular dementia (20%)
- Dementia with Lewy bodies (10-15%)
- Frontotemporal dementia (2%)
When to suspect dementia?
- cog impairment= memory problems (eg. diff learning new info_, dysphasia and dyspraxia, disorientation to time and place, impairment of executive function (diff planning & problem solving)
- difficulties with ADLs= eating, hygiene, grooming, dressing
- behavioural & psychological symptoms of dementia (BPSD)= delusions, hallucinations, agitiation, emotional lability, depression, anxiety, apathy, social/sexual disinhibition, motor disturbance (eg. wandering or repetitive activity), sleep disruption
Behavioural & psychological symptoms of dementia (BPSD)
delusions, hallucinations, agitiation, emotional lability, depression, anxiety, apathy, social/sexual disinhibition, motor disturbance (eg. wandering or repetitive activity), sleep disruption
Ix for dementia?
- cognitive assessment: MMSE, Montreal Cognitive Assessment, 10 point cognitive screen or 6 item Cognitive Impairment Test
- Hx from someone who knows pt well= IQCODE or FAQ
- neuro exam
- Rule out reversible causes= depression, hypothyroidism, delirium
Mx of pt with suspect dementia should invl what?
- admission is severely disturbed
- refer to memory assessment service for specialist assessment and Mx
Pt with mild cognitive impairment?
follow up regularly to monitor possible progression of cognitive deficit- if deteriorate refer to assessment and Mx ?dementia
Follow up in primary care for pt with dementia?
- planning ahead= lasting power of attorney, advance statement, decisions, preferences for place of care and death (ReSPECT)
- ensure have regularly reviewed care plan
- monitor response and adverse effects of Tx and progression
- review meds
How may difficulties with ADLs for pt with dementia deteriorate?
early stages= diff carrying out complex household tasks
later= bathing, toileting, eating and walking affected
Symptoms specific for Alzheimer’s disease?
- presenting symptom= loss of recent memory first and diff with executive function and/or normal dysphasia
- loss of episodic memory= memory loss of recent events, repeated questioning and diff learning new info
- cognitive deficits= aphasia, apraxia and agnosia
aphasia, apraxia and agnosia eg in Alzheimer’s?
inability to remember a word (amnesia) and the inability to understand a word (aphasia) or the inability to recognize a fork as a fork (agnosia) and the inability to use it properly (apraxia)
Symptoms specific for vascular dementia?
- stepwise increases in severity of symptoms= subcortical ischaemic vascular dementia may present insidiously with gait and attention problems and changes in personality
- focal neuro signs (hemiparesis or visual field defects) may be present
Symptoms specific for dementia with Lewy bodies?
fluctuating cognition, recurrent visual hallucinations, REM sleep behaviour disorder and one or more symptoms of parkinsonism= bradykinesia, rest tremor or rigidity
memory impairment may not be apparent in early stages
Symptoms specific for frontotemporal dementia?
- personality change and behavioural disturbance eg. apathy or social/sexual disinhibition; may develop insidiously
- other cognitive functions eg. memory and perception may be relatively preserved
10-point Cognitive Screener (10-CS)?
a type of cognitive assessment tool for dementia
involves 3 temporal orientation questions (year, month, date), a 3-word recall, and a 4 point scaled animal naming task.
One point is scored for each of the temporal questions and each word recalled, and the scores for the animal naming task range from 0 points for 0–5 animals, to 4 points for 15 or more animals.
A score of 8 or more is normal, 6–7 indicates possible cognitive impairment, and 0–5 indicates probable cognitive impairment
Differential diagnosis for dementia?
- normal age related memory changes
- mild cognitive impairment
- depression
- delirium
- vitamin def eg. thiamine, B12
- hypothyroidism
- adverse drug effects
- normal pressure hydrocephalus
- sensory deficits eg. vision or hearing
subcortical dementia is a what condition
HIV indicator condition
Normal pressure hydrocephalus can present with what symptoms?
early cognitive impairment, urinary incontinence and gait disorder
What to do with pt with suspected rapidly progressive dementia?
refer to neuro with access to tests (including cerebrospinal fluid examination) for Creutzfeldt–Jakob disease and similar conditions.
Specialist Ix for suspected dementia?
- MRI
- Alzheimer’s= examine CSF for tau protiens and amyloid beta plaques
- Lewy body= can use single-photon emission computed tomography (SPECT) (aka DaTscan)
Specialist Mx for dementia?
Non-pharmacological= cognitive stimulation therapy eg. activities and discussions aimed at improvement of cognitive and social functioning
Medical:
- Acetylcholinesterase (AChE) inhibitors= donepezil, galantamine or rivastigmine (mild to moderate)
- Memantine (N-methyl-D aspartic acid receptor antagonist) for severe- only to be used in Alzheimer’s
Drug for mild-moderate Alzheimer’s?
Donepezil, galantamine or rivastigmine
(Acetylcholinesterase inhibitors)
2nd line drug Tx/severe Alzheimers?
memantine
(NMDA receptor antagonist)
Managing non-cognitive symptoms in dementia?
antidepressants NOT recommended
antipsychotics for distress, agitation, hallucinations, risk of harming themselves/others (AVOID in Lewy bodys as may get irrevserible parkinsonism)
Donepezil (for demenita) contraindicated in who?
pts with bradycardia
Donepezil (for dementia) adverse effect?
insomnia
What is Alzheimers?
progressive degenerative disease of brain & most common type of dementia
RFs for Alzheimers?
- ageing
- FHx
- inherited
- Caucasian ethnicity
- Down’s syndrome
Inheritance of Alzheimers?
5% of cases are inherited as an autosomal dominant trait
mutations in the amyloid precursor protein (chromosome 21), presenilin 1 (chromosome 14) and presenilin 2 (chromosome 1) genes are thought to cause the inherited form
apoprotein E allele E4 - encodes a cholesterol transport protein
Macroscopic pathological changes in Alzheimers?
widespread cerebral atrophy, particularly involving cortex and hippocampus
Microscopic pathological changes in Alzheimer’s?
cortical plaques due to deposition of type A-Beta-amyloid protein and intraneuronal neurofibrillary tangles caused by abnormal aggregation of the tau protein
hyperphosphorylation of the tau protein has been linked to AD
Biochemical pathological changes in Alzheimers?
there is a deficit of acetylcholine from damage to an ascending forebrain projection
Neurofibrillary tangles?
seen in Alzheimers
paired helical filaments are partly made from a protein called tau
tau is a protein that interacts with tubulin to stabilize microtubules and promote tubulin assembly into microtubules
in AD are tau proteins are excessively phosphorylated, impairing its function
MMSE score of what suggests dementia?
24 or less out of 30
What tests can be done to rule out reversible causes of dementia?
bloods= FBC, U&E, FLTs, Ca, glucose, ESR/CRP, TFTs, vit B12, folate
neuroimaging to exclude subdural haematoma, normal pressure hydrocephalus ect and to provide info on aetiology for prognosis and Mx
What is FTLD?
frontotemporal lobar degeneration (FTLD)
3 types of frontotemporal lobar degeneration?
- Frontotemporal dementia (Pick’s disease)
- Progressive non fluent aphasia (chronic progressive aphasia, CPA)
- semantic dementia
Main common features of frontotemporal lobar demenita?
onset <65yrs
insidious onset
relatively preserved memory and visuospatial skills
personality change and social conduct problems
hallucinations, hyperorality, disinhibition, increased appetite, perseveration behaviours
Another name for frontotemporal dementia?
Pick’s disease
What is characteristic finding of Pick’s disease (frontotemporal dementia)?
focal gyral atrophy with knife-blade appearance
Macroscopic changes seen in Pick’s disease (frontotemporal dementia)?
atrophy of frontal and temporal lobes
(focal gyral atrophy with knife-blade appearance)
Microscopic changes seen in Pick’s disease (frontotemporal dementia)?
Pick bodies - spherical aggregations of tau protein (silver-staining)
Gliosis
Neurofibrillary tangles
Senile plaques
Mx of frontotemporal dementia?
DO NOT recommend AChE inhibitors or memantine
SSRIs or antipsychotics
CPA?
(Posterior cortical atrophy)
non fluent speech
short utterances that are agrammatic
comprehension preserved
Semantic dementia?
Fluent progressive aphasia.
The speech is fluent but empty and conveys little meaning.
Unlike in Alzheimer’s memory is better for recent rather than remote events.
Characteristic pathological feature in Lewy body dementia?
alpha-synuclein cytoplasmic inclusions (Lewy bodies) in the substantia nigra, paralimbic and neocortical areas.
Up to 40% of pts with Alzheimers also have?
lewy bodies
Relationship between Parkinsons and Lewy body dementia?
LB= cognitive impairment before parkinsonism but usually occur within a yr of each other
P= motor symptoms present at least 1yr before cognitive symptoms
Main features of Lewy body dementia?
- progressive cognitive impairment 1st
- followed by parkinsonism symptoms (within 1 yr)
- cognition may be fluctuating (different to other dementias)
- visual hallucinations
- may have delusions and non-visual hallucinations
How is Alzheimer’s different to Lewy body dementia?
A= early impairment is in attention and executive function rather than just memory loss
LB= mainly memory loss
Diagnosis of Lewy body dementia?
usually clinical
can use single-photon emission computed tomography (SPECT) (aka DaTscan) (90% sensitivity and 100% specificity)
Mx of Lewy body dementia?
same as Alzheimer’s
AVOID neuroleptics ( eg. antipsychotics) as pts v sensitive and may develop irreversible parkinsonism
Pt with Lewy body dementia deteriorated and with irreversible parkinsonism- why?
been given neuroleptic drug eg. antipsychotic (eg. 1st gen)
MUST AVOID IN LEWY BODY DEMENTIA
What is vascular dementia?
not a single disease but group of syndromes of cognitive impairment caused by different mechanisms causing ischaemia or haemorrhage secondary to cerebrovascular disease
Most severe form of the spectrum of deficits encompassed by the term vascular cognitive impairment (VCI)?
vascular dementia
What is important in the prevention of vascular dementia?
early detection and accurate diagnosis
Prevalence of dementia in vascular dementia following a stroke?
Prevalence of dementia following a first stroke varies depending on location and size of the infarct, definition of dementia, interval after stroke and age among other variables. Overall, stroke doubles the risk of developing dementia.
RFs for vascuclar dementia?
- ageing
- stroke doubles risk
- History of stroke or transient ischaemic attack (TIA)
- Atrial fibrillation
- Hypertension
- Diabetes mellitus
- Hyperlipidaemia
- Smoking
- Obesity
- Coronary heart disease
- A family history of stroke or cardiovascular
Subtypes of vascular dementia?
stroke-related VD
subcortical VD
mixed dementia
Subtypes of vascular dementia: stroke-related VD?
multi infarct or single infarct dementia
Subtypes of vascular dementia: subcortical VD?
caused by small vessel disease
Subtypes of vascular dementia: caused by small vessel disease?
subcortical VD
Subtypes of vascular dementia: mixed dementia?
presence of both VD and Alzheimers
Mixed dementia?
presence of both VD and Alzheimers
Example of vascular dementia being inherited (rare)?
CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy)
Pts with vascular dementia typically present with what?
Several months or several years of a history of a sudden or stepwise deterioration of cognitive function.
Symptoms and speed of progression in vascular dementia vary but may include what?
Focal neurological abnormalities e.g. visual disturbance, sensory or motor symptoms
The difficulty with attention and concentration
Seizures
Memory disturbance
Gait disturbance
Speech disturbance
Emotional disturbance
Diagnosis of vascular dementia?
- clinical
- formal screen for cognitive impairment
- exclude medication cause of cognitive decline
- MRI= may show infarcts and extensive white matter changes
What criteria can be used to diagnose probable vascular dementia?
NINDS-AIREN
1) Presence of cognitive decline that interferes with activities of daily living, not due to secondary effects of the cerebrovascular event
- established using clinical examination and neuropsychological testing
2) Cerebrovascular disease
- defined by neurological signs and/or brain imaging
3) A relationship between the above two disorders inferred by:
- the onset of dementia within three months following a recognised stroke
- an abrupt deterioration in cognitive functions
- fluctuating, stepwise progression of cognitive deficits
General Mx for vascular dementia?
- symptomatic: adress pts problems and provide support
- detect and address CV risk factors for slowing down progression
Non-pharmacological Mx for vascular dementia?
Tailored to the individual
Include: cognitive stimulation programmes, multisensory stimulation, music and art therapy, animal-assisted therapy
Managing challenging behaviours e.g. address pain, avoid overcrowding, clear communication
Pharmacological Mx for vascular dementia?
There is no specific pharmacological treatment approved for cognitive symptoms
ONLY consider AChE inhibitors or memantine for people with vascular dementia if they have suspected comorbid Alzheimer’s disease, Parkinson’s disease dementia or dementia with Lewy bodies.
There is no evidence that aspirin is effective in treating patients with a diagnosis of vascular dementia.
No randomized trials found evaluating statins for vascular dementia
Possible MRI findings in vascular dementia?
infarcts and extensive white matter changes
Example of a temporal lobe sign
aphasia
In herpes simplex encephalitis, where does the virus characteristically affect?
temporal lobes
inferior frontal lobes
Features of herpes simplex encephalitis?
- fever, headache, psychiatric symptoms, seizures, vomiting
- focal features eg. aphasia
- peripheral lesions (eg. cold sores) have to relation to presence of HSV encephalitis
Are cold sores related to HSV encephalitis?
no
Pathophysiology of herpes simplex encephalitis?
HSV-1 responsible for 95% cases
typically affects temporal and inferior frontal lobes
Ix for herpes simplex encephalitis?
- CSF= lymphocytosis, elevated protein
- PCR for HSV
- CT (MRI better)
- EEG
What would CT show in herpes simplex encephalitis (MRI better)?
medial temporal and inferior frontal changes eg. petechial haemorrhages
normal in 1/3
EEG pattern in herpes simplex encephalitis?
lateralised periodic discharges at 2Hz
What would CSF show in herpes simplex encephalitis?
lymphocytosis, elevated protein
MRI findings in herpes simplex encephalitis?
hyperintensity of affected white matter and cortex in the medial temporal lobes and insular cortex
Tx for herpes simplex encephalitis?
IV aciclovir
Prognosis for herpes simplex encephalitis?
dependent on whether aciclovir is commenced early
if Tx prompt then mortality 10-20%
untreated= 80%
Seizure?
transient occurence of signs or symptoms due to abnormal XS or synchronous neuronal activity in the brain
Epilepsy?
disease of brain defined by any of the following:
- at least 2 unprovoked seizures occurring >24hrs apart
- 1 unprovoked seizure and probability of further seizures similar to general recurrence risk after 2 unprovoked seizures, occurring over next 10yrs
- diagnosis of epilepsy syndrome
Convulsive status epilepticus?
prolonged convulsive seizure for 5mins or longer, or recurrent seizures one after the other without recovery in between
Causes of epilepsy?
only identified in 1/3 pts
structural, genetic, infectious, metabolic, immune
more common in people with learning disabilities
Clinical features of epilepsy are specific to what?
type of seizure
Features of epilepsy: tonic seizure?
Short-lived (less than 1 minute), abrupt, generalised muscle stiffening (may cause fall) with rapid recovery
Features of epilepsy: generalised tonic-clonic seizure?
generalised stiffening and subsequent rhythmic jerking of the limbs, urinary incontinence, tongue biting
Features of epilepsy: absence seizure?
behavioural arrest
Features of epilepsy: atonic seizure?
sudden onset of loss of muscle tone
Features of epilepsy: myoclonic seizure?
brief, shock-like involuntary single or multiple jerks
Immediate Mx of pt having a seizure?
- protect pt from injury, check airway, place them in recovery position until seizure stops
- if tonic-clonic is prolonged or recurrent= emergency buccal midazolam 1st line in community if prescribed
- emergency admission to hospital if seizures don’t respond prompt to Tx
All pts suspected of having 1st epileptic seizure should be?
urgently referred to specialist to confirm diagnosis
How often should all pt with epilepsy be routinely reviewed?
yearly
All people with epilepsy should have a routine review at least once a year to assess what?
- seizure control, adverse effects, compliance with Tx
- impact on work, education, leisure activities and how to manage risks
- entitlement to drive
- carers’ skills in managing seizures
- contraception needs and preg planning info if needed
Specialist advice should be sought for a person with confirmed epilepsy with what?
- poor seizure control or poorly tolerated Tx
- previous prolonged or recurrent seizures, who have not been prescribed emergency Tx for us in community
- possible cognitive impairment
- seizure-free history for at least 2yrs who would like to consider tapering or withdrawl from drug Tx
- plans for pregnancy
RFs causing a predisposition for epilepsy?
- Premature birth.
- Complicated febrile seizures.
- A genetic condition that is known to be associated with epilepsy, eg. tuberous sclerosis or neurofibromatosis.
- Brain development malformations – usually associated with epilepsy developing before adulthood.
- FHx of epilepsy or neurologic illness.
- Head trauma, infections (for example meningitis, encephalitis), or tumours — can occur at any age.
- Comorbid conditions such as cerebrovascular disease or stroke — more common in older people.
- Dementia and neurodegenerative disorders (people with Alzheimer’s disease are up to ten times more likely to develop epilepsy than the general population).
What genetic conditions are known to be associated with epilepsy?
tuberous sclerosis
neurofibromatosis
What to ask pt presenting with first seizure?
- symptoms at start of seziure (aura)= may suggest focal epilepsy
-potential triggers
- features of seizure= what type?
- residual symptoms after the attack (post-ictal)
- exam oral mucosa for lateral tongue bites
- incontinence?
- anyone wittnessed
Epilepsy: potential triggers of a seziure?
sleep deprivation, stress, light sensitivity, alcohol use
Epilepsy: post-ictal symptoms?
drowsiness, headaches, amnesia, confusion
In epilepsy, after what type of seizures do you get post-ictal symptoms after?
only after generalised tonic and/or clonic seizures
What type of seizure is sometimes associated with urinary incontinence and tongue biting?
generalised tonic clonic
Initial Ix when pt presents with 1st seizure?
- REFER
- cardiac neuro, mental state and developmental assessment if appropriate
- examine for tongue biting
- any injuries sustained
- 12-lead ECG= ?cardiac related conditions that could mimic epileptic seizure
- baseline tests eg. bloods= FBC, U&E, LFTs, glucose and Ca
Differential diagnosis for epilepsy?
- Vasovagal syncope.
- Cardiac arrhythmias.
- Panic attacks with hyperventilation.
- Non-epileptic attack disorders (psychogenic non-epileptic seizures, dissociative seizures, or pseudoseizures).
- TIA
- Migraine.
- Medication, alcohol, or drug intoxication.
- Sleep disorders.
- Movement disorders.
- Hypoglycaemia and metabolic disorders.
- Transient global amnesia.
- Delirium or dementia — altered awareness may be mistaken for seizure activity.
Differential diagnosis for epilepsy in children?
- febrile convulsions
- breath-holding attacks
- night terrors
- stereotyped/ritualistic behaviour eg. those with learning disability
What to advise a pt with suspected epilepsy before had their referral appointment?
- epilepsy used to describe condition where people have tendency to have seizures but there are other possible causes for seizures
- stop driving whilst waiting to see specialist, avoid dangerous work, heights, swimming, shower not baths
- lifestyle factors that may lower seizure threshold eg. sleep deprivation, alcohol, social drugs
- take witness of seizure to 1st appointment if possible
Mx of pt having tonic clonic seizure and note time, if lasts less than 5 mins…
- look for epilepsy identity card/jewellery
- protect from injury= cushion head, remove glasses and harmful objects
- do not restrain or put anything in mouth
- when stops= check airway and put in recovery position
- observe until recovered
- examine and manage any injuries
- arrange emergency admission if= 1st seizure; another reoccurs shortly after 1st one; if pt is injured, trouble breathing or difficult to wake
Additional measures to Mx tonic-clonic seizure lasting more than 5 mins or who have >3 seizures in 1 hr?
- Tx with either= buccal midazolam; rectal diazepam if buccal midazolam not available; IV lorazepam if IV access already established
- call ambulance if don’t respond to Tx or if= prolonged or recurrent Tx given; high risk of recurrence (Hx of status epilepticus or repeated seizures); difficulty monitoring condition; 1st seizure
- arrange for specialist review to consider buccal midazolam
Mx for pt with epilepsy having a focal seizure eg. unusual movements or behaviour, wandering?
- protect them from injury= remove harmful objects or if not possible remove pt from immediate danger
- do not restrain
- observe until fully recovered- do not give anything to eat or drink until fully recovered
- reassure and explain
- examine for injury
- if 1st seizure, >5mins or need urgent medical attention then emergency admission
Tx in epilepsy for pt having seizure >5mins or recurrent seizure without recovery in between or >3 in 1hr?
community= buccal midazolam; (2nd line: rectal diazepam)
hospital= IV lorazepam
Advise for women with epilepsy taking lamotrigine?
Oestrogen-containing contraceptives may reduce the effectiveness of lamotrigine because of a reduction in circulating lamotrigine levels which may result in increased seizure activity.
Progestogen-only contraceptives can be used without restriction, but the woman should report any symptoms or signs of lamotrigine toxicity.
What antieplieptic should not be given to women of child-bearing age/sexually active?
valproate
topiramate
contraceptive advice should be given to women and girls before they become sexually active
What conditions are associated with epilepsy?
cerebral palsy (30% have epilepsy)
tuberous sclerosis
mitochondrial disease
Causes of seizures?
infection
trauma
metabolic disturbance
epilepsy
febrile convulsions
alcohol withdrawl
psychogenic non-epileptic seizures
Summary of febrile convulsions?
6m-5yrs
usually early in a viral infection as temp rises
brief and generalised tonic/tonic-clonic
3% of all children will have at least 1
When does alcohol withdrawl seizures occur?
when pt with history of alcohol XS suddenly stops drinking eg. admitted to hospital
around 36hrs after cessation of drinking
What is usually given to pts with history of alcohol XS following cessation of drinking to reduce risk of withdrawl seizures?
benzodiazepines
Pathophysiology behind seizures following alcohol withdrawl?
chronic alcohol consumption enhances GABA mediated inhibition in the CNS (similar to benzodiazepines) and inhibits NMDA-type glutamate receptors.Alcohol withdrawal is thought to be lead to the opposite (decreased inhibitory GABA and increased NMDA glutamate transmission)
Psychogenic non-epileptic seizures / psuedoseizures?
pts who present with epileptic like seizures but do not have characteristic electrical discharges
may have Hx of mental health problems or personality disorder
Classification of seizures?
1) where seizure begin in brain
2) level of awareness during seizure (can affect safety)
3) other features
Focal seizures (partial seizures) start where?
in a specific area, on one side of the brain
Level of awareness in focal seizures?
can vary
focal aware; focal impaired awareness and awareness unknown
How to classify focal seizures?
where in the brain they start
awareness
motor, non-motor or other features eg. aura
Are focal seizures motor?
can be motor eg. Jacksonian march; non motor eg. deja vu, jamais vu; or other features eg. aura
Jacksonian march typically in what seizure type?
focal
Generalised seizures involve what area of brain?
networks on both sides of the brain at the onset
Awareness in generalised seizures?
consciousness lost immediatley
Are generalised seizures motor?
motor eg. tonic clonic and non-motor eg. absence
Specific types of generalised seizure?
- tonic-clonic (grand mal)
- tonic
- clonic
- typical absence (petit mal)
- myoclonic= brief rapid muscle jerks
- atonic
Seizure: brief rapid muscle jerks?
myoclonic
Seizure of unknown onset?
when origin of seizure is unknown
Focal to bilateral seizure?
starts on one side of brain in specific area before spreading to both lobes
Special forms of epilepsy in children?
- infantile spasms (West’s syndrome)
- Lennox-Gastaut syndrome
- Benign rolandic epilepsy
- Juvenile mycoclonic epilepsy (Janz syndrome)
Infantile spasms (West’s syndrome)?
Brief spasms beginning in first few months of life
- Flexion of head, trunk, limbs → extension of arms (Salaam attack); last 1-2 secs, repeat up to 50 times
- Progressive mental handicap
- EEG: hypsarrhythmia
usually 2nd to serious neurological abnormality (e.g. TS, encephalitis, birth asphyxia) or may be cryptogenic
poor prognosis
Lennox-Gastaut syndrome?
Special form of epilepsy in children.
May be extension of infantile spasms (50% have hx)
onset 1-5 yrs
atypical absences, falls, jerks
90% moderate-severe mental handicap
EEG: slow spike
ketogenic diet may help
Benign rolandic epilepsy?
paraesthesia (e.g. unilateral face), seizures characteristically occur at night in children
Juvenile myoclonic epilepsy (Janz syndrome)?
Typical onset in the teens, more common in girls
- Infrequent generalized seizures, often in morning eg. after poor night sleep
- Daytime absences
- Sudden, shock-like myoclonic seizure
usually good response to sodium valproate
Postictal phase following seizure?
feel drowsy and tired for around 15mins
Ix for epilepsy?
EEG and MRI
Most neurologists start antiepileptics when?
following a second epileptic seizure
Why do we prescribe antiepileptics by brand rather than generically?
due to risk of slightly different bioavailability resulting in a lower seizure threshold
What are important things to think about when starting Mx for epilepsy?
- pts who drive
- pts taking other meds eg. warfarin
- women wishing to get pregnant
- women taking contraception due to possible interactions
Pts can’t drive for how long following seizure?
6m
Pts with established epilepsy must be fit free for how long before able to drive?
12m
What meds are important to look for before starting pt on antiepileptic?
can induce/inhibit the P450 system resulting in varied metabolism of other meds eg. warfarin
Name 4 antiepileptics?
sodium valproate
carbamazepine
lamotrigine
phenytoin
MOA of sodium valproate?
increases GABA activity
Adverse effects for sodium valproate?
increased appetite and weight gain
alopecia: regrowth may be curly
P450 enzyme inhibitor
ataxia
tremor
hepatitis
pancreatitis
thrombocytopaenia
teratogenic (neural tube defects) - should not be used in females of a reproductive age
Why is sodium valproate teratogenic?
can cause neural tube defects
MOA of carbamazepine?
binds to sodium channels increasing their refractory period
Adverse effects of carbamazepine (antiepileptic)?
P450 enzyme inducer
dizziness and ataxia
drowsiness
leucopenia and agranulocytosis
syndrome of inappropriate ADH secretion
visual disturbances (especially diplopia)
MOA of lamotrigine?
sodium channel blocker
Adverse effect of lamotrigine?
Stevens-Johnson syndrome
MOA of phenytoin?
binds to sodium channels increasing their refractory period
Adverse effects of phenytoin?
no longer used 1st line due to side effect profile
P450 enzyme inducer
dizziness and ataxia
drowsiness
gingival hyperplasia, hirsutism, coarsening of facial features
megaloblastic anaemia
peripheral neuropathy
enhanced vitamin D
metabolism causing osteomalacia
lymphadenopathy
What antiepileptics are P450 enzyme inducers and inhibitors?
Inhibitors= sodium valproate
Inducers= phenytoin, carbamazepine
Most seizures terminate spontaneously, but if don’t terminate after how long it is appropriate to administer medication?
5-10mins
What class of medication can be used as a ‘rescue med’ to terminate seizures lasting >5mins that can be given to family members?
benzodiazepines
Contraception whilst on antiepileptics?
consistent use of condoms + other method
If a COCP is chosen then it should contain a minimum of 30 µg of ethinylestradiol.
For women taking phenytoin,carbamazepine, barbiturates, primidone, topiramate, oxcarbazepine:
UKMEC 3: the COCP and POP
UKMEC 2: implant
UKMEC 1: Depo-Provera, IUD, IUS
For lamotrigine:
UKMEC 3: the COCP
UKMEC 1: POP, implant, Depo-Provera, IUD, IUS
Focal seizure= head/leg movements, posturing, post-ictal weakness, Jacksonian march?
frontal lobe (motor) focal seizure
Focal seizure= paraesthesia?
parietal lobe (sensory) focal seizure
Focal seizure= floaters/flashes?
occipital lobe (visual)
Focal seizures= rising epigastric sensation, deja vu and lip smacking?
temporal lobe
Signs and symptoms of temporal lobe focal seizure?
May occur with or without impairment of consciousness or awareness
An aura occurs in most patients
typically a rising epigastric sensation
also psychic or experiential phenomena, such as dejà vu, jamais vu
less commonly hallucinations (auditory/gustatory/olfactory)
Seizures typically last around one minute
automatisms (e.g. lip smacking/grabbing/plucking) are common
All women thinking about becoming pregnant and they have epilepsy should take what?
folic acid 5mg a day well before pregnancy to reduce risk of neural tube defects
Uncontrolled epilepsy during pregnancy?
The risks of uncontrolled epilepsy during pregnancy generally outweigh the risks of medication to the fetus
Antiepileptics in pregnancy?
aim for monotherapy
there is no indication to monitor antiepileptic drug levels
sodium valproate: associated with neural tube defects
carbamazepine: often considered the least teratogenic of the older antiepileptics
phenytoin: associated with cleft palate
lamotrigine: studies to date suggest the rate of
congenital malformations may be low. The dose of lamotrigine may need to be increased in pregnancy
Is breast feeding safe if mother takes antiepileptics?
yes but with the exception of the barbiturates
Pregnant women taking phenytoin are given what in the last month of pregnancy?
vit K to prevent clotting disorders in the newborn
Antiepileptics should be started after the first seizure if any of the following are present…
- pt have neuro deficit
- brain imaging shows structural abnormality
- EEG shows unequivocal epileptic activity
- pt or family consider risk of further seizure unacceptable
Maternal use of sodium valporate is associated with what?
significant risk of neurodevelopmental delay in children and neural tube defects
DO NOT USE in preg or women of childbearing age unless necessary
Drug Tx in epilepsy: generalised tonic-clonic seizures?
males: sodium valproate
females: lamotrigine or levetiracetam
girls aged under 10 years and who are unlikely to need treatment when they are old enough to have children or women who are unable to have children may be offered sodium valproate first-line
Drug Tx in epilepsy: focal seizures?
first line: lamotrigine or levetiracetam
second line: carbamazepine, oxcarbazepine or zonisamide
Drug Tx in epilepsy: absence seziures (Petit mal)?
first line: ethosuximide
second line:
male: sodium valproate
female: lamotrigine or levetiracetam
carbamazepine may exacerbate absence seizures
What antiepileptic may exacerbate absence seizures?
carbamazepine so avoid in absence
Drug Tx in epilepsy: myoclonic seizures?
males: sodium valproate
females: levetiracetam
Drug Tx in epilepsy: tonic or atonic?
males: sodium valproate
females: lamotrigine
Essential tremor (benign essential tremor)?
autosomal dominant condition which usually affects both upper and lower limbs
Features of essential tremor?
postural tremor: worse if arms outstretched
improved by alcohol and rest
most common cause of titubation (head tremor)
Mx for essential tremor?
1st line= propranolol
primidone sometimes used
Type of tremor in essential tremor?
postural tremor: worse if arms outstretched/intention movement
What improves essential tremor?
alcohol and rest
Febrile seizure (febrile convulsion)?
seizure accompanied by fever (temp >38) without CNS infection, infants and children aged 6m-5yrs
Type of seizure in simple febrile convulsion?
isolated generalised tonic-clinic seizures lasting <15mins that do not recur within 24hrs or within the same febrile illness, with complete recovery within 1hr
Features of complex febrile convulsion?
1 or more of:
- partial (focal) seizure (movement limited to 1 side of body or one limb)
- >15mins
- recurrence within 24hrs or within same febrile illness
- incomplete recovery within 1hr
Risk of recurrent seizures in a child following first febrile convulsion?
1/3
‘Complex’ febrile convulsion vs epilepsy?
FC complex= seizure >15mins complex
Febrile status epilepticus= >30mins
Epilepsy= >5mins is status epilepticus
Assessment of child with suspect febrile convulsions?
- red flags
- serious cause ? meningitis/meningococcal disease or encephalitis
- underlying cause of fever
- fever onset, peak temp, duration and relationship to seizure
- seizure and any post ictal drowsiness
- previous episodes and FHx of FC or epilepsy
- child’s temp, consciousness level, focal neuro deficit, fluid status, signs of alternative cause
Febrile convulsions: when should immediate hospital assessment by paediatrican be arranged?
- for first febrile seizure
- <18m age
- complex febrile seizure
- uncertainty
- unexplained fever
- focal neuro deficit, recent Abx use or parental anxiety
Refer to paeds/paeds neurologist should be arranged if child has febrile convulsion and…
has neurodevelopmenta delay and/or signs of neurocutaneous syndrome or metabolic disorder
Advice to parent/carer of child with Hx of febrile seizures?
- generally benign
- info and support
- prompt recognition and Mx of future seizures
-prophylactic antiepileptics or antipyretics NOT routinely prescribed unless speicalist advises
- ensure complete all childhood immunisations
What happens during febrile convulsion?
usually <5mins, rarely >10mins
eneralized tonic-clonic type (muscle stiffening followed by rhythmical jerking or shaking of the limbs, which may be asymmetrical); twitching of the face, rolling back of the eyes, staring and losing consciousness.
May be foaming at the mouth, difficulty breathing, pallor, or cyanosis.
A brief post-ictal period of drowsiness, irritability, or confusion, with complete recovery within 1 hour.
Fever in febrile convulsions may occur when?
any time during or after a seizure
majority febrile seizures occur within 24hrs of fever onset
Febrile convulsions differential diagnosis?
- CNS infection= bacterial or viral meningitis or encephalitis
- rigors or delirium (acute confusional state)
- shivering (may occur with or without fever)
- febrile myoclonus
- syncope
- breath-holding spells or reflex anoxic seizures
- head injury
- hypoglycaemia or metabolic disorders eg. mitochondrial cytopathy
- drug withdrawl or use
- epilepsy
- epilsepy syndromes eg. Dravet syndrome (severe myoclonic epilepsy of infancy); Genetic epilepsy with febrile seizures plus (GEFS+)
- other neuro conditions eg. cerebral palsy; Sturge-Weber syndrome; tuberous sclerosis; neurofibromatosis
Febrile myoclonus?
a benign disorder causing myoclonic jerks usually involving the upper limbs during fever. They may last from 15 minutes to several hours.
Breath-holding spells or reflex anoxic seizures?
brief, involuntary cessation of breathing often triggered by sudden, unexpected fright, fear, or pain. May present with pallor or cyanosis and low tone, with possible loss of consciousness and transient tonic clonic movements if the apnoea is prolonged.
Acute Mx for febrile convulsions?
- monitor duration
- protect from injury= cushion head, remove harmful objects from nearby
- do not restrain child
- once stopped, put on side in recovery position
- observe until recovered
- don’t give food or drink until fully recovered
- check for injuries
Acute Mx for febrile convulsions if tonic-clonic movements last for >5mins?
- call ambulance or
- give emergency benzo rescue med if advised by specialist= buccal midazolam or rectal diazepam
Is the intermittent use of antipyretics eg. paracetamol and/or ibuprofen at onset of fever advised to prevent febrile convulsions?
not recommended as does not reduce or prevent febrile seizure recurrence
only give to reduce fever if child uncomfortable or distressed and to prevent dehydration
Febrile convulsions typically occur in children of what age?
6m-5yrs
Simple vs complex febrile convulsion vs febrile status epilepticus?
simple= <15mins; generalised seizure; no recurrence within 24hrs; complete recovery within 1hr
complex= 15-30mins; focal seizure; may have repeat seizures within 24hrs
FSE= >30mins
Mx for febrile convulsions?
admit to paeds if 1st seizure or any features of complex seizure
phone ambulance if >5mins
recurrent= buccal midazolam or rectal diazepam
RFs for further seizures in febrile convulsions?
the overall risk of further febrile convulsion = 1 in 3.
- age of onset < 18 months
- fever < 39ºC
- shorter duration of fever before the seizure
- a family history of febrile convulsions
Febrile convulsions link to epilepsy?
risk factors for developing epilepsy include a family history of epilepsy, having complex febrile seizures and a background of neurodevelopmental disorder
children with no risk factors have a 2.5% risk of
developing epilepsy
if children have all 3 features the risk of developing epilepsy is much higher (e.g. 50%)
Migraine?
common primary headache disorder characterised by attacks of moderate or severe headache and associated symptoms eg. photophobia, phonophobia, nausea, vomiting
Migraine features?
- unilateral headache, pulsating or throbbing
- lasts 4-72hrs
- photophobia, phonophobia, N & V
- with or without aura
- episodic or chronic
- aggravated by or cause avoidance of routine activities of daily life eg. walking
Aura in migraine?
transient focal neuro symptoms eg. visual: fortification spectra and/or scotoma, speach disturbance or sensory symptoms: paraesthesia
usually precede or accompany the headache
Episodic migraine?
migraine occurs on fewer than 15d per m
Chronic migraine?
headache occurring at least 15d per m (features of headache on at least 8d per m) for more than 3m
Migraine more common in women or men?
women
Prognosis of migraine?
usually improves with increasing age
pregnancy= improvement in freq and severity of attacks
What may be useful to do to identify potential triggers and monitor effectiveness of Tx in migraines?
headache diary
Triggers of migraine?
Cheese
Chocolate
Oral contraceptive pill
Caffiene
Alcohol
Anxiety
Travel
Exercise
Stress
Dehydration
Missed meals
Disturbed sleep
Mx of acute migraines?
- trigger avoidance and lifestyle changes: hydration, sleep, stress Mx, exercise, regular meals
- headache diary
- simple analgesia: paracetamol, NSAIDs
- triptan
- NSAID + triptan
- may add anti-emetic (prochlorperazine or metoclopramide) even in absence of N or V
Preventative Tx of migraine if not pregnant or breastfeeding?
propranolol (80-160mg daily divided doses)
or topiramate (50-100mg daily in divided doses- CONTRAINDICATED in PREG)
When may preventative Tx be needed for migraines?
- frequent or prolonged and severe despite acute Tx
- risk of medication overuse headache
do NOT initiate in primary care if child, pregnant or breastfeeding
Admission or referral is indicated for migraines when?
- serious cause suspected
- severe, uncontrolled status migrainosus (>72hrs)
- Cx has developed
- atypical symptoms
- uncertain
- Tx failed
- considering preventative Tx in children or pregnant/breastfeeding
How long does the headache of migraines last?
4-72hrs adults
2-72hrs adolescents
Migraine is a headache with what?
at least 2 of:
- unilateral (bilateral in children)
- pulsating, throbbing or banging
- moderate/severe pain
- aggravated by routine ADLs eg. walking, climbing stairs
Headache with associated symptoms, at least one of:
- N +/or V
- photophobia and phonophobia
Migraine without aura can be diagnosed in pt with what? (diagnostic criteria)
at least 5 attacks fulfilling criteria:
- lasts 4-72hrs
at least 2 of:
- unilateral (bilateral in children)
- pulsating, throbbing or banging
- moderate/severe pain
- aggravated by routine ADLs eg. walking, climbing stairs
Headache with associated symptoms, at least one of:
- N +/or V
- photophobia and phonophobia
Not attributed to another disorder
Headache with aura can be diagnosed in pt with what? (diagnostic criteria)
at least 2 attacks fulfilling criteria:
1 or more fully reversible aura symptoms:
- visual (most common)= fortification spectra
- sensory= unilateral paraesthesia or numbness
- speach/language= dysphasia
at least 3 of:
- at least 1 aura symptoms spreads gradually over at least 5 mins
- 2+ aura occur in succession
- each aura symptom lasts 5-60mins
- one aura symptom unilateral
- one aura symptom is positive (eg. scintillations or paraesthesia)
- aura accompanied or followed within 60mins by headache
Not attributed to another disorder
Aura in migraines types?
- visual (most common)= fortification spectra
- sensory= unilateral paraesthesia or numbness
- speach/language= dysphasia
Aura in migraine features?
at least 2 attacks fulfilling criteria:
1 or more fully reversible aura symptoms:
- visual (most common)= fortification spectra
- sensory= unilateral paraesthesia or numbness
- speach/language= dysphasia
at least 3 of:
- at least 1 aura symptoms spreads gradually over at least 5 mins
- 2+ aura occur in succession
- each aura symptom lasts 5-60mins
- one aura symptom unilateral
- one aura symptom is positive (eg. scintillations or paraesthesia)
- aura accompanied or followed within 60mins by headache
Not attributed to another disorder
Atypical aura in migraine?
- motor, brainstem or retinal aura
- admission or urgent specialist advice for atypical aura= motor weakness, double vision, vision symptoms affecting only one eye, poor balance, decreased level of consciousness
Migraine: aura without headache?
can occur particularly in older people.
The absence of headache makes the exclusion of other causes (such as transient ischaemic attack) difficult.
Ix is often indicated, especially if aura without migraine headache occurs for the first time after 40 years of age and symptoms are exclusively negative (for example, hemianopia) or if aura is prolonged or very short.
Prodromal and postdromal symptoms of migraine?
- prodromal= fatigue, poor conc, neck stiffness, yawning; may occur hrs or 1-2d before onset of other migraine symptoms
- postdromal= fatigue, elevated or depressed mood; may occur after resolution of headache and last up to 48hrs
Suspect mestrual-related migraine in who?
Women/girls with migraine occurring predominantly between 2 days before and 3 days after the start of menstruation for at least 2 out of 3 consecutive menstrual cycles.
Types of migraine? (7)
- episodic or chonic
- with aura
- without aura
- atypical aura
- aura without headache
- ?prodromal and postdromal symptoms
- mentrual-related migraine
Migraine differential diagnosis?
- tension type headache
- trigeminal autonomic cephaligias eg. cluster headache, paroxysmal hemicranias
- other primary headache disorders: primary cough headache, cold-stimulus headache
- trauma or injury to head/neck
- intracerebral haemorrhage
- central venous thrombosis
- GCA
- TIA
- idiopathic intracranial HTN
- cerebral neoplasm
- exposure or withdrawl from CO, alcohol, cocaine
- infection: meningitis, encephalitis
- hypoxia, HTN, pre-eclampsia
- somatisation disorder
- trigeminal neuralgia
- post-herpetic neuralgia
- optic neuritis
Medication overuse headache (MOH) common in people with what?
migraine
How to avoid medication overuse headache?
restrict acute medication to max of 2d per week
When can medication overuse headache (MOH) occur?
with 15 or more days per month use of simple analgesics (such as aspirin, ibuprofen and paracetamol) or 10 or more days use per month of triptans or combination analgesics.
How to manage medication overuse headache?
advise abrupt cessation of all overused simple analgesics and triptans for at least 1 month, and explain the potential for headache symptoms to worsen in the short term before they improve.
Ensure women who have migraine with aura are not using what?
COCP
Medication for migraines?
- ibuprofen (400mg) or paracetamol (1000mg)
or
- triptan= sumatriptan oral 50-100mg
or
- triptan + NSAID with long half life (naproxen)
condisider:
+ metoclopramide 10mg or prochlorperazine 10mg even in absence N&V
Advise for pt taking medication for migraine?
Acute medication should be taken early while pain is mild.
If they have aura, triptans should be taken at the start of the headache and not at the start of the aura (unless the aura and headache start simultaneously).
Treatments which have not been effective 2 hours after use are unlikely to be effective for treating the attack, in such cases higher doses, where appropriate, or alternative simple analgesics or combination treatments should be considered.
Can u offer opioids for migraine?
NO
Follow up for pt with migraine?
2-8w after starting Tx
Consider what Tx to prevent menstrual-related migraine?
Frovatriptan (2.5 mg twice daily) on the days migraine is expected or from two days before until three days after bleeding starts.
Zolmitriptan (2.5 mg twice or three times daily) on the days migraine is expected or from two days before until three days after bleeding starts.
Tx for migraine in children?
- NSAIDs or paracetamol
- NOT ASPIRIN
- nasal triptans if uneffective as oral contraindicated in <18yrs
Tx for migraines in pregnant or breastfeeding women?
paracetamol 1st line
consider ibuprofen if <20w gestation
sumatriptan but seek specialist advice if unsure
Why should NSAIDs be avoided after 20w gestational age in pregnant women (eg. for migraine Mx)?
risk of premature closure of ductus arteriosus and oligohydramnios
Primary headache?
not associated with an underlying condition, includes migraine, tension-type headache, and trigeminal autonomic cephalgias (including cluster headache).
Secondary headache?
precipitated by another condition or disorder (local or systemic), such as head or neck trauma; cranial or cervical vascular disorders including stroke and temporal arteritis; central nervous system (CNS) malignancy; exposure to or withdrawal from a substance including medication overuse headache; infection (intracranial or systemic); disorders of facial or cranial structures, such as the eyes, ears, sinuses, or teeth; and psychiatric disorders.
Categorisation of headaches?
1) primary
2) secondary
3) Painful cranial neuropathies, other facial pains, and other headaches including trigeminal neuralgia and post-herpetic neuralgia.
Red flags in headaches?
- new severe or unexpected headache= thunderclap reaching max intensity within 5mins may be subarachnoid haermorrhae
- progressive or persitent headache, or changed dramatically
- new onset headache in pt >50= temporal arteritis or space occupying lesion
- associated features= neck pain/stiffness; fever; impaired consciousness; papilloedema; new onset focal neuro deficit; atypical aura; dizziness; V; visual disturbances
- contacts with similar symptoms eg. CO poisoning
- precipitating factors eg. recent trauma; triggered by valsalva maneouvre; worse on standing; worse lying down
- immunocompromised
- malignancy
- current or recent preg= ?pre-eclampsia
Diagnostic criteria for headaches?
ICHD
Diagnostic criteria for tension-type headache?
Recurrent episodes of headache lasting from 30 minutes to 7 days which are not associated with nausea or vomiting. The headache may also be associated with no more than one of photophobia or phonophobia, and
The headache has at least two of:
- Bilateral location.
- Pressing, tightening, non-pulsating quality.
- Mild or moderate intensity.
- Not aggravated by routine physical activity (such as walking or climbing stairs).
Not attributed to another disorder
Diagnostic criteria for cluster headache?
At least five attacks of severe or very severe unilateral orbital, supraorbital, and/or temporal pain lasting 15–180 minutes (untreated), and
The headache is associated with a sense of restlessness or agitation and/or at least one of the following ipsilateral to the headache:
- Conjunctival injection and/or lacrimation.
- Nasal congestion and/or rhinorrhoea.
- Eyelid swelling.
- Forehead and facial sweating.
- Forehead and facial flushing.
- Sensation of fullness in the ear.
- Miosis (excessive pupillary constriction) and/or ptosis.
Attacks occur between one every other day and eight per day for more than half of the time when the disorder is active. Note: the British Association for the Study of Headache (BASH) guideline states a frequency of 1–3 attacks per day (up to 8) and usually occur daily for 2–3 months at a time
Not attributed to another disorder
Diagnostic criteria for medication overuse headache?
Headache occurring on at least 15 days per month for at least 3 months, with a pre-existing headache disorder (typically a predisposition to migraine and/or tension-type headache).
Regularly overused, for more than 3 months, one or more drugs that can be taken for acute and/or symptomatic treatment of headache such as ergotamines, triptans, simple analgesics, or opioids.
The BASH guideline states ergotamines, triptans, combination analgesics, or opioids taken on 10 or more days per month, or 15 days for simple analgesics such as paracetamol, aspirin, or a nonsteroidal anti-inflammatory drug (NSAID), for more than 3 months
Not attributed to another disorder
Headache triggered by Valsalva manoeuvre eg. coughing, sneezing, bending, exertion?
consider a Chiari malformation type 1 (a herniation of the cerebellar tonsils), a posterior fossa lesion, or other space-occupying lesions.
Headache worsens on standing?
?CSF leak
Headache worsens on lying down?
consider a space-occupying lesion or cerebral venous sinus thrombosis.
Headache preceding recent head trauma (within past 3m)?
consider subacute or chronic subdural haematoma.
Patients characteristically do what when they experience migraine?
go to dark quiet room
Summary of typical aura in migraine?
are visual, progressive, last 5-60 minutes and are characterised by transient hemianopic disturbance or a spreading scintillating scotoma
Hemiplegic migraine?
a variant of migraine in which motor weakness is a manifestation of aura in at least some attacks
around half of patients have a strong family history
very rare - estimated prevalence is 0.01% (i.e. around only 1 in 1,000 migraine patients), more common in adolescent females
How are migraines typically different in children?
commonly bilateral, shorter lasting, GI disturbance more prominent
Migraine: auras may occur with or without headache and…
1) are fully reversible
2) develop over at least 5 minutes
3) last 5-60 minutes
Migraine with motor weakness, double vision, visual symptoms affecting only one eye, poor balance, decreased level of consciousness?
aura symptoms are atypical and may prompt further investigation/referral
Why should caution be used when prescribing metoclopramide to young pts with migraines?
as acute dystonic reactions may develop
General rule for migraine Tx? (in terms of drug class used)
5-HT receptor agonists are used in the acute treatment of migraine whilst 5-HT receptor antagonists are used in prophylaxis.
1st line Tx of acute migraine?
oral triptan and NSAID
nasal triptan if 12-17yrs
Motor neurone disease?
neuro condition of unknown cause which can present with both UMN and LMN signs
Age MND commonly presents?
rare before 40yrs
Patterns of MND?
- amytrophic lateral sclerosis (ALS) (50% pts)
- progressive muscular atrophy
- progressive bulbar palsy
- primary lateral sclerosis
sometimes a combination of patterns in pts
Features of MND?
- LMN AND UMN signs
- asymmetric limb weakness
- fasiculations
- wasting of small hand muscles/tibialis anterior
- absence of sensory signs/symptoms= vague sensory symptoms may occur early in disease (eg. limb pain) but never sensory signs
- does not affect external ocular muscles
- no cerebellar signs
- abdominal reflexes usually preserved and sphincter dysfunction if present is late feature
What is not affected in MND?
- absence of sensory signs/symptoms= vague sensory symptoms may occur early in disease (eg. limb pain) but never sensory signs
- does not affect external ocular muscles
- no cerebellar signs
- abdominal reflexes usually preserved and sphincter dysfunction if present is late feature
LMN and UMN signs, fasiculations, asymmetric limb weakness and wasting of tibialis anterior/small hand muscles?
MND
most common presentation of ALS (MND)?
asymmetric limb weakness
Motor neurones affected in MND?
UMN AND LMN
Diagnosis of MND?
- clinical
- nerve conduction studies show normal motor conduction and exclude neuropathy
- Electromyography= reduced number of action potentials with increased amplitude
- MRI= excludes cervical cord compression and myelopathy
What does electromyography in MND show?
reduced number of action potentials with increased amplitude
Mx of MND?
- Riluzole
- non-invasive ventilation (BIPAP) at night (survival benefit of 7m)
- PEG tube to support nutrition
Prognosis for MND?
poor, 50% die within 3yrs
What does PEG tube stand for?
percutaneous gastrostomy tube
Riluzole for MND?
prevents stimulation of glutamate receptors
used mainly in ALS
prolongs life by about 3m
Drug used in MND?
riluzole
MND types: what does ALS stand for?
amyotrophic lateral sclerosis
Most common type of MND?
ALS
MND types: amyotrophic lateral sclerosis (ALS)?
typically LMN signs in arms and UMN signs in legs
in familial cases the gene responsible lies on chromosome 21 and codes for superoxide dismutase
MND types: primary lateral sclerosis?
UMN signs only
MND types: progressive muscular atrophy?
LMN signs only
affects distal muscles before proximal
carries best prognosis
MND types: progressive bulbar palsy?
palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei
carries worst prognosis
What type of MND has worst prognosis?
progressive bulbar palsy
What type of MND has best prognosis?
progressive muscular atrophy
What type of MND has typically LMN signs in arms and UMN signs in legs?
ALS
What type of MND has UMN signs only?
primary lateral sclerosis
What type of MND has LMN signs only and affects distal muscles before proximal?
progressive muscular atrophy
Multiple sclerosis (MS)?
acquired immune-mediated inflam condition of the CNS resulting in areas of demyelination, gliosis and secondary neuronal damage throughout the CNS
lesions disseminated in time and space
When does MS first develop?
young adults
most common non-traumatic cause of signif neuro disability in people <40yrs
3 Main patterns of disease in MS?
- relapsing-remitting MS (RRMS)
- secondary progressive MS (SPMS)
- primary progressive MS (PPMS)
Most common pattern of MS?
relapsing remitting
MS disease patterns: relapsing-remitting?
Episodes of symptoms (relapses) are followed by recovery (remissions) and periods of stability. Typically, after several relapses residual damage to parts of the CNS remains resulting in only partial recovery during remissions.
most common pattern.
MS disease patterns: secondary progressive?
occurs when there is a gradual accumulation of disability unrelated to relapses, which become less frequent or stop completely. About two thirds of people with RRMS progress to SPMS.
MS disease patterns: primary progressive?
in PPMS there is a steady gradual worsening of the disease from the onset, without remissions. This occurs in about 10–15% of people with MS.
Cause of MS?
unknown
though that acute then chronic immune-mediated inflammation is precipitated by an abnormal response to environmental triggers in people who are genetically predisposed.
RFs that may contribute to MS development?
genetic factors, vitamin D deficiency, infection, geographical location (extremes of latitude), smoking, obesity during adolescence, and female gender.
4 most common presentations of MS?
1) optic neuritis
2) transverse myelitis
3) cerebellar-related symptoms
4) brainstem syndromes
History of relapsing remitting MS?
unpredictable
severity and freq of relapses vary greatly, can take time to progress to SPMS and to signif permanent diability
What may reduce the number and severity of relapses and delay disability progression in MS?
disease-modifying therapies (DMTs)
Ix if suspect MS?
- prompt referral to neuro consultant
- bloods to exclude alternative cause
What to do if pt with MS is suspected of having a relapse?
- rule out infection: UTI and resp
- fluctuations in disease, progression and other conditions unrelated to MS that may present with similar features should be considered
- contact MS team to discuss Mx
Mx of MS relapse?
oral methylprednisolone 0.5g daily for 5d
may shorten the length and severity of relapse
How often should pt with MS be reviewed in secondary care?
at least once per yr
Cx of MS?
fatigue, spasticity, ataxia, tremor, mobility problems, visual problems, pain, bladder problems, sexual problems, and mental health problems
Where do MS lesions develop?
almost anywhere in CNS so can get various Cx
Age MS typically presents?
20-50yrs
older age at onset is associated with progressive course
How long may MS symptoms last?
may evolve over more than 24hrs, may persist over several days or weeks then improve
Optic neuritis in MS?
partial or total unilateral visual loss developing over a few days, pain behind the eye (in particular on eye movement) and/or loss of colour discrimination (particularly reds)
Bilateral optic neuritis in MS?
may occur but if it does need to rule out neuromyelitis optica which needs urgent Tx and is confused with MS
Examination of optic neuritis in MS?
Fundoscopy is often normal but the disc may appear pale or swollen. There may be paradoxical dilation of the pupil when light is rapidly shifted from the unaffected eye to the affected eye (relative afferent pupillary defect).
What is transverse myelitis in MS?
focal inflam within spinal cord
How may transverse myelitis present in MS?
sensory symptoms (such as paraesthesia) or motor symptoms (such as weakness) below the level of the inflammation that typically develop over hours or days.
Some people describe a tight band sensation around the trunk at the level of the inflammation, or a shock-like sensation radiating down the spine induced by neck flexion (Lhermitte’s phenomena).
There may be urinary symptoms such as urgency, frequency, or retention.
symmetrical or asymmetrical= reflect a partial myelitis that only affects part of spinal cord; full spinal cord transection affected is rare
What may examination reveal in pt with MS with transverse myelitis?
focal muscle weakness and reduced sensation below the affected spinal level. Muscle tone is initially reduced.
Cerebellar-related symptoms in MS?
ataxia, vertigo, clumsiness, and dysmetria (as demonstrated by abnormalities with finger-to-nose testing and walking heel to toe).
Brainstem syndromes in MS?
Ataxia.
Eye movement abnormalities that can cause diplopia, oscillopsia (a sensation of movement of the vision), nystagmus, and internuclear ophthalmoplegia (inability to adduct one eye and nystagmus in the abducting eye on oculomotor examination).
Bulbar muscle problems resulting in dysarthria or dysphagia.
Signs and symptoms of MS vary greatly and may include?
Loss or reduction of vision in one eye with painful eye movements.
Diplopia.
Ascending sensory disturbance and/or weakness.
Balance or gait problems, unsteadiness, or clumsiness.
Altered sensation radiating down the back and sometimes into the limbs on neck flexion (Lhermitte’s symptom).
2 phenomenon in MS?
Uhthoff’s= worsening of symptoms when body’s core temp increases eg. hot shower, hot weather, exercise, fever, sauna
L’hermitte’s= shock like sensation radiating down spine induced by neck flexion
Uhthoff’s phenomenon?
seen in MS
worsening of symptoms when body’s core temp increases eg. hot shower, hot weather, exercise, fever, sauna
L’hermitte’s sign?
seen in MS
shock like sensation radiating down spine induced by neck flexion
Criteria used to diagnose MS?
McDonald criteria
Diagnosing MS?
- Confirm that episodes are consistent with an inflammatory process.
- Excluding alternative diagnoses.
- Establishing that lesions have developed at different times and are in different anatomical locations for a diagnosis of relapsing-remitting MS.
- Establishing progressive neurological deterioration over at least 1 year for a diagnosis of primary progressive MS.
What type of disease is MS?
demyelinating
Differential diagnosis for MS?
- other demyelinating disease= neuromyelitis optica; idiopathic transverse myelitis; acute disseminated encephalomyelitis
- Metabolic disorders= B12 def; diabetic peripheral neuropathy, hypocalcaemia, hypothyoridism; copper def; zinc toxicity; adult onset leukodystrophies
- Infection= lyme disease; tertiary syphilis; HIV
- Vascular= primary CNS vasculitis; ischaemic stroke
- systemic inflam disorders= SLE; Behcet’s; sarcoidosis
- neoplasia= primary or metastatic brain lesions; paraneoplastic syndromes
MS in pregnancy?
- relapse rates may reduce during preg and may increase 3-6m postpartum before back to normal
- doesn’t increase risk of progression
- vit D before conception and during preg
- some DMTs contraindicated in preg and breastfeeding
- UTI more frequent in preg
- increased risk of postpartum depression
When to consider hospital admission for MS relapse?
- severe
- DM or mental health needs monitoring
- oral steroids failed
- difficult to meet care needs
Does pt with MS have supply of steroids at home to manage future relapses?
no
each relapse needs discussed with specialist team and may need change in therapy
Info to tell pt with MS about relapses?
A relapse may have short-term effects on cognitive function.
Significant recovery can be expected within 2–3 months, but improvement can continue for up to 12 months.
Some residual disability occurs following 30–50% of all relapses, and is more likely to occur if the relapse is severe.
Drug to manage spasticity in MS?
baclofen
also physio important
Drug to manage oscillopsia (visual fields appear to oscillate) in MS?
gabapentin (off-label)
Chronic cell-mediated autoimmune disorder characterised by demyleination in CNS?
MS
Genetics in MS?
monozygotic twin concordance = 30%
dizygotic twin concordance = 2%
How long do acute attacks typically last in relapsing-remitting MS?
1-2m
around 65% of patients with relapsing-remitting MS go on to develop what within 15 years of diagnosis
secondary progressive disease
Gait and bladder disorders generally seen in what type of MS?
Secondary progressive disease
What type of MS is more common in older pts?
Primary progressive disease
75% pts with MS have what symptom?
significant lethergy
How can diagnosis of MS be made?
on the basis of two or more relapses and either objective clinical evidence of two or more lesions or objective clinical evidence of one lesion together with reasonable historical evidence of a previous relapse.
Visual features of MS?
optic neuritis: common presenting feature
optic atrophy
Uhthoff’s phenomenon: worsening of vision following rise in body temperature
internuclear (usually just worsening of fatigue, pain, vision, wekaness)
ophthalmoplegia
Sensory features of MS?
pins/needles
numbness
trigeminal neuralgia
Lhermitte’s syndrome: paraesthesiae in limbs on neck flexion
Motor features of MS?
spastic weakness: most commonly seen in the legs
Cerebellar features of MS?
ataxia: more often seen during an acute relapse than as a presenting symptom
tremor
Other features of MS?
urinary incontinence
sexual dysfunction
intellectual deterioration
What does diagnosis of MS require?
demonstration of lesions disseminated in time and space
Specialist Ix for MS?
- MRI
- CSF
- Visual evoked potentials- delayed but well preserved waveform
What does MRI in MS show?
high signal T2 lesions
periventricular plaques
Dawson fingers: often seen on FLAIR images - hyperintense lesions penpendicular to the corpus callosum
MRI finding= Dawson fingers: often seen on FLAIR images - hyperintense lesions penpendicular to the corpus callosum?
MS
What does CSF in MS show?
oligoclonal bands (and not in serum)
increased intrathecal synthesis of IgG
Oligoclonal bands in CSF?
MS
What does Tx of MS focus on?
reducing frequency and duration of relapses, there is no cure
Acute relapse of MS Mx?
high dose steroids= oral or IV methylprednisolone 0.5g daily 5d to shorten length of relapse
don’t alter degree of recovery (whether pt returns to baseline)
Indications for disease modifying drugs in MS?
relapsing-remitting disease + 2 relapses in past 2 years + able to walk 100m unaided
secondary progressive disease + 2 relapses in past 2 years + able to walk 10m (aided or unaided)
Role of disease modifying drugs in MS?
reduce risk of relapse
Drug used to reduce risk of relapse in MS?
natalizumab
Natalizumab for MS?
a recombinant monoclonal antibody that antagonises alpha-4 beta-1-integrin found on the surface of leucocytes
inhibit migration of leucocytes across the endothelium across the blood-brain barrier
generally considered to have the strongest evidence base for preventing relapse of the disease-modifying and hence is often used first-line
given IV
Summary of Mx of MS?
Acute relapse= oral or IV methylprednisolone 0.5g daily 5d to shorten relapse
Disease modifying drugs to reduce risk of relapse= natalizumab
Becker muscular dystrophy?
x-linked recessive dystrophinopathy
‘less severe’ version of Duchenne muscular dystrophy
Dystrophinopathy? (muscular dystrophy)
group of X-linked muscle diseases caused by mutations in the DMD gene, which encodes the dystrophin protein found in muscle
gradually cause the muscles to weaken, leading to an increasing level of disability.
mainly inherited by males
Types of dystrophinopathy? (muscular dystrophy)
- Becker muscular dystrophy
- Duchenne muscular dystrophy
- Myotonic dystrophy
Genetics of muscular dystrophy?
due to mutation in the gene encoding dystrophin, dystrophin gene on Xp21
dystrophin is part of a large membrane associated protein in muscle which connects the muscle membrane to actin, part of the muscle cytoskeleton
in Duchenne muscular dystrophy there is a frameshift mutation resulting in one or both of the binding sites are lost leading to a severe form
in Becker muscular dystrophy there is a non-frameshift insertion in the dystrophin gene resulting in both binding sites being preserved leading to a milder form
Features of Becker muscular dystrophy?
develops after the age of 10 years
less common than Duchenne and less severe
difficulty walking, climbing stairs, and getting up from the floor, muscle cramps during exercise, and learning to walk later than usual. Other symptoms include breathing problems, fatigue, loss of balance and coordination, and cognitive problems
intellectual impairment much less common than in Duchenne
Duchenne muscular dystrophy?
X-linked recessive inherited disorder in the dystrophin genes required for normal muscular function.
Gower’s sign?
child uses arms to stand up from a squatted position
Duchenne muscular dystrophy
Features of Duchenne muscular dystrophy?
progressive proximal muscle weakness from 5 years
calf pseudohypertrophy
Gower’s sign: child uses arms to stand up from a squatted position
30% of patients have intellectual impairment
More severe type of muscular dystrophy?
Duchenne
Ix for Duchenne muscular dystrophy?
- raised creatinine kinase
- genetic testing has now replaced muscle biopsy as the way to obtain a definitive diagnosis
Mx for Duchenne muscular dystrophy?
largely supportive as unfortunately there is currently no effective treatment
Prognosis of Duchenne muscular dystrophy?
most children cannot walk by the age of 12 years
patients typically survive to around the age of 25-30 years
What condition is Duchenne muscular dystrophy associated with?
dilated cardiomyopathy
Muscular dystrophy (MD)?
a group of genetic diseases that cause progressive weakness and degeneration of skeletal muscles
Myotonic dystrophy (dystrophia myotonica)?
inherited myopathy with features developing at around 20-30 years old. It affects skeletal, cardiac and smooth muscle.
2 main types of Myotonic dystrophy?
DM1 and DM2
Differences between the 2 types of myotonic dystrophy= DM1 and DM2?
DM1= DMPK gene on chromosome 19; Distal weakness more prominent
DM2= ZNF9 gene on chromosome 3; Proximal weakness more prominent; Severe congenital form not seen
Genetics of myotonic dystrophy?
autosomal dominant
a trinucleotide repeat disorder
DM1 is caused by a CTG repeat at the end of the DMPK (Dystrophia Myotonica-Protein Kinase) gene on chromosome 19
DM2 is caused by a repeat expansion of the ZNF9 gene on chromosome 3
Main features of myotonic dystrophy?
myotonic facies (long, ‘haggard’ appearance)
frontal balding
bilateral ptosis
cataracts
dysarthria
myotonic facies (long, ‘haggard’ appearance)
frontal balding
bilateral ptosis
cataracts
dysarthria
Myotonic dystrophy
Features of myotonic dystrophy?
myotonic facies (long, ‘haggard’ appearance)
frontal balding
bilateral ptosis
cataracts
dysarthria
myotonia (tonic spasm of muscle)
weakness of arms and legs (distal initially)
mild mental impairment
diabetes mellitus
testicular atrophy
cardiac involvement: heart block, cardiomyopathy
dysphagia
Myasthenia gravis?
autoimmune disorder resulting in insufficient functioning acetylcholine receptors
What gender is myasthenia gravis more common in?
women
85-90% cases of myasthenia gravis have what antibodies?
antibodies to acetylcholine receptors
less common to have antibodies in disease limited to ocular muscles
Key features of myasthenia gravis?
muscle fatigability= muscles become progressively weaker during periods of activity and slowly improve after periods of rest
extraocular muscle weakness: diplopia
proximal muscle weakness: face, neck, limb girdle
ptosis
dysphagia
In myasthenia gravis, when does muscle fatigability (and so weakness) worsen and improve?
worsens during periods of activity and slowly improves after periods of rest
Extraocular and proximal muscle weakness, dysphagia and ptosis?
myasthenia gravis
Association with myasthenia gravis?
thymomas in 15%
autoimmune disorders= pernicious anaemia, thyroid disorder, SLE, rheymatoid
thymic hyperplasia in 50-70%
What neuro condition is associated with thymoma?
myasthenia gravis
Ix for myasthenia gravis?
- single fibre electromyography
- CT thorax to exclude thymoma
- CK normal
- antibodies to acetylcholine receptors (85-90%); if negative then half +ve for anti-muscle-specific tyrosine kinase antibodies
Mx for myasthenia gravis?
1st line= pyridostigmine
eventually need immunosupression= pred initially then azathioprine, cyclosporine, mycophenolate mofetil may also be used
thymectomy
Mx of myasthenic crisis?
plasmapheresis
IVIG
may need BiPAP (non-invasive ventilation) or mechanical ventilation
Thymoma?
thymus gland tumour
Pathophysiology in myasthenia gravis?
normal= axon release neurotransmitter acetylcholine from presynaptic membrane, this attaches to receptors on postsynaptic membrane stimulating muscle contraction
MG= acetylecholine receptor (AChR) antibodies block to postsynaptic receptors, blocking them and preventing stimulation by acetylcholine. The more the receptors are used during muscle activity, the more they become blocked. Less effective stumulation of muscles with increased activity. With rest, receptors are cleared so symptoms improve.
Antibodies can also activate the complement system within neuromusc junction, leading to cell damage at postsynaptic membrane, worsening symptoms.
Antibodies that can cause myasthenia gravis?
1) acetylcholine receptor antibodies
2) muscle-specific kinase (MuSK) antibodies
3) low-density lipoprotein receptor-related protein 4 (LRP4) antibodies
MuSK and LPR4 important proteins to create acetylcholine receptor so destruction of these proteins with antibodies leads to inadequate receptors.
Symptoms in MG affect where most commonly?
proximal limb mucles
small muscles of head and neck
CP of MG?
- fatigue in jaw when chewing
- slurred speech
- diff climbing stairs, standing from seat or raising hands above head
- diplopioa (extraocular weakness)
- ptosis (eyelid weakness)
- weakness in facial movements
- difficulty swallowing
Examinations in MG to elicit fatiguability in muscles?
- repeated blinking= exacerbate ptosis
- prolonged upward gaze= exacerbate diplopia
- repeated abduction of one arm 20x= unilateral weakness
- thymectomy scar?
- test FVC
Myasthenic crisis?
potentially life-threatening Cx of MG. Causes acute worsening of symptoms, often triggered by another illness eg. resp tract infection.
Resp muscle weakness can lead to resp failure
Drug for MG, its class and MOA?
pyroidostigmine
(long-acting acetylcholinesterase inhibitor)
prolongs the action of acetylcholine and improves symptoms
Hallmark feature in MG?
exertion resulting in muscle fatigability
What drugs may exacerbate myasthenia gravis?
penicillamine
quinidine, procainamide
beta-blockers
lithium
phenytoin
antibiotics: gentamicin, macrolides, quinolones, tetracyclines
Parkinson’s disease?
chronic, progressive neurodegenerative condition resulting from loss of dopamine-containing cells of substantia nigra pars compacta
Parkinsonism?
umbrella term for clinical syndrome invl bradykinesia and at least 1 of: tremor, rigidity and/or postural instability
Bradykinesia?
slowness of movement and speed
Most common form of parkinsonism?
parkinson’s disease
Are parkinson’s disease features unilateral or bilateral?
unilateral initially, may become bilateral as disease progresses
Causes of parkinsonism?
- parkinson’s disease
- drug-induced
- cerebrovascular disease
- Lewy body dementia
- multiple system atrophy
- progressive supranuclear palsy
Prognosis of Parkinson’s disease?
slowly progressive
pts with early-onset disease may have later onset of motor Cx and cognitive impairment
Cx of parkinson’s disease?
motor (usually related to use of anti-parkinsonism meds)= immobility, slowness, freezing of gait, motor fluctuations, falls, dyskinesia, communication difficulties
non-motor= depression, anxiety, impulse control disorders, psychotic symptoms, dementia, sleep disturbance, constipation, orthostatic hypotension and pain
Why should pt not suddenly stop anti-parkinsonian meds/miss a dose if they have parkinson’s disease?
may precipitate acute akinesia or neuroleptic malignant syndrome
Akinesia vs dyskinesia?
Akinesia= absence of movement; can present as no movement at all, delayed response or freezing mid action
Dyskinesia= involuntary muscle movement eg. tremor, sudden jerking (spastic movements)
Diagnosis of parkinson’s?
clinical
CP of parkinson’s disease?
gradual onset progressive…
1) bradykinesia OR
2) hypokinesia (poverty of movement
plus one of…
1) cogwheel rigidity or lead-pipe rigidity
2) resting tremor
3) postural instability and/or gait disorders
May have non-motor symptoms that precede motor by yrs= depression, anxiety, anosmia, cog impairment, sleep disturbance, constupation
Hypokinesia (poverty of movement) in parkinson’s disease?
reduced facial expression, reduced arm swing or blinking; difficulty with fine movements eg. buttoning clothes, slow/small handwritting (micrographia); slow shuffling, festinating gait (invol gait acceleration to regain balance), freezing gait
Bradykinesia in parkinsons disease?
slowness in initiation of voluntary movement with progressive reduction in speed and amplitude of sustained repetitive actions, such as finger or foot tapping
Cogwheel rigidity or lead-pipe rigidity in parkinson’s disease?
lead-pipe= constant resistance felt when a limb is passively flexed in the presence of increased tone without tremor
cogwheel= regular intermittent relaxation of tension felt when a limb is passively flexed in the presence of tremor and increased tone.
Tremor in parkinson’s?
- resting tremor
- 3-5 Hz
- worse when stressed or tired
- improves with voluntary movement, mental concentration and during sleep
- may affect distal muscles of thumb and index finger= pin-rolling; wrist or leg
- may affect lips, chin, jaw but rarely head, neck or voice
Tremor is absent in what % of pts with parkinson’s?
20% no tremor
Balance problems and/or gait disorders in parkinson’s disease?
postural instability= pull test (tendency to fall back after a sharp pull from examiner)
stooped posture
Non-motor symptoms of parkinsons?
depression, anxiety, fatigue, cognitive impairment, reduced sense of smell (anosmia), sleep disturbance, constipation
Summary of the main features in parkinson’s disease?
bradykinesia (slow initiation of movement) or hypokinesia (poverty of movement eg. reduced facial expression, micrographia, slow shuffling festinating gait, reduced arm swing, diff with fine movements like opening jars)
plus at least 1 of= cogwheel/lead-pipe rigidity; resting pin-rolling tremor improves on moving; postural instability
non-motor symptoms
What drugs may cause parkinsonism?
antispychotics (within 10w) esp 1st gen eg. haloperidol
- metoclopramide and prochloperazine
- SSRIs
- CCB
- amiodarone
- lithium
- cholinesterase inhibitors eg. donepezil or memantine
- sodium valproate
- methyldopa
Drug induced parkinsonism vs parkinson’s disease?
drug induced= rapid onset, bilateral motor symptoms; often no rigidity or resting tremor, may be action tremor
How is essential tremor different to parkinson’s tremor?
common; onset is at any age, and often there is a family history. Typically, the tremor is bilateral and symmetrical; may involve the whole hand; may worsen with stress, caffeine, and sleep deprivation; typically involves the head, neck, voice, and limbs; and often improves with alcohol and beta-blockers.
Differential diagnosis of parkinson’s?
- drug induced
- stroke, TIA
- alzheimers or dementia with lewy bodies
- progressive supranuclear palsy
- multiple system atrophy
- corticobasal degeneration
- Wilson’s disease
- repeated head injury
- essential tremor
- exaggerated physiological tremor
- dystonic tremor
- hyperthyroidism
- beta-2 agonisits
- cerebellar disorders= intention tremor
Progressive supranuclear palsy?
early dysphagia, gaze palsy, or recurrent falls
Multiple system atrophy?
severe early autonomic involvement such as postural hypotension or cerebellar ataxia
Corticobasal degeneration?
asymmetric rigidity and dystonia, with apraxia, progressive aphasia, and cognitive impairment
What to do if suspect parkinson’s disease?
urgent referral to neuro untreated
What if parkinson’s disease is suspected but pt is taking a drug known to induce parkinsonism?
reduce or stop drug in primary care if possible and appropriate
do not delay referral by waiting to assess symptom response
Who may be involved in care of pt with Parkinson’s?
DMT
SALT, physio, occupational, dietician, adult social care, bowel and bladder continence team, psych, community nursing
How often should pt with parkinson’s be reviewed?
every 6-12m
Motor symptoms/Cx related to use of anti-parkinsonian meds?
- deteriorating function
- loss of drug effect
- motor fluctuations: end-of-dose fading, on-off phenomenon
- dyskinesia
- freezing of gait
- falls
Mx of Parkinson’s disease?
(all 1st line Tx)
1) Levodopa, usually given with dopa decarboxylase inhibitor, as co-beneldopa or co-careldopa
or
2) oral monoamine oxidase-B (MOA-B) inhibitors= rasagiline, selegiline
or
3) oral dopamine agonists= ropinirole; or transdermal dopamine agonsit= rotigotine
May + adjuvant treatments
Co-careldopa for parkinson’s disease?
usually given in early stages
more improvement on motor symptoms, less adverse effects but may cause more motor Cx eg. dyskinesia than other drug classes
MOA-B inhibitors for parkinson’s disease eg. rasagiline?
less improvement in motor symptoms but fewer motor Cx and fewer adverse effects
Oral (ropinirole) or transdermal dopamine agonists (rotigotine) for parkinson’s disease?
less improvement in motor symptoms; fewer motor Cx but more adverse effects= XS sleepiness, hallucinations, impulse control disorders
transdermal good if swallowing problems
name 3 drugs that can be used 1st line to manage parkinson’s disease
Co-careldopa= levodopa + dopa decarboxylase inhibitor
Rasagiline= oral MOA-B inhibitor
Ropinirole= oral dopamine agonist (or rotigotine which is transdermal)
Adjuvant treatments in parkinson’s disease?
oral catechol-O-methyl transferase (COMT) inhibitors eg. antacapone= eg. if have dyskinesia depsite optimal levodopa therapy; adverse effects
oral amantadine= if dyskinesia still not managed adequately; adverse effects
subcut apomorphine (potent dopamine agonist)= advanced disease; adverse effects (N&V, ortho hypot)
Deep-brain stimulation surgery of the subthalamic nucleus= if advanced disease with motor Cx but fit and levodopa responsive.
How to manage N&V in parkinson’s?
take meds with food, often settles if due to meds (levodopa or dopamine agonist)
low-dose domperidone
DO NOT USE metoclopramide or prochlorperazine as can exacerbate parkinsonism
What causes Parkinson’s disease?
degeneration of dopaminergic neurones in the substantia nigra pars compacta
Triad of parkinsons?
bradykinesia, tremor, rigidity
Type of sleep disorder in parkinson’s?
REM sleep behaviour disorder
Example of autonomic dysfunction in parkinson’s?
postural hypotension
What test may be done if there is difficulty in differentiating between essential tremor and Parkinson’s disease?
SPECT (I-FP-CIT single photon emission computed tomography)
How to decide what 1st line meds to give pt with parkinson’s disease?
of motor symptoms affecting pts QOL= levodopa
not affecting QOL= dopamine agonist (non-ergot derived), levodopa or monoamine oxidase B (MAO-B) inhibitor
Parkinson’s disease= Levodopa:
1) motor symptoms
2) ADLs
3) motor Cx
4) adverse effects
1) more improvement on motor symptoms
2) more improvement in ADLs
3) more motor Cx
4) fewer adverse effects
Parkinson’s disease= dopamine agonists:
1) motor symptoms
2) ADLs
3) motor Cx
4) adverse effects
1) less improvement in motor symptoms
2) less improvement in ADLs
3) fewer motor Cx
4) more adverse effects= XS sleepiness, hallucinations and impulse control disorders
Parkinson’s disease= MAO-B inhibitors:
1) motor symptoms
2) ADLs
3) motor Cx
4) adverse effects
1) less improvement in motor symptoms
2) less improvement in ADLs
3) fewer motor Cx
4) less adverse effects
What parkinson’s meds have lower risk or higher risk of hallucinations?
higher risk= dopamine agonists
lower= MOA-B inhibitors, COMT inhibitors
What if pt with Parkinson’s continues to have symptoms despite optimal levodopa treatment or has developed dyskinesia?
adjuvant Tx: addition of a dopamine agonist, MAO-B inhibitor or catechol-O-methyl transferase (COMT) inhibitor
When is there a risk of acute akinesia or neuroleptic malignant syndrome in parkinson’s?
abruptly stop or miss dose of meds
or if not absorbed eg. hastroenteritis
What drugs for parkinson’s have an increased risk of impulse control disorders?
dopamine agonist therapy eg. ropinirole
Hx of previous impulsive behaviour
Hx of alcohol and/or smoking
XS daytime sleepiness in parkinson’s?
do not drive
adjust meds
if fail then consider Modafinil
Orthostatic hypotension in parkinson’s?
med review looking at potential causes
if persist then consider midodrine (acts on peripheral alpha-adrenergic receptors to increase arterial resistance)
How to manage drooling of saliva in parkinson’s?
consider glycopyrronium bromide
Parkinson’s: what should levodopa be combined with and why?
decraboxylase inhibitor (carbidopa or benserazide)= co-careldopa or co-beneldopa
prevents the peripheral metabolism of levodopa to dopamine outside of the brain and hence can reduce side effects
Common adverse effects of levodopa in parkinsons?
dry mouth
anorexia
palpitations
postural hypotension
psychosis
What are some adverse effects of levodopa due to in parkinsons?
end-of-dose wearing off= symptoms often worsen towards the end of dosage interval. This results in a decline of motor activity
‘on-off’ phenomenon= large variations in motor performance, with normal function during the ‘on’ period, and weakness and restricted mobility during the ‘off’ period
dyskinesias at peak dose= dystonia, chorea and athetosis (involuntary writhing movements)
these effects may worsen over time with - clinicians therefore may limit doses until necessary
Pt with parkinsons admitted to hospital, unable to take oral levodopa?
give dopamine agonist patch as rescue med to prevent acute dystonia
Adverse effects of dopamine receptor agonists?
impulse control disorders and excessive daytime somnolence
more likely than levodopa to cause hallucinations in older patients. Nasal congestion and postural hypotension are also seen in some patients
Mechanism of action of monoamine oxidase-B inhibitors eg. selegiline for parkinsons?
inhibits the breakdown of dopamine secreted by the dopaminergic neurons
Amantadine in parkinsons?
increases dopamine release and inhibits its uptake at dopaminergic synapses
side-effects include ataxia, slurred speech, confusion, dizziness and livedo reticularis
COMT inhibitors in parkinsons?
e.g. entacapone, tolcapone
COMT is an enzyme involved in the breakdown of dopamine, and hence may be used as an adjunct to levodopa therapy
used in conjunction with levodopa in patients with established PD
Antimuscarinics in parkinsons?
block cholinergic receptors
now used more to treat drug-induced parkinsonism rather than idiopathic Parkinson’s disease
help tremor and rigidity
e.g. procyclidine, benzotropine, trihexyphenidyl (benzhexol)
2 examples of brachial plexus injuries?
Erb-Duchenne paralysis
Klumpke’s paralysis
Erb-Duchenne paralysis?
brachial plexus injury
damage to C5, 6 roots
winged scapula
may be caused by breech presentation
Klumpke’s paralysis?
brachial plexus injury
damage to T1
loss of intrinsic hand muscles
due to traction
Carpal tunnel syndrome (CTS)?
entrapment neuropathy caused by compression of median nerve in the carpal tunnel at the wrist
Most common entrapment neuropathy of upper limb?
carpal tunnel syndrome
Pathophysiology behind carpal tunnel syndrome?
Reduction in the dimensions of the carpal tunnel or increase in the volume of its contents produce an intermittent or sustained high pressure in the tunnel, which causes ischaemia of the median nerve and impairs nerve conduction, leading to pain and paraesthesia in the distribution of the median nerve.
RFs for carpal tunnel?
majority idiopathic
- activities with high hand/wrist repetition rate
- obesity
- pregnancy
- osteoarthritis of MCP joint of thumb
- RA
- hypothyroidism
- DM
- lunate fracture
- oedema eg. HF
Signs of severe disease in carpal tunnel syndrome?
- wasting of thenar mesucles
- sensory loss of median nerve distribution
- reduced hand grip and pinch grip strength
Median nerve distribution?
thumb, index finger, middle finger, radial half of ring finger
Mx for carpal tunnel syndrome?
- may resolve spontaneously
- lifestyle= avoid repetitive hand/wrist movements; work-place assessment if appropriate, driving safety
- 6w trial of conservative Tx= nocturnal wrist splint (in neutral position); corticosteroid injection; hand exersises
- review after 6w if persistent
Specialist:
- nerve conduction studies (only if uncertain or considering surgery)
- surgical decompression
When should specialist referral be made for carpal tunnel syndrome?
- uncertain
- persistent symptoms
- progressive or severe (impacting daily function)
- recurrent or persistent following surgery
Nerve conduction studies for carpal tunnel syndrome?
specialist Mx
measure the strength and speed of impulses propagated down the length of a peripheral nerve, and may be useful to detect impaired median nerve conduction across the carpal tunnel, quantify the severity of disease, and provide a baseline for assessment of recovery
may also help to exclude other conditions, such as polyneuropathy and radiculopathy
CP for carpal tunnel syndrome?
- intermittent paraesthesia, numbness or altered sensation, and burning or pain in the distribution of the median nerve (the thumb, index finger, middle finger, and radial half of the ring finger)
- one or both hands
- worse at night, can wake from sleep
- loss of grip strength, weakness or reduced manual dexterity eg. when doing up buttons
- severe= persistent and neuro deficit (constant sensory deficit, or thenar muscle wasting and weakness)
Atypical symptoms of carpal tunnel syndrome?
sensory changes in all fingers, or with pain in the hand radiating up into the wrist, forearm, or shoulder.
Relieving factors for carpal tunnel syndrome?
changing hand posture or shaking the wrist (‘the flick sign’)
Signs in carpal tunnel syndrome which are more likely with prolonged or severe symptoms?
Trophic ulceration at the tips of the digits (rare, indicating loss of protective sensation).
Wasting of the thenar eminence muscles.
Sensory loss in the distribution of the median nerve.
Weakness of thumb abduction and opposition; reduced hand grip and pinch grip strength; reduced hand co-ordination.
Hand examination manoevures which may support diagnosis of carpal tunnel syndrome?
Phalen’s test — positive if flexing the wrist for 60 seconds causes pain and paraesthesia in the median nerve distribution.
Tinel’s test — positive if tapping lightly over the median nerve at the volar surface of the wrist produces paraesthesia or pain in the median nerve distribution.
Durkan’s test (carpal tunnel compression test) — positive if direct pressure over the proximal edge of the transverse carpal ligament (proximal wrist crease) with the thumbs produces or worsens paraesthesia in the median nerve distribution.
Carpal tunnel syndrome caused by compression of what nerve in the carpal tunnel?
median
pain/pins and needles in thumb, index, middle finger
unusually the symptoms may ‘ascend’ proximally
patient shakes his hand to obtain relief, classically at night
carpal tunnel syndrome
Examination findings in carpal tunnel syndrome?
weakness of thumb abduction (abductor pollicis brevis)
wasting of thenar eminence (NOT hypothenar)
Tinel’s sign: tapping causes paraesthesia
Phalen’s sign: flexion of wrist causes symptoms
Electrophysiology in carpal tunnel syndrome?
motor + sensory: prolongation of the action potential
Severe symptoms or symptoms persist with conservative Mx in carpal tunnel syndrome?
surgical decompression (flexor retinaculum division)
Sciatic nerve divides into what?
tibial and common peroneal nerves
Common peroneal nerve lesion: injury often occurs where?
neck of fibula
Most characteristic feature of common peroneal nerve lesion?
foot drop
Features of common peroneal nerve lesion?
- foot drop
- weakness of foot dorsiflexion
- weakness of foot eversion
- weakness of extensor hallucis longus
- sensory loss over the dorsum of the foot and the lower lateral part of the leg
- wasting of the anterior tibial and peroneal muscles
Injury at neck of fibula
Foot drop
Weakness of foot dorsiflexion and foot eversion
Weakness of extensor hallucis long
common peroneal nerve lesion
Meralgia paraesthetica?
syndrome of paraesthesia or anaesthesia in the distribution of the lateral femoral cutaneous nerve (LFCN).
It is an entrapment mononeuropathy of the LFCN, but can also be iatrogenic after a surgical procedure, or result from a neuroma.
Although uncommon, meralgia paraesthetica is not rare and is hence probably underdiagnosed.
lateral femoral cutaneous nerve (LFCN)?
The LFCN is primarily a sensory nerve, carrying no motor fibres.
It most commonly originates from the L2/3 segments.
After passing behind the psoas muscle, it runs beneath the iliac fascia as it crosses the surface of the iliac muscle and eventually exits through or under the lateral aspect of the inguinal ligament.
As the nerve curves medially and inferiorly around the anterior superior iliac spine (ASIS), it may be subject to repetitive trauma or pressure.
Compression of this nerve anywhere along its course can lead to the development of meralgia paraesthetica.
Epidemiology of meralgia paraesthetica?
- 30-40yrs
- sometimes both legs affected
- men more common
- more common in those with DM
RFs for meralgia paraesthetica?
Obesity
DM
Pregnancy
Tense ascites
Trauma
Iatrogenic, such as pelvic osteotomy, spinal surgeries, laparoscopic hernia repair and bariatric surgery. In some cases, may result from abduction splints used in the management of Perthe’s disease.
Various sports have been implicated, including gymnastics, football,
bodybuilding and strenuous exercise.
Some cases are idiopathic.
Symptoms of meralgia paraesthetica?
upper lateral aspect of the thigh:
Burning, tingling, coldness, or shooting pain
Numbness
Deep muscle ache
Aggravated by standing, and relieved by sitting
They can be mild and resolve spontaneously or may severely restrict the patient for many years.
Signs of meralgia paraesthetica?
Symptoms may be reproduced by deep palpation just below the ASIS (pelvic compression) and also by extension of the hip.
There is altered sensation over the upper lateral aspect of the thigh.
There is no motor weakness.
Ix for meralgia paraesthetica?
The pelvic compression test is highly sensitive, and often, meralgia paraesthetica can be diagnosed based on this test alone.
Injection of the nerve with local anaesthetic will abolish the pain. Using ultrasound is effective both for diagnosis and guiding injection therapy in meralgia paraesthetica
Nerve conduction studies may be useful.
Mx for meralgia paraesthetica?
Physical therapy to strengthen the muscles of the legs and buttocks, and reduce injury to the hips. Wearing less restrictive clothing. Weight loss management. Corticosteroid injection to reduce swelling.
Radial nerve is a continuation of what?
posterior cord of the brachial plexus (root values C5 to T1)
Path of the radial nerve?
In the axilla: lies posterior to the axillary artery on subscapularis, latissimus dorsi and teres major.
Enters the arm between the brachial artery and the long head of triceps (medial to humerus).
Spirals around the posterior surface of the humerus in the groove for the radial nerve.
At the distal third of the lateral border of the humerus it then pierces the intermuscular septum and descends in front of the lateral epicondyle.
At the lateral epicondyle it lies deeply between brachialis and brachioradialis where it then divides into a superficial and deep terminal branch.
Deep branch crosses the supinator to become the posterior interosseous nerve.
Radial nerve: motor innervation (main nerve)?
Triceps
Anconeus
Brachioradialis
Extensor carpi radialis
Radial nerve: motor innervation (posterior interosseous branch)?
Supinator
Extensor carpi ulnaris
Extensor digitorum
Extensor indicis
Extensor digiti minimi
Extensor pollicis longus and brevis
Abductor pollicis longus
Radial nerve: sensory innervation?
The area of skin supplying the proximal phalanges on the dorsal aspect of the hand is supplied by the radial nerve (this does not apply to the little finger and part of the ring finger)
Radial nerve:
- anatomical location= shoulder
- muscle affected?
- effect of paralysis?
Long head of triceps
Minor effects on shoulder stability in abduction
Radial nerve:
- anatomical location= arm
- muscle affected?
- effect of paralysis?
- triceps
- loss of elbow extension
Radial nerve:
- anatomical location= forearm
- muscle affected?
- effect of paralysis?
Supinator
Brachioradialis
Extensor carpi radialis longus and brevis
Weakening of supination of prone hand and elbow flexion in mid prone position
Patterns of damage of radial nerve?
wrist drop
sensory loss to small area between the dorsal aspect of the 1st and 2nd metacarpals
Radial nerve: axillary damage?
paralysis of triceps
Wrist drop?
radial nerve damage
Foot drop?
common peroneal nerve damage
Thoracic outlet syndrome (TOS)?
disorder involving compression of brachial plexus, subclavian artery or vein at the site of the thoracic outlet.
TOS can be neurogenic or vascular; the former accounts for 90% of the cases.
Thoracic outlet?
the space between the collarbone, known as the clavicle, and the first rib.
This narrow passageway is crowded with blood vessels, nerves and muscles.
Typical pts affected with thoracic outlet syndrome?
young thin women possessing long neck and drooping shoulders
peak onset in 4th decade
Causes of thoracic outlet syndrome?
- neck trauma in pts with anatomical predispositions
- neck trauma can be single acute or repeated stresses
- anatomical anomalies can be in form of soft tissue eg. scalene muscle hypertrophy and anomalous bands
- or osseous structures eg. presence of cervical rib
Cervical rib?
extra rib that forms above 1st rib growing from base of neck just above collarbone
may cause thoracic outlet syndrome if compresses nearby nerves and blood vessels
2 types of thoracic outlet syndrome
neurogenic TOS
vascular TOS
CP of neurogenic thoracic outlet syndrome?
painless muscle wasting of hand muscles, with patients complaining of hand weakness e.g. grasping
sensory symptoms such as numbness and tingling may be present
if autonomic nerves are involved, the patient may experience cold hands, blanching or swelling
CP of vascular thoracic outlet syndrome?
subclavian vein compression leads to painful diffuse arm swelling with distended veins
subclavian artery compression leads to painful arm claudication and in severe cases, ulceration and gangrene
Examination findings in thoracic outlet syndrome?
neurological examination and musculoskeletal examination are necessary
stress manoeuvres such as Adson’s manoeuvres may be attempted although they have limited utility
careful examinations should aim to rule out other pathologies of the cervical spine, the shoulder or peripheral nerves. For instance, cervical radiculopathy, shoulder injuries and carpal tunnel syndrome
Ix for thoracic outlet syndrome?
chest and cervical spine plain radiographs to check for any obvious osseous abnormalities e.g. cervical ribs, exclude malignant tumours or other differentials e.g. cervical spine degenerative changes
other imaging modalities may be helpful e.g. CT or MRI to rule out cervical root lesions
venography or angiography may be helpful in vascular TOS
an anterior scalene block may be used to confirm neurogenic TOS and check the likelihood of successful surgical treatment
Tx for thoracic outlet syndrome?
there is a limited evidence base
conservative management with education, rehabilitation, physiotherapy, or taping is typically the first-line management for neurogenic TOS
surgical decompression is warranted where conservative management has failed especially if there is a physical anomaly. Early intervention may prevent brachial plexus degeneration
in vascular TOS, surgical treatment may be preferred
other therapies being investigated include botox injection
Where does the ulnar nerve arise?
from medial cord of brachial plexus (C8, T1)
Ulnar nerve: motor innervation to?
medial two lumbricals
aDductor pollicis
interossei
hypothenar muscles: abductor digiti minimi, flexor digiti minimi
flexor carpi ulnaris
Ulnar nerve: sensory innervation to?
medial 1 1/2 fingers (palmar and dorsal aspects)
Path of the ulnar nerve?
the ulnar nerve travels through the posteromedial aspect of the upper arm to the flexor compartment of the forearm
it then enters the palm of the hand via the Guyon’s canal, superficial to the flexor retinaculum and lateral to the pisiform bone
Branches of the ulnar nerve?
1) muscular branch
2) palmar cutaneous branch (Arises near the middle of the forearm)
3) dorsal cutaneous branch
4) superficial branch
5) deep branch
Branches of the ulnar nerve: muscular branch supplies what?
Flexor carpi ulnaris
Medial half of the flexor digitorum profundus
Branches of the ulnar nerve: Palmar cutaneous branch (Arises near the middle of the forearm) supplies what?
skin on medial part of the palm
Branches of the ulnar nerve: dorsal cutaneous branch supplies what?
dorsal surface of medial part of hand
Branches of the ulnar nerve: superficial branch supplies what?
Cutaneous fibres to the anterior surfaces of the medial one and one-half digits
Branches of the ulnar nerve: deep branch supplies what?
Hypothenar muscles
All the interosseous muscles
Third and fourth lumbricals
Adductor pollicis
Medial head of the flexor pollicis brevis
Ulnar nerve: 2 patterns of damage?
damage at the wrist
damage at elbow
Ulnar nerve: damage at the wrist?
‘claw hand’ - hyperextension of the metacarpophalangeal joints and flexion at the distal and proximal interphalangeal joints of the 4th and 5th digits
wasting and paralysis of intrinsic hand muscles (except lateral two lumbricals)
wasting and paralysis of hypothenar muscles
sensory loss to the medial 1 1/2 fingers (palmar and dorsal aspects)
Ulnar nerve: damage at elbow?
like at wrist (however, ulnar paradox - clawing is more severe in distal lesions)
radial deviation of wrist
Claw hand?
damage of ulnar nerve at wrist
hyperextension of the metacarpophalangeal joints and flexion at the distal and proximal interphalangeal joints of the 4th and 5th digits
Another name for sciatica?
lumbar radiculopathy
Sciatica (lumbar radiculopathy)?
describes radiating leg pain caused by inflam or compression of the lumbosacral nerve roots (L4-S1) forming the sciatic nerve
Pain in sciatica (lumbar radiculopathy)?
- sudden or slow onset
- vary in severity
- felt in back or buttock and radiates down leg below the knee into the foot and toes in the distribution of the sciatic nerve
- tingling, numbness, loss of muscle strength in same leg
In sciatica (lumbar radiculopathy), nerve root compression may be caused by what?
- herniated intervertebral disc (90%)
- spondylolisthesis
- spinal stenosis
- infection
- cancer
Modifiable RFs which be be associated with a first onset of sciatica (lumbar radiculopathy)?
- smoking
- obesity
- general health
- occupational factors eg. whole body vibration, strenuous exercise
other RFs= older age, genetic influences
How long do episodes of sciatica (lumbar radiculopathy) last?
usually transient, with rapid improvements in pain and disability seen within a few weeks to a few months, but recurrence of symptoms is common.
S&S sciatica (lumbar radiculopathy)?
Unilateral leg pain radiating below the knee to the foot or toes.
Low back pain — if present, which is less severe than any leg pain.
Numbness, tingling (paraesthesia) in the distribution of a nerve root.
Weakness or reflex changes, or both in a myotomal distribution.
A positive result in a straight leg raise test.
Red flags for pts with sciatica include?
Bowel/bladder dysfunction (most commonly urinary retention).
Progressive neurological weakness.
Saddle anaesthesia.
Bilateral radiculopathy.
Localised tenderness.
Incapacitating or progressive pain.
Unrelenting night pain.
Use of steroids or intravenous drugs.
Serious conditions whose signs and symptoms may overlap with sciatica?
Cauda equina syndrome.
Spinal fracture.
Cancer.
Infection.
What does Mx of sciatica involve?
- admit or urgent refer if red flags
- STarT Back risk assessment tool to inform shared decision-making about stratified management for people who do not have red flag signs or symptoms.
- self-Mx advice
- follow up if worsen, persist >2w, severe pain not subsided within 1w, new symptoms or recur
- consider referal to group exercise programme, physio, psychological therapy
- promote return to work or ADLs
Mx if symptoms of sciatica persist, worsen or recur?
reassessment
Considering offering a combined physical and psychological programme incorporating a cognitive behavioural approach.
Referring where appropriate.
Sciatica Mx?
- NSAIDs +/- PPI for lower back pain NOT neuropathic analgesia eg. duloxetine
- physio, exercises, CBT esp if higher risk of poor outcome
- if persist (>4-6w) refer for consideration of MRI
- do not recommend prolonged best rest
- local heat, work adjustments, keep active and exercise to reduce risk of recurrence (modest increase in pain on resuming activities does not indicate damage)
Specialist referral for people with sciatica and?
Severe radicular pain at 2–6 weeks (depending on severity and improvement).
Non-tolerable radicular pain at 6 weeks.
Acute and severe sciatica — for consideration for an epidural corticosteroid/local anaesthetic injection.
Sciatica when non-surgical treatment has not improved pain or function — for consideration for spinal decompression.
Positive result in straight leg raise test in sciatica meaning?
person lying supine, the hip is flexed gradually with the knee extended. Pain reproduced below 60 degrees of hip flexion on the ipsilateral side indicates a positive test.
A prolapsed lumbar disc usually produces what?
clear dermatomal leg pain associated with neuro deficits
Prolapsed lumbar disc features?
- leg pain usually worse than back
- pain often worse when sitting
L3 nerve root compression?
Sensory loss over anterior thigh
Weak hip flexion, knee extension and hip adduction
Reduced knee reflex
Positive femoral stretch test
L4 nerve root compression?
Sensory loss anterior aspect of knee and medial malleolus
Weak knee extension and hip adduction
Reduced knee reflex
Positive femoral stretch test
L5 nerve root compression?
Sensory loss dorsum of foot
Weakness in foot and big toe dorsiflexion
Reflexes intact
Positive sciatic nerve stretch test
S1 nerve root compression?
Sensory loss posterolateral aspect of leg and lateral aspect of foot
Weakness in plantar flexion of foot
Reduced ankle reflex
Positive sciatic nerve stretch test
Prolapsed lumbar disc producing clear dermatomal leg pain eg. L3, L4, L5 or S1 compression Mx?
similar to that of other musculoskeletal lower back pain and sciatica: analgesia, physiotherapy, exercises
using the same drugs as for back pain without sciatica symptoms i.e. first-line is NSAIDs +/- proton pump inhibitors rather than using neuropathic analgesia (e.g. duloxetine)
if symptoms persist e.g. after 4-6 weeks) then referral for consideration of MRI is appropriate
Low back pain?
refers to pain in the lumbosacral area, from the 12th ribs to the iliac crest, and sometimes the buttocks and gluteal folds.
Non-specific low back pain?
describes pain not attributable to an underlying cause. It is also referred to as ‘mechanical’, ‘musculoskeletal’, or ‘simple’ low back pain in the literature.
Acute low back pain typically lasts less than 3 months.
Chronic low back pain typically lasts 3 months or more.
Low back pain RFs?
obesity, physical inactivity, heavy lifting, and stress or depression.
Low back pain- resolves when?
self-limiting condition for the majority of people, and usually resolves within a few weeks.
Cx of low back pain?
impact on daily activities including work, study, leisures, and sleep; depression and anxiety; increased risk of falls and chronic pain.
Stroke site of lesion: anterior cerebral artery?
contralateral hemiparesis and sensory loss
lower extremity > upper
Stroke site of lesion: middle cerebral artery?
contralateral hemiparesis and sensory loss
upper extremity > lower
contralateral homonymous hemianopia
aphasia
Stroke site of lesion: posterior cerebral artery?
Contralateral homonymous hemianopia with macular sparing
Visual agnosia
Stroke site of lesion: Weber’s syndrome (branches of the posterior cerebral artery that supply the midbrain?
Ipsilateral CN III palsy
Contralateral weakness of upper and lower extremity
Stroke site of lesion: Posterior inferior cerebellar artery (lateral medullary syndrome, Wallenberg syndrome)?
Ipsilateral= facial pain and temp loss
Contralateral= limb/torso pain and temp loss
Horner’s (ipsilateral), dysphagia, ataxia and nystagmus
Stroke site of lesion: Anterior inferior cerebellar artery (lateral pontine syndrome)?
Similar to Wallenberg’s but…
ipsilateral= facial paralysis and deafness
Stroke site of lesion: retinal/opthalmic artery?
amaurosis fugax
Stroke site of lesion: basilar artery?
‘locked in’ syndrome
Stroke site of lesion: Lacunar strokes?
either isolated hemiparesis, hemisensory loss or hemiparesis with limb ataxia
strong association with HTN
common sites include= basal ganglia, internal capsule, pons and thalamus
What is a lacunar stroke?
stroke that occurs in a deep area of brain eg. basal ganglia, thalamus, pons or internal capsule
Stroke site of lesion: what vessel is affected in lateral pontine syndrome?
anterior inferior cerebellar artery
Another name for lateral medullary syndrome?
Wallenberg syndrome
Stroke site of lesion: what vessel is affected in Wallenberg syndrome/lateral medullary syndrome?
posterior inferior cerebellar artery
Stroke site of lesion: what vessel is affected in Weber’s syndrome?
branches of the posterior cerebral artery that supply the midbrain
Stroke?
clinical syndrome of presumed vascular origin characterised by rapidly developing signs of focal or global disturbance of cerebral functions which lasts >24hrs or leads to death
What does TIA stand for?
transient ischaemic attack
What is a TIA?
transient (<24hrs) neuro dysfunction caused by focal brain, spinal cord or retinal ischaemia, without evidence of acute infarction
What % of strokes are ischaemic and haemorrhagic?
85% ischaemia
15% haemorrhagic
Once pt has had a stroke or TIA they are at high risk of…
further vascular event
Cx of stroke?
neuro problems, depression, anxiety, communication difficulties, difficulties with ADLs
Stroke and TIA present with what typically?
sudden onset of focal neuro symptoms= numbness, weakness, slurred speech or visual disturbance
can’t be explained by another condition eg. hypoglycaemia
widespread cerebral hypoperfusion may present with non-focal or global deficits
TIA vs stroke?
both present with sudden onset neuro deficit but
TIA= completely resolved within 24hrs of onset
stroke= ongoing or has persisted for longer than 24hrs
Mx of suspected acute stroke (or emergent TIA) in primary care?
- immediate emergency admission to stroke unit
- avoid antiplatelets until exclude haemorrhagic stroke
When should follow up in primary care happen following a stroke?
on discharge from hospital, at 6m then annually to:
optimise lifestyle measures and drug Tx for secondary prevention
Mx of suspected TIA in primary care?
aspirin 300mg immediately
arrange specialist
assessment within 24hrs if suspected TIA occured within last week
if it occurred >1w ago then within 7 days
do not drive until see specialist
What in a stroke may present with non-focal or global neuro deficits?
widespread cerebral hypoperfusion eg. subarachnoid/intracranial haemorrhage or massive infarct
How long do TIAs typically take to resolve?
within 1hr but can persist up to 24hrs
Examples of focal neuro deficits in TIA?
unilateral weakness or sensory loss
dysphasia
ataxia, vertigo or loss of balance
syncope
amaurosis fugax, diplopia or homonymous hemianopia
cranial nerve defects
Amaruosis fugax?
sudden transient loss of vision in one eye eg. in TIA
Clinical features of stroke?
depend on cause and area affected
- confusion, altered consciousness, coma
- headache
- unilateral weakness/paralysis in face, arm or leg
- sensory loss= paraesthesia or numbness
- ataxia
- dysphasia
- dysarthria
- visual disturbance= homonymous hemianopia, diplopia
- gaze paresis
- photophobia
- dizziness, vertigo or loss of balance (isolated dizziness not usually TIA)
- N +/or V
- Specific cranial nerve deficits: unilateral tongue weakness, Horner’s
- difficulty with fine motor coordination & gait
- neck or facial pain
What may neck or facial pain in stroke be associated with?
arterial dissection
Type of headache pt may get with stroke?
usually of insidious onset and gradually increasing intensity in intracranial haemorrhage, and sudden, severe headache in subarachnoid haemorrhage which may be associated with neck stiffness. Sentinel headache(s) may occur in the preceding weeks.
Specific cranial nerve deficits pt may get when having a stroke?
unilateral tongue weakness or Horner’s syndrome (miosis, ptosis, and facial anhidrosis).
Horner’s triad?
miosis, ptosis, facial anhidrosis
What is gaze paresis eg. in stroke?
(conjugate gaze palsy) is inability to move both eyes together in a single horizontal (most commonly) or vertical direction
Posterior circulation strokes may be difficult to diagnose and should be suspected if pt presents with what?
Symptoms of acute vestibular syndrome — acute, persistent, continuous vertigo or dizziness with nystagmus, nausea or vomiting, head motion intolerance, and new gait unsteadiness.
Examine pt with suspected stroke/TIA?
- Consciousness: Glasgow
coma scale - ABC
- BP, HR, O2, T
- focused neuro exam= FAST
- cvd= AF, murmur, pulmonary oedema or HF present?
- blood glucose to rule out hypoglycaemia (<3.3)
- ECG to exclude arrhythmias
Tool to use to carry out rapid assessment of neuro function if suspect pt is having a stroke/TIA?
FAST
What does the FAST tool stand for?
Face Arm Speech Test
When to suspect a stroke when using FAST?
one or more of the following are present: new facial weakness (asymmetry such as the mouth or eye drooping), arm or leg weakness, or speech disturbance (such as slurring or difficulty in finding names for commonplace objects).
What is it really important to do if suspect a pt is having a stroke?
DO NOT start anticoag (eg. in AF) or antiplatelet until haemorrhagic stroke has been excluded by brain imaging
Mx for suspected acute stroke?
- immediate emergency admission to acute stroke unit
- whilst awaiting transfer= ABCs, give O2 if sats <95%
- do not start antiplatelet/anticoag
- exclude hypoglycaemia
- immediate CT head
Because transient ischaemic attack (TIA) cannot be confidently diagnosed unless the symptoms have resolved within 24 hours, people with ongoing neurological symptoms and signs suggestive of acute stroke or TIA should be treated as if they have
stroke
Mx if pt has had a TIA within the last week?
1) aspirin 300mg immediately
2) refer for specialist assessment to be seen within 24hrs
pt may have ongoing neuro deficits despite a negative FAST so these pts must be managed as acute stroke rather than TIA
Mx for pt who had a suspected TIA more than 7 days ago?
1) aspirin 300mg immediately
2) refer for specialist assessment to be seen within 7 days
Pt who had suspected TIA within the last week but have a bleeding disorder or taking an anticoag?
have urgent CT to exclude haemorrhage
Lifestyle advice for pt following stroke/TIA?
- physical activity every day
- stop smoking
- healthy diet
- limit alcohol to 14 units a week spread over at least 3 days
Secondary prevention following stroke or TIA without paroxysmal or permanent AF?
1) Antiplatelet therapy:
Dual therapy= aspirin + clopidogrel (for up to 90 days) or aspirin + ticagrelor (for 30 days)
then= clopidogrel 75mg daily long term
2nd line= Aspirin 75 mg daily with modified-release dipyridamole 200 mg twice daily (long term)
2) AND atorvastatin 20-80mg daily= if cholesterol >3.5mmol/l
3) do they need Tx for HTN?
Aim of statin therapy used following stroke/TIA in secondary prevention?
reduce non-HDL cholesterol by more than 40%.
If this reduction is not achieved within 3 months, consider adherence, diet, lifestyle, and increasing dose
How is secondary prevention following stroke or TIA WITH paroxysmal or permanent AF different to without?
if AF then need anticoag not antiplatelet
TIA= will start immediately on diagnosis once imaging has excluded haemorrhage.
Stroke= delayed but within 14d
adjusted-dose warfarin (target INR 2.5, range 2.0 to 3.0) or a direct thrombin or factor Xa inhibitor (for people with non-valvular atrial fibrillation).
What is a stroke?
(aka cerebrovascular accident, CVA) represents a sudden interruption in the vascular supply of the brain.
Neural tissue is completely dependent on aerobic metabolism so any problem with oxygen supply can quickly lead to irreversible damage.
2 main types of stroke?
1) ischaemic= subtype is TIA- due to transient decrease in blood flow
2) haemorrhagic
Essential problem in ischaemic stroke?
‘Blockage’ in the blood vessel stops blood flow
Subtypes of ischaemic stroke?
Thrombotic stroke= thrombosis from large vessels e.g. carotid
Embolic stroke= usually a blood clot but fat, air or clumps of bacteria may act as an embolus. Atrial fibrillation is an important cause of emboli forming in the heart
General RFs for CVD and therefore stroke?
age
HTN
smoking
hyperlipidaemia
DM
RFs for cardioembolism causing ischaemic stroke?
AF
Essential problem in haemorrhagic stroke?
blood vessel ‘bursts’ leading to reduction in blood flow
Subtypes of hameorrhagic stroke?
Intracerebral haemorrhage= bleeding within the brain
Subarachnoid haemorrhage= bleeding on the surface of the brain
RFs for haemorrhagic stroke?
age
HTN
anticoag therapy
arteriovenous malformation
Cerebral hemisphere infarct stroke may have what symptoms?
contralateral hemiplegia: initially flaccid then spastic
contralateral sensory loss
homonymous hemianopia
dysphasia
Brainstem infarct stroke may result in what symptoms?
more severe symptoms including quadriplegia and lock-in-syndrome
What classification system can be used to classify strokes based on initial symptoms?
Oxford Stroke Classification (also known as the Bamford Classification)
Oxford Stroke Classification (also known as the Bamford Classification) criteria?
The following criteria should be assessed:
1. unilateral hemiparesis and/or hemisensory loss of the face, arm & leg
2. homonymous hemianopia
3. higher cognitive dysfunction e.g. dysphasia
Oxford Stroke Classification (also known as the Bamford Classification): total anterior circulation infarcts (TACI, 15%)?
- invl middle and anterior cerebral arteries
- all 3 of the criteria are present
Oxford Stroke Classification (also known as the Bamford Classification): partial anterior circulation infarcts (PACI, 25%)?
- involves smaller arteries of anterior circulation e.g. upper or lower division of middle cerebral artery
- 2 of criteria present
Oxford Stroke Classification (also known as the Bamford Classification): lacunar infarcts (LACI, 25%)?
involves perforating arteries around the internal capsule, thalamus and basal ganglia
presents with 1 of the following:
1. unilateral weakness (and/or sensory deficit) of face and arm, arm and leg or all three.
2. pure sensory stroke.
3. ataxic hemiparesis
Oxford Stroke Classification (also known as the Bamford Classification): posterior circulation infarcts (POCI, 25%)?
involves vertebrobasilar arteries
presents with 1 of the following:
1. cerebellar or brainstem syndromes
2. loss of consciousness
3. isolated homonymous hemianopia
How to differentiate between haemorrhage stroke vs ischaemia?
symptoms alone can’t need imaging but pts with haemorrhages more likely to have:
- decreased level consciousness
- headache much more common
- N&V common
- seizures in 25%
What does the FAST mneumonic for stroke stand for and mean?
Face - ‘Has their face fallen on one side? Can they smile?’
Arms - ‘Can they raise both arms and keep them there?’
Speech - ‘Is their speech slurred?’
Time - ‘Time to call 999 if you see any single one of these signs.’
Ix for stroke?
- emergency neuroimaging= non-contrast head CT
Main Mx of stroke once it has been classified as ischaemic following imaging?
aspirin 300mg
present <4.5hrs of onset of stroke symptoms= thrombolysis with alteplase
<6hr and confirmed occlusion of proximal anterior circulation confirmed on CTA= thrombectomy and IV thrombolysis (if within 4.5hrs)
6-24hrs (incl wake up strokes) and confirmed occlusion of proximal anterior circulation or potential to salvage brain tissue shown on diffusion weight MRI or CT perfusion showing limited infarct core volume= thrombectomy
Confirmed occlusion of the proximal posterior circulation (that is, basilar or posterior cerebral artery) demonstrated by CTA or MRA and
if there is the potential to salvage brain tissue= thrombectomy with IV thrombolysis (if within 48hrs)
How does alteplase work in stroke Mx?
tissue plasminogen activator that rapidly breaks down clots
What if pt has HTN and ischaemic stroke?
lowering the blood pressure can worsen the ischaemia.
High blood pressure treatment is only indicated in hypertensive emergency or to reduce the risks when giving intravenous thrombolysis.
Blood pressure is aggressively treated in patients with a haemorrhagic stroke.
Criteria for thrombolysis with alteplase in Mx of ischaemic stroke?
present <4.5hrs of onset of symptoms
and has not had previous intracranial haemorrhage, uncontrolled HTN, pregnant ect
Crescendo TIA?
more than 1 TIA within a week- high risk of stroke
Symptoms of stroke are typically what?
asymmetrical
What tool is used to identify stroke in hospital?
The ROSIER tool (Recognition Of Stroke In the Emergency Room) gives a score based on the clinical features and duration. Stroke is possible in patients scoring one or more.
Tool to identify stroke in community vs hospital?
community= FAST
hospital= ROSIER
Ix for TIA?
Diffusion weighted MRI scan
Assessment for underlying causes of TIA or stroke?
Ix for carotid artery stenosis and AF
CAS= confirmed with carotid US/CT/MRI angiogram; carotid endarterectomy is Mx
AF= ECG or ambulatory ECG monitoring; anticoag
Drugs for stroke secondary prevention?
clopidogrel 75mg od
and
atorvastatin 20-80mg (started after 48hrs not immediately)
if AF then give anticoag instead of antiplatelet
Stroke rehab may involve who?
Stroke physicians
Nurses
Speech and language (SALT) to assess swallowing
Dieticians in those at risk of malnutrition
Physiotherapy
Occupational therapy
Social services
Optometry and ophthalmology
Psychology
Orthotics
Mx of haemorrhagic stroke?
neurosurgical consult, majority not fit for surgery.
Mx is supportive= stop anticoag and antiplatelet meds
What is it important to exclude first when suspect stroke?
hypoglycaemia
ROSIER score?
Exclude hypoglycaemia first, then assess the following:
Loss of consciousness or syncope= - 1 point
Seizure activity= - 1 point
New, acute onset of:
* asymmetric facial weakness + 1 point
* asymmetric arm weakness + 1 point
* asymmetric leg weakness + 1 point
* speech disturbance + 1 point
* visual field defect + 1 point
A stroke is likely if > 0.
What may acute ischaemic stroke show on non-contrast CT head?
may show areas of low density in the grey and white matter of the territory. These changes may take time to develop
other signs include the ‘hyperdense artery’ sign corresponding with the responsible arterial clot - this tends to visible immediately
What may acute haemorrhagic stroke show on non-contrast CT head?
typically show areas of hyperdense material (blood) surrounded by low density (oedema)
When should BP control be considered for pts who present with acute ischaemic stroke?
if present within 6hrs and S BP of >150
there are Cx eg. hypertensive encephalopathy
or being considered for thrombolysis
ANY OTHER CASE DO NOT LOWER BP in acute phase of ischaemic stroke
Why is statin Tx delayed at least 48hrs after Tx for ischaemic stroke?
due to risk of haemorrhagic transformation
When to start pt on statin following ischaemic stroke?
if cholesterol >3.5mmol/l
patients with an acute ischaemic stroke, regardless of age or stroke severity, who were last known to be well more than 4.5 hours earlier, should be considered for thrombolysis with alteplase if:
treatment can be started between 4.5 and 9 hours of known onset, or within 9 hours of the midpoint of sleep when they have woken with symptoms, AND
they have evidence from CT/MR perfusion (core-perfusion mismatch) or MRI (DWI-FLAIR mismatch) of the potential to salvage brain tissue
What should be done before thombolysis Tx for stroke?
lower BP to 185/110
Absolute contraindications for thrombolysis?
- Previous intracranial haemorrhage
- Seizure at onset of stroke
- Intracranial neoplasm
- Suspected subarachnoid haemorrhage
- Stroke or traumatic brain injury in preceding 3 months
- Lumbar puncture in preceding 7 days
- Gastrointestinal haemorrhage in preceding 3 weeks
- Active bleeding
- Oesophageal varices
- Uncontrolled hypertension >200/120mmHg
Relative contraindications for thrombolysis?
- Pregnancy
- Concurrent anticoagulation (INR >1.7)
- Haemorrhagic diathesis
- Active diabetic haemorrhagic retinopathy
- Suspected intracardiac thrombus
- Major surgery / trauma in the preceding 2 weeks
When is carotid endarterectomy recommended?
pt suffered a stroke or TIA in the carotid territory and is not severely disabled
if >50% stenosis and within 7d
Subarachnoid haemorrhage (SAH)?
intracranial haemorrhage defined as the presence of blood within the subarachnoid space i.e. deep to subarachnoid layer of the meninges
Where is the subarachnoid space?
deep to the subarachnoid layer of the meninges
Most common cause of subarachnoid haemorrhage?
head injury= traumatic SAH
What is a spontaneous subarachnoid haemorrhage?
occurs in the absence of trauma
Traumatic SAH?
subarachnoid haemorrhage occurring due to trauma
2 types of subarachnoid haemorrhage?
spontaneous and traumatic
Most common cause of spontaneous subarachnoid haemorrhage?
intracranial aneurysm (saccular ‘berry’ aneurysms) 85%
Conditions associated with berry aneurysms (most common cause of spontaneous subarachnoid haemorrhage)?
HTN
adult polycystic kidney disease
Ehlers-Danlos syndrome
Coarctation of aorta
Causes of spontaneous subarachnoid haemorrhage?
- intracranial aneurysm (berry)
- arteriovenous malformation
- pituitary apoplexy
- mycotic (infective) aneurysms
Features of subarachnoid haemorrhage?
- headache= sudden, ‘thunderclap’, severe, occipital, peaks in intensity in 1-5mins, may be Hx of less severe ‘sentinel’ headache weeks prior
- N&V
- meningism (photophobia, neck stiffness)
- coma
- seizures
What ECG changes may be seen in subarachnoid haemorrhage?
ST elevation may be seen
2 to either autonomic neural stimulation from hypothalamus or elevated levels of circulating catecholamines
Headache in subarachnoid haemorrhage?
- sudden-onset
- ‘thunderclap or hit with baseball bat’
- severe: ‘worst of my life’
- occipital
- peaks in intensity within 1-5mins
- may be history of a less-severe ‘sentinel’ headache in the weeks prior to presentation
Meningism in subarachnoid haemorrhage?
photophobia, neck stiffness
Thunderclap headache and meningism?
subarachnoid haemorrhage
Ix for subarachnoid haemorrhage?
1st line= non-contrast CT head
if shows evidence of SAH then refer to neurosurgery urgent
What would non-contrast head CT show in subarachnoid haemorrhage?
acute blood (hyperdense/bright on CT) typically distributed in basal cisterns, sulci and in severe cases the ventricular system
What does acute bleeding look like on head CT eg. in subarachnoid haemorrhage?
hyperdense/bright
What if suspect subarachnoid haemorrhage but CT head is done >6hrs after symptom onset and is normal?
do lumbar puncture
if within 6hrs and normal consider different diagnosis
When should an LP done if subarachnoid haemorrhage is suspected?
if CT head is done >6hrs after symptom onset do a LP
LP needs to be done at least 12hrs following the onset of symptoms
If CT head normal and has been >6hrs since symptom onset & suspect subarachnoid haemorrhage, why does the LP need to be done at least 12hrs after symptom onset?
to allow the development of xanthochromia (result of RBC breakdown)
xanthochromia helps distinguish true SAH from a ‘traumatic tap’ (blood introduced by the LP procedure)
If LP is indicated in suspected subarachnoid haemorrhage, what would the findings be?
xanthocrhomia in CSF and normal or raised opening pressure
LP CSF: xanthochromia and normal or raised opening pressure?
subarachnoid haemorrhage
What to do after confirming subarachnoid haemorrhage (with CT or LP) and it is a spontaneous SAH (no trauma)?
Ix to find causative pathology that needs urgent Tx:
- CT intracranial angiogram= identify vascular lesion eg. aneurysm or AVM
- /- digital subtraction angiogram (catheter angiogram)
Causes of thunderclap headache?
SAH
migraine
cough
coitus
exertion
When should non-contrast head CT be done in suspected subarachnoid haemorrhage to diagnose?
diagnostic accuracy highest within 6hrs of symptom onset
if >6hrs then do LP
Mx of confirmed aneurysmal subarachnoid haemorrhage?
- supportive= best rest, analgesia, VTE prophy, discontinuation of antithrombotics (reverse anticoag if present)
- oral nimodipine= present vasospasm
- risk of rebleeding so need intervention within 24hrs= Tx with coil by interventional neuroradiologists, minority need craniotomy and clipping by surgeon
Prevent vasospasm in subarachnoid haemorrhage?
oral nimodipine: increases BP and blood volume
What is vasospasm in subarachnoid haemorrhage?
Cx that may occur 7-14 days after SAH
narrowing of brain’s arteries that reduces blood flow to brain
weakness, confusion, restlessness
Cx of aneurysmal SAH?
- re-bleeding
- hydrocephalus
- vasospasm (delayed blood volume)
- hyponatraemia
- seizures
Cx of aneurysmal SAH: re-bleeding?
happens in 10%
most common in 1st 12hrs
if suspected (sudden worsening of neuro symptoms) then repeat CT
up to 70% mortality
Cx of aneurysmal SAH: hydorcephalus?
temp treated with external ventricular drain (CSF diverted into a bad at the bedside) or if required, long-term ventriculoperitoneal shunt
XS CSF in brain due to disruption to drainage and production
Cx of aneurysmal SAH: vasospasm (delayed cerebral ischaemia)?
typically 7-14d after onset
ensure euvolaemia (normal blood volume)
consider vasopressor if persists
Cx of aneurysmal SAH: hyponatraemia?
most typically due to SIADH
What electrolyte abnormality may be a Cx of SAH?
hyponatraemia
Important predictive factors in SAH?
conscious level on admission
age
amount of blood visible on CT head
Why is DAPT (dual antiplatelet therapy) not currently recommended for ‘major’ ischaemic stroke?
risk of haemorrhagic transformation is too high
Definition of TIA?
a transient episode of neurologic dysfunction caused by focal brain, spinal cord, or retinal ischaemia, without acute infarction.
lasting <24hrs
Clinical features in TIA?
focal neurological deficit but, rather than persisting, the features resolve, typically within 1 hour.
unilateral weakness or sensory loss.
aphasia or dysarthria
ataxia, vertigo, or loss of balance
visual problems= sudden transient loss of vision in one eye (amaurosis fugax), diplopia, homonymous hemianopia
Examples of TIA mimics that REQUIRE exclusion?
hypoglycaemia
intracranial haemorrhage
Pt had TIA and is on anticoag or has similar RFs?
admit for urgent non-contrast head CT to exclude haemorrhage
Ix in TIA?
- MRI (diffusion weighted and blood sensitive sequences) to determine territory of ischaemia on same day as specialist assesment
- only do CT unless clinical suspicion of other diagnosis eg. haemorrhage as pt is taking anticoag
When would you do CT over an MRI in TIA?
if pt is on anticoag so suspect haemorrhage
all other cases do MRI
Pt who has had a TIA are at high risk for what?
further vascular events, esp in 1st few days
Antithrombotic medication following TIA?
1) resolved TIA, awaiting specialist review within 24hrs, low risk of bleeding= aspirin 300mg
2) been reviewed, now inital 21days when high risk for further events= aspirin + clopidogrel (both inital dose 300mg then 75mg od)
3) then long term secondary prevention after 21d= clopidogrel 75mg od
if not appropriate for DAPT then 300mg clopi loading dose then 75mg od
consider PPI with DAPT
ticagrelor + clopidogrel is an alternative
Medication following TIA?
1) 300mg aspirin immediately
2) DAPT for 21days after seen specialist
3) long term clopi 75mg od
4) atorvastatin 20-80mg daily
if have AF anticoag immediately after haemorrhage excluded
consider PPI with DAPT
DAPT= aspirin + clopidogrel (both loading dose of 300mg then 75mg od)
atherosclerosis in the carotid artery may be a source of emboli in some patients
patients who are considered candidates for carotid intervention should have carotid imaging performed within 24 hours of assessment
what is this?
carotid duplex ultrasound or either CT angiography or MR angiography
Trigeminal neuralgia occurs in what nerve?
in the distribution of one or more branches of the 5th (trigeminal) cranial nerve
How frequent does pain in trigeminal neuralgia occur?
may be infrequent (periods of remission may last yrs) or hundreds of times a day
In over 90% of cases, what is trigeminal neuralgia thought to be caused by?
vascular compression of the trigeminal nerve
What gender and age group is trigeminal neuralgia more common in?
women
increasing age, rare in <40yrs
Cx of trigeminal neuralgia?
depression
inability to eat and so weight loss
Features of trigeminal neuralgia?
paroxysmal attacks of pain which may be precipitated by trigger factors
- pain in trigeminal nerve distribution (cheek or lower jaw)
- severe= electric shock like, shap, shotting
- unilateral
- few secs-2mins and stops suddenly
- recurrent, refractory period between attacks
- episodic- may be remission for weeks or months, pain free periods shorten between episodes gradually
- provoked by light touch to face, eating, talking, exposure to cold air
- may have lacrimation, nasal congestion, ptosis, facial sweating (autonomic features)
‘Atypical’ or ‘mixed’ trigeminal neuralgia?
when there is persitent discomfort between episodes/paroxysms
Mx for trigeminal neuralgia?
carbamazepine= titrated until pain relieved, start at 100mg 2x d then titrate up in steps of 100-200mg every 2w until pain relieved;
typically 200mg 3/4x a day to prevent paroxysms of pain
when pain is in remission then reduce and gradually withdraw dose
if ineffective or contraindicated then specialsit advice
When is specialist referral needed in trigeminal neuralgia?
- severe pain or pain that limits ADLs (neuro or pain Mx clinic)
- atypical CP
- features suggestive of serious underlying cause
Serious conditions that can lead to compression of the trigeminal nerve or cause symptoms similar to those of trigeminal neuralgia?
- tumours= posterior fossa tumours, cavernous sinus masses, extracranial masses
- MS
- epidermoid, dermoid, or arachnoid cysts
- aneurysm or arteriovenous malformation
Red flag symptoms and signs in trigeminal neuralgia that may suggest more serious underlying cause?
Sensory changes.
Deafness or other ear problems.
History of skin or oral lesions that could spread perineurally.
Pain only in the ophthalmic division of the trigeminal nerve (eye socket, forehead, and nose), or bilaterally.
Optic neuritis.
Family history of multiple sclerosis.
Age of onset before 40 years.
Drug used for trigeminal neuralgia?
carbamazepine
start 100mg bd and titrate up
Describe trigeminal neuralgia?
unilateral disorder
brief electric shock-like pains, abrupt in onset and termination, limited to one or more divisions of the trigeminal nerve
evoked by light touch: washing, shaving, smoking, talking, and brushing the teeth (trigger factors), and frequently occurs spontaneously
small areas in the nasolabial fold or chin may be particularly susceptible to the precipitation of pain (trigger areas)
the pains usually remit for variable periods
Trigger ares for trigeminal neuralgia?
nasolabial fold or chin
Trigger factors for trigeminal neuralgia?
light touch:
- shaving
- brushing teeth
- washing
- smoking
- talking
can occur spontaneously
Suspected trigeminal neuralgia but failure to respond to treatment or atypical features (e.g. < 50 years old)?
refer to neurology
unilateral brief electric shock like pains in cheek/lower jaw
triggered by shaving or brushing teeth
lasts few secs then stops suddenly
few attacks a day then none for few weeks and then repeat
trigeminal neuralgia
Drug for trigeminal neuralgia?
carbamazepine (anticonvulsant so think electric shock pain use this)
Wernicke’s encephalopathy?
neuropsychiatric disorder caused by thiamine deficiency which is most commonly seen in alcoholics
Wernicke’s encephalopathy caused by def in what?
thiamine
Wernicke’s encephalopathy commonly seen in who?
alcoholics
Causes of Wernicke’s encephalopathy?
thiamine def in…
- alcoholics (common)
rare:
- persistent vomiting
- stomach ca
- dietary def
Wernicke’s encephalopathy triad?
ophthalmoplegia/nystagmus, ataxia and encephalopathy
What occurs in Wernicke’s encephalopathy?
petechial haemorrhages occur in a variety of structures in the brain including the mamillary bodies and ventricle walls.
Features of Wernicke’s encephalopathy?
- oculomotor dysfunction= nystagmus (most common), opthalmoplegia (lateral rectus palsy, conjugate gaze palsy)
- gait ataxia
- encephalopathy= confusion, disorientation, indifference and inattentiveness
- peripheral sensory neuropathy
In Wernicke’s encephalopathy triad, what is encephalopathy?
confusion, disorientation, indifference, and inattentiveness
In Wernicke’s encephalopathy triad, what is included in oculomotor dysfunction (nystagmus/opthalmoplegia)?
nystagmus (the most common ocular sign)
ophthalmoplegia: lateral rectus palsy, conjugate gaze palsy
Ix for Wernicke’s encephalopathy?
- decreased red cell transketolase
- MRI
Mx for Wernicke’s encephalopathy?
urgent replacement of thiamine
What if Wernicke’s encephalopathy is not treated?
Korsakoff’s syndrome may develop as well= called Wernicke-Korsakoff syndrome
Korsakoff’s syndrome?
- antero and retrograde amnesia
- confabulation
What is the difference between confabulation and confusion?
Confabulation is a memory error that involves creating false memories, while confusion is a state of being uncertain
confusion=wernickes
confab= korsakoff
Relationship between Wernicke’s encephalopathy and Korsakoff syndrome?
Wernicke’s:
- ataxia
- nystagmus, opthalmoplegia
- encephalopathy (confusion, disorientation)
Korsakoff’s:
- amnesia (antero and retrograde)
- confabulation)
If Wernicke’s is not treated with thiamine you get Wernicke’s + Korsakoff’s, called= Wernicke-Korsakoff
Delirium tremens, Wernicke’s and Korsakoff’s?
DT= >72hrs of alcohol withdrawl, confusion, Lulliputain hallucinations, paranoid delusions and tremor
W= acute thiamine def (eg. in alcoholics); triad= opthalmoplegia and nystagmus, ataxia and confusion
K= untreated WE; confabulation, irreversible ST memory loss, time disorientation
Chorea?
involuntary, rapid, jerky movements which often move from one part of the body to another.
Pathophysiology cause of chorea?
chorea caused by damage to basal ganglia, esp the caudate nucleus
Athetosis?
slow sinuous movements of the limbs
Chorea vs athetosis
C= rapid jerky movements
A= slower sinuous movements
Causes of chorea?
Huntington’s disease, Wilson’s disease, ataxic telangiectasia
SLE, anti-phospholipid syndrome
rheumatic fever: Sydenham’s chorea
drugs: oral contraceptive pill, L-dopa, antipsychotics
neuroacanthocytosis
pregnancy: chorea gravidarum
thyrotoxicosis
polycythaemia rubra vera
carbon monoxide poisoning
cerebrovascular disease
What drugs may cause chorea?
oral contraceptive pill, L-dopa, antipsychotics
Hemiballism?
Ballisic movements are involuntary, sudden, jerking movements which occur contralateral to the side of the lesion.
The ballisic movements primarily affect the proximal limb musculature whilst the distal muscles may display more choreiform-like movements
Hemiballism occurs when?
following damage to the subthalamic nucleus
Symptoms in Hemiballism decrease when?
when the pt is asleep
Treatment of Hemiballism?
antidopaminergic agents eg. haloperidol
Example of an antidopaminergic agent?
haloperidol
Huntington’s disease?
inherited neurodegenerative condition
progressive and incurable condition that typically results in death 20 years after the initial symptoms develop.
Type of inheritance in Huntingtons?
autosomal dominant
Genetics in Huntingtons?
trinucleotide repeat disorder: repeat expansion of CAG
there is a defect in the huntingtin gene on chromosome 4
autosomal dominant
anticipation
As Huntigton’s is a trinucleotide repeat disorder, what phenomenon may be seen?
anticipation
What is anticipation in Huntingtons?
where the disease is present at an earlier age and more severe in successive generations
Pathophysiology of Huntingtons?
trinucleotide repeat disorder: repeat expansion of CAG
results in degeneration of cholinergic and GABAergic neurons in the striatum of the basal ganglia
due to the defect in the huntingtin gene on chromosome 4
When do features of Huntingtons typically develop?
after 35yrs
Features of Huntingtons?
chorea
personality changes (e.g. irritability, apathy, depression) and intellectual impairment
dystonia
saccadic eye movements
dysarthria (speech difficulties)
rigidity
dysphagia
Saccadic eye movements, personality change (more irritable) and chorea?
Huntingtons
Gene mutation in huntingtons?
Trinucleotide repeat disorder invl a mutation in HTT gene on chromosome 4 which codes for huntingtin (HTT) protein.
What does trinucleotide repeats mean in Huntingtons?
repetitions of a sequence of three nucleotides (e.g., CAGCAGCAGCAGCAG).
Examples of trinucleotide repeat disorders?
- huntingtons
- fragile X syndrome
- Spinocerebellar ataxia
- Myotonic dystrophy
- Friedrich ataxia
Anticipation is a feature of what disorders?
Trinucleotide repeat disorders
Anticipation is where successive generations have more repeats in the gene resulting in what?
earlier age of onset
increased severity of disease
Dystonia?
abnormal muscle tone, leading to abnormal postures eg. Huntingtons
Rigidity?
increased resistance to passive movement of a joint
Diagnosis of Huntingtons?
genetic testing via a specialist genetic centre and involves pre and post-test counselling.
eg. The child of someone with the disease has a 50% chance of inheriting the faulty gene (it is autosomal dominant). They need to be 18 before they can decide whether to get tested. Your job is to provide information for the patient to make an informed decision for themselves, not to advise them whether to have a test or not. The outcome is usually that the patient will think about it further and return if they have further questions.
Mx for Huntingtons?
no Tx for slowing or stopping progression of disease
- genetic counselling
- breaking bad news effectively
- MDT
- physio
- SALT: if speech and swallowing difficulties
- SSRIs for depression
- Advanced care directives
- End of life care
What drug may be used for chorea in Huntingtons?
tetrabenazine
Role of physiotherapy in Huntingtons?
improve mobility, maintain joint function and prevent contractures
Contractures?
permanent or temporary shortening and stiffening of muscles, tendons, skin, and nearby tissues that limits normal movement in a joint or body part. Contractures can be caused by injury, nerve damage, scarring, or disuse of the muscles
Prognosis of Huntingtons?
progressive condition
life expectancy is around 10-20yrs after onset of symptoms
Typical cause of death in Huntingtons?
aspiration pneumonia or suicide
as disease progresses they become more frail andf susceptible to illness eg. infections, weight loss, falls, pressure ulcers
Dysarthria?
speech disorder that makes it difficult to form and pronounce words
Oculogyric crisis?
dystonic reaction to certain drugs or medical conditions
Features of Oculogyric crisis?
restlessness
agitation
involuntary upward deviation of the eyes
Oculopgyric crisis causes?
antipsychotics
metoclopramide
postencephalitic Parkinson’s disease
Oculopgyric crisis Mx?
cessation of causative medication if possible
IV antimuscarinic: benztropine or procyclidine
Restless leg syndrome (RLS)?
neuro disorder characterised by irresistible urge to move the limbs (usually legs) accompanied by uncomfotable sensations
Symptoms of restless leg syndrome typically worse when?
in the evenings, and often associated with sleep disturbance
Pathophysiology of RLS?
likely there is a dysfunction of the dopaminergic system
Causes of RLS (restless leg syndrome)?
idiopathic
may be secondary to:
- preg
- iron def
- stage 5 CKD
- drugs= some antidepressants, some antipsychotics, lithium
In idiopathic RLS the severity and frequency of symptoms typically…
increases over time
Diagnosis of RLS?
criteria must all be met:
1) urge to move the legs, usually accompanied by, or felt to be caused by, unpleasant sensations.
2) Symptoms begin or worsen during periods of rest or inactivity such as lying down or sitting.
3) Symptoms are partially or totally relieved by movement, such as walking or stretching, at least as long as the activity continues.
4) Symptoms during rest or inactivity only occur, or are worse, in the evening or night.
5) not primarily caused by another medical or behavioural condition (for example, leg cramps or habitual foot tapping).
Differential diagnosis of RLS?
nocturnal leg cramps, peripheral neuropathy, akathisia, intermittent claudication
Ix for RLS?
Clinical diagnosis criteria.
No specific to confirm, Ix may help identify underlying cause.
- serum ferritin (?iron def)
- renal function, FBC, thyroid function, blood glucose, vit B12
Mx for RLS?
- if underlying condition, treat this
if not… - self help advice
- reassurance and explanation
- moderate-severe= drug Tx
- neuro referral or sleep specialist is Tx unsuccessful or uncertain
Self help advice for RLS?
prevent attack= good sleep hygiene, reduce caffeine and alcohol, stop smoking, regular exercise
relieve attack= walking, stretching, apply heat with heat pads or hot bath, relaxation exercises, mental distraction at times of rest, massage affected limbs
Drug Tx for RLS if needed?
non-ergot dopamine agonist eg. ropinirole
or
alpha-2-delta ligan (pregabalin or gabapentin) off label
if painful, can use weak opioid instead eg. codeine
spontaneous, continuous lower limb movements that may be associated with paraesthesia
common
FHx may be present
akathisia
worse at night
RLS
Clinical features of RLS?
uncontrollable urge to move legs (akathisia). Symptoms initially occur at night but as condition progresses may occur during the day.
Symptoms are worse at rest
paraesthesias e.g. ‘crawling’ or ‘throbbing’ sensations
movements during sleep may be noted by the partner - periodic limb movements of sleeps (PLMS)
Akathisia?
uncontrollable urge to move legs/be still
Causes and associations of RLS?
there is a positive family history in 50% of patients with idiopathic RLS
iron deficiency anaemia
uraemia
diabetes mellitus
pregnancy
1st line drug Tx for RLS?
ropinirole
Ropinirole drug class?
dopamine agonist
Tics?
intermittent, stereotypical, repetitive, involuntary movements.
Around 15% of primary school age children have tics such as blinking and shrugging
Tx options for tics?
clonidine
atypical antipsychotics
Innervation of the face?
face is innervated by the trigeminal nerve (cranial nerve V), which has three main branches: the ophthalmic (V1), maxillary (V2), and mandibular (V3) nerves. Pain sensation from the face travels via these nerves to the trigeminal nucleus in the brainstem before being relayed to higher cortical centres. Various structures in the face can give rise to pain including skin, muscles, sinuses, teeth, eyes and neural tissue.
Differential diagnosis for facial pain?
- trigeminal neuralgia= severe electric shock like pain along one or more branches of trigeminal nerve
- sinusitis= accompanied by nasal discharge or congestion
- dental problems= dental caries or abscesses- localised facial pain
- tension-type headache= band like pressure around forehead, can extend into facial regions
- migraine= unilateral throbbing head and face pain associated with N, V or photophobia
- giant cell arteritis= older pts, new onset facial pain, can threaten vision
Head injury?
trauma to head other than superficial injuries to the face
traumatic brain injury- when head injury results in disturbance of normal brain function; can be classes as mild (aka concussion), moderate or severe
Cx of head injury?
death, disability, intracranial lesions (extradural or subdural haematoma, SAH), skull fracture, seizures, hypopituitarism, mood disorders, cognitive impairment
Aim of initial assessment of head injury?
rapidly identify RFs for intracranial Cx or cervical spine injury
Immediate admission to hospital following head injury when?
- GCS <15
- shock or signif injury
- dangerous cause of injury
- Hx of bleeding, coag disorders or current anticoag med
- current alcohol/drug intoxication
- any loss of consciousness after injury (even if fully alert now)
- post-traumatic seizure
- previous brain injury
- amnesia lasting >5mins
- persistent headache since injury
- suspected open or depressed skull fracture or tense fontanelle in child
- suspected basal skull fracture
- sigh of penetrating injury or visible trauma to scalp/skull
- <1yrs: bruise, swelling or laceration >5cm on head
- suspected cervical spine injury (full spine immobilisation arranged)
- irritability or altered behaviour, esp <5yrs
- clinical concern/concern from pt/relatives
- adult unable to stay with pt for 1st 24hrs after injury
- possible non-accidential injury, safeguarding concerns, vulnerable person affected
Mx for adults and children who are at low risk of intracranial Cx or cervical spine injury following head injury?
- responsible adult needs to stay with pt for 1st 24hrs following injury, alert for worrying S&S
- self-care advice
When to refer to appropriate specialist following head injury?
- persistent non-specific symptoms >3m
- another Cx or cause of head injury suspected
- concerns or uncertain about the nature or severity of symptoms
What is classed as a dangerous mechanism of head injury?
Fall from a height of greater than 1 metre or 5 stairs.
High-speed motor vehicle collision either as a pedestrian, cyclist, or vehicle occupant.
Rollover motor accident or ejection from a motor vehicle.
Accident involving motorized recreational vehicles or bicycle collision.
Diving accident.
Examine pt after head injury?
- Glasgow coma scale
- vital signs
- inspect for visible trauma
- cranial nerve exam incl pupil size and reactivity (fundoscopy: ?retinal haemorrhage or papulloedema)
- signs of focal neuro deficit
- signs of basal skull fracture
- neck tenderness= midline cervical spine tenderness may indicate cervical spine injury
- range of neck movements
Signs of basal skull fracture?
- clear fluid (?CSF) leaking from ear(s) or nose
- periorbital haematoma(s) with no damage around eyes= panda eyes
- bleeding from ear(s), blood behind the ear drum (haemotympanium), new deafness
- battle’s sign
Battle’s sign?
sign of basal skull fracture
bruising behind one or both ears over mastoid process- suggests fracture of the middle cranial fossa
Glasgow Coma Scale?
assesses level of consciousness of pt
15 highest (fully awake)
3 lowest (deep coma or death)
What makes up the GCS?
Eyes= max 4
Verbal= 5
Motor= 6
so best is E4, V5, M6 = 15
GCS: eyes?
open spontaneously= 4
open in response to voice= 3
open to pressure (finger tip stimulus)= 2
doesn’t open= 1
GCS: verbal?
orientated and converses normal= 5
confused & disorientated= 4
inappropriate words= 3
incomprehensible sounds= 2
makes no sounds= 1
GCS: motor?
obeys simple commands= 6
localises to stimulus= 5
flexion or withdrawl in response to painful stimulus= 4
abnormal flexion in response to painful stimulus= 3
extension in response to painful stimulus= 2
makes no movement in response to pain= 1
GCS: how to assess verbal response in pre-verbal infant?
coos & babbles= 5
irritable cries= 4
cries to pain= 3
moans to pain= 2
no response= 1
Full cervical spine immobilisation should be attempted for pt with head injury and any RFs for cervical spinal injury including:
- GCS <15 on initial assessment
- neck pain/tenderness
- focal neuro deficit
- paraesthesia in extremities
Why is it important to manage head injury initially with advanced life support?
as inadequate cardiac output will compromise CNS perfusion irrespective of the nature of cranial injury
Extradural haematoma?
bleeding into space between dura mater and skull
Extradural haematoma often results from what?
acceleration-deceleration trauma or blow to the side of the head
Where do extradural haematomas typically happen?
temporal region (pterion region where all skull bones join- thin and weak)
skull fractures cause a rupture of middle meningeal artery
Features of extradural haematoma?
raised ICP and some pts may exhibit a lucid interval
Subdural haematoma?
bleeding into the outermost meningeal layer
may be acute or chronic
Where do subdural haematomas commonly occur?
around frontal and parietal lobes
bridging veins rupture
RFs for subdural haematoma?
old age (bridging veins stretched) and alcoholism (bridging veins thin)
Features of subdural haematoma?
slower onset of symptoms than extradural
Why do subdural haematomas have slower onset than extradural?
bridging veins have lower pressure so delayed onset of symptoms (days-weeks)
Primary brain injury may be what?
focal (contusion/ haematoma) or diffuse (diffuse axonal injury)
Diffuse axonal injury occurs as a result of mechanical shearing following deceleration, causing disruption and tearing of axons
Intra-cranial haematomas can be what?
extradural, subdural or intracerebral
contusions may occur adjacent to (coup) or contralateral (contre-coup) to the side of impact
When does secondary brain injury?
when cerebral oedema, ischaemia, infection, tonsillar or tentorial herniation exacerbates the original injury.
The normal cerebral auto regulatory processes are disrupted following trauma rendering the brain more susceptible to blood flow changes and hypoxia
Cushings reflex?
physiological response to increased intracranial pressure (ICP) that can be a sign of impending brain herniation or death (often occurs late)
Cushing’s reflex triad?
Widened pulse pressure (increasing systolic, decreasing diastolic)
Bradycardia (decreased heart rate)
Irregular respirations (Cheyne-Stokes breathing)
What can be used when there is life threatening rising ICP eg. following head injury, extradural haematoma?
IV mannitol
diffuse cerebral oedema= decompressive craniotomy
Mx of depressed skull fractures that are open?
require formal surgical reduction and debridement, closed injuries may be managed nonoperatively if there is minimal displacement
Head injury: when is ICP monitoring appropriate?
in those with GCS 3-8 and normal CT scan
Head injury: when is ICP monitoring mandatory?
in those with GCS 3-8 and normal CT scan
Head injury: what may hyponatraemia most likely be due to?
SIADH
Minimum of cerebral perfusion pressure in adults?
70mmHg
Minimum cerebral perfusion pressure in children?
40-70mmHg
Pupil size= unilaterally dilated
Light response= sluggish or fixed
Interpretation?
3rd nerve compression secondary to tentorial herniation
Pupil size= bilaterally dilated
Light response= sluggish or fixed
Interpretation?
poor CNS perfusion
bilateral 3rd nerve palsy
Pupil size= unilaterally dilated or equal
Light response= cross reactive (Marcus-Gunn)
Interpretation?
optic nerve injury
Pupil size= bilaterally constricted
Light response= may be difficult to assess
Interpretation?
opiates
pontine lesions
metabolic encephalopathy
Pupil size= unilaterally constricted
Light response= preserved
Interpretation?
sympathetic pathway disruption
Following head injury, what pts need a CT head within 1hr?
GCS < 13 on initial assessment
GCS < 15 at 2 hours post-injury
suspected open or depressed skull fracture
any sign of basal skull fracture (haemotympanum, ‘panda’ eyes, cerebrospinal fluid leakage from the ear or nose, Battle’s sign).
post-traumatic seizure.
focal neurological deficit.
more than 1 episode of vomiting
Following head injury, what pts need CT head within 8hrs?
for adults with any of the following risk factors who have experienced some loss of consciousness or amnesia since the injury:
- age 65 years or older
any history of bleeding or clotting disorders including anticogulants - dangerous mechanism of injury (a pedestrian or cyclist struck by a motor vehicle, an occupant ejected from a motor vehicle or a fall from a height of greater than 1 metre or 5 stairs)
- more than 30 minutes’ retrograde amnesia of events immediately before the head injury
Pt on warfarin sustained a head injury with no indications for a CT head?
perform CT head within 8hrs of injury
Intracerebral (or intraparenchymal haemorrhage)?
collection of blood within the parenchyma of the brain
Causes/RFs of intracerebral haematoma (haemorrhage)?
hypertension, vascular lesion (e.g. aneurysm or arteriovenous malformation), cerebral amyloid angiopathy, trauma, brain tumour or infarct (particularly in stroke patients undergoing thrombolysis).
Features of intracerebral haematoma?
present similarly to ischaemic stroke (which is why it is crucial to get CT head in all stroke pts prior to thrombolysis)
or present with decrease in consciousness
Ix for intracerebral haematoma?
contrast CT head= hyperdensity (bright lesion) within the substance of the brain
Mx of intracerebral haematoma?
often conservative under the care of stroke physicians, but large clots in patients with impaired consciousness may warrant surgical evacuation.
What does extradural (epidural) haematoma look like on CT head?
lemon shape (biconvex)
hyperdense
between dura and skull
CONFINED to suture lines
associated features- skull fractures
What does subdural haematoma look like on head CT?
banana shape (cresent)
hyperdense if acute, hypodense if chronic
between dura and arachnoid
associated features= trauma, anticoag, elderly, alcoholics
What does SAH look like on CT head?
irregular shape
hyperdense
location- sulci, cisterns
associated features- aneurysms, trauma
What does intracerebral haematoma look like on head CT?
round/irregular shape
hyperdense (acute), may be surrounded by hypodense oedema in more chronic stages
brain parenchyma
associated features- HTN, trauma, tumour ect
What haematoma is confined by suture lines?
extradural
What haematoma may be more chronic, more common in elderly pts & on anticoags?
subdural
Hemotympanum?
blood visible behind tympanic membrane in ear eg. in basal skull fracture
What should you NOT do in basal skull fractures?
insert NG or nasotracheal tube as risk of tube entering the cranial cavity if fracture involves cribriform plate or ethmoid bone (anterior cranial fossa); can lead to brain injury or infection
Pneumochephalus?
presence of air within cranial cavity, can be caused by basal skull fracture allowing air to enter intracranial space (Cx of basal skull fracture)
Cluster headaches?
most common ‘trigeminal autonomic cephalalgia’ and characterised by frequently recurring, localised short lasting but severe headache, usually accompanied by autonomic symptoms
What type of symptoms can accompany headache in cluster headaches?
autonomic symptoms
Cluster headaches frequency of attacks?
usually last between 2w and 3m (clusters or bouts)
- Episodic (90%)= occur in periods lasting from 7d to 1yr and separated by pain free periods lasting at least 3m
- Chronic= occur for 1yr or longer without remission, or with remission periods lasting <3m
What may cluster headaches be due to?
nuerovascular, genetic and environmental factors (alcohol, smoking, exposure to volatile substances)
Cx of cluster headaches?
signif impact of QOL: work, home, relationships
prolonged and severe= suicidal ideation
Cluster headaches with increasing age?
may be less frequent bouts and longer periods of remission between bouts with increasing age
ICHD classifies cluster headaches as what?
at least 5 attacks of severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15mins-3hrs (untreated) & either or both of:
- sense of restlessness or agitiation
- 1 of ipsilateral= lacrimation or conjunctival injection; nasal congestion/rhinorrhea; eyelid swelling; forehead and facial sweating or flushing; sensation of fullness in ear; miosis and/or ptosis
Misosis?
XS pupillary constriction
Suspected cluster headaches?
specialist advice or arrange urgent neuro referral for 1st suspected bout to confirm diagnosis
When to refer to neuro/specialist advice for cluster headaches?
- uncertain
- atypical symptoms
- ?managing acute attacks
- verapamil being considered for preventative Tx
- symptoms persist despite Tx
- needs Tx and is pregnant
What should pts avoid in acute attacks of cluster headaches?
oral triptans or other oral analgesia
Mx for cluster headaches?
- assess triggers
- acute attacks= sumcut sumatriptan or intranasal sumatriptan/zolmitriptan
- short burst high flow 100% O2 for acute attacks
Average amount of cluster headaches per ‘bout’?
may experience 1–3 attacks per day (up to 8) and they usually occur daily for 2–3 months at a time. They usually last 2 weeks to 3 months, and most often occur every 1–2 years.
Potential triggers for cluster headaches?
alcohol, smoking, diet, stress, anxiety, mood disorders
Drug Tx for acute attacks of cluster headaches?
subcut sumatriptan= 6mg one dose, if respond then 6mg after 1hr if recurs; max 12mg a day
or
sumatriptan intranasal spray (18yrs-65)= 10-20mg into 1 nostril, if recurs can take another after 2hrs (max 40mg a d)
if multiple attacks:
+ high flow 100% oxygen 12-15L per min via non-rebreather face mask for 15-20mins
Drug prophylaxis for cluster headaches that can be initiated by specialist?
verapamil
What to do if verapamil is being considered for preventative Tx for cluster headaches?
needs ECG monitoring before and during Tx
Typical clusters in cluster headaches?
clusters lasting several weeks, with clusters themselves typically once a yr
Are cluster headaches severe?
known to be one of the most painful conditions
RFs for cluster headaches?
men (3:1)
smokers
alcohol may trigger attack
Features of cluster headaches?
intense sharp, stabbing pain around one eye:
- pain typical occurs once or twice a day, each episode lasting 15 mins - 2 hours
- the patient is restless and agitated during an attack due to the severity
- clusters typically last 4-12 weeks
- accompanied by redness, lacrimation, lid swelling
- nasal stuffiness
- miosis and ptosis in a minority
Ix for cluster headaches?
- clinical
- MRI with gadolinium contrast= rule out underlying brain lesions
Overview of cluster headache Mx?
acute:
- 100% oxygen (80% response rate within 15 minutes)
- subcutaneous triptan (75% response rate within 15 minutes)
prophylaxis= verapamil
Trigeminal autonomic cephalgia?
term used to group number of conditions: cluster headache, paroxysmal hemicrania and short-lived unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT)
paroxysmal hemicrania responds very well to
indomethacin
Patient who initially loses, briefly regains and then loses again consciousness after a low-impact head injury. The brief regain in consciousness is termed the ‘lucid interval’. What condition may this be?
extradural haematoma
In extradural haematoma, when will the lucid interval be lost?
due to expanding haematoma and brain herniation
haematoma expands the uncus of the temporal lobe herniates around the tentorium cerebelli and the patient develops a fixed and dilated pupil due to the compression of the parasympathetic fibers of the third cranial nerve.
What may happen to pts pupils if they have an extradural haematoma and why?
fixed and dilated pupil due to the compression of the parasympathetic fibers of the third cranial nerve.
happens whenhaematoma expands the uncus of the temporal lobe herniates around the tentorium cerebelli
On CT head= biconvex (or lentiform), hyperdense collection around the surface of the brain. They are limited by the suture lines of the skull. Condition?
extradural haematoma
Extradural haematoma Mx?
no neurological deficit, cautious clinical and radiological observation is appropriate.
The definitive treatment is craniotomy and evacuation of the haematoma.
Most common cause of extradural haematoma?
‘low-impact’ trauma (e.g. a blow to the head or a fall). The collection is often in the temporal region since the thin skull at the pterion overlies the middle meningeal artery and is therefore vulnerable to injury.
Trauma to pterion?
think extradural haematoma
(pterion overlies middle meningeal artery)
FIbromyalgia?
syndrome of widespread pain throughout the body with tender points at specific anatomical sites
cause is unknown
Gender and age fibromyalgia is most common?
women 5x more
30-50yrs
Features of fibromyalgia?
- chronic pain= multiple site, sometimes ‘pain all over’
- cognitive impairment= fibro fog
- sleep disturbance, headaches, dizziness
‘pain all over’
fibromyalgia
Diagnosis of fibromyalgia?
clinical:
American College of Rheumatology classification criteria= 9 pairs of tender points on the body, if pt is enter in at least 11/18 then diagnosis likely
Mx of fibromyalgia?
- psychosocial and MDT approach
- explanation
- aerobic exercise
- CBT
- meds= pregabalin, duloxetine, amitriptyline
Summarise temporal arteritis?
Typically patient > 60 years old
Usually rapid onset (e.g. < 1 month) of unilateral headache
Jaw claudication (65%)
Tender, palpable temporal artery
Raised ESR
Headache differentials= single acute episode?
meningitis
encephalitis
subarachnoid haemorrhage
head injury
sinusitis
glaucoma (acute closed-angle)
tropical illness e.g. Malaria
Headache differentials= chronic?
chronically raised ICP
Paget’s disease
psychological
ICHD stands for?
international classification of headache disorder
Medication overuse headache defined by ICHD?
Headache occurring on 15 or more days per month in a person with a pre-existing primary headache disorder, which develops as a consequence of regular overuse of one or more drugs that can be taken for acute and/or symptomatic treatment of headache, for more than 3 months. It usually, but not always, resolves after the overused medication is stopped.
How many meds do you need to take that may cause medication overuse headache?
Ergotamines, triptans, opioids, or combination analgesics are taken on 10 days or more per month.
Simple analgesics such as paracetamol, NSAIDs, or aspirin (either alone or in any combination) are taken on 15 days or more per month.
usually for >3m
Type of headache disorder in medication overuse headache?
chronic
pre-exisiting headache is usually migraine (60-80%) or rarely tension type
generally of the same phenotype as the primary headache
Cx of medication overuse headache?
stress, anxiety, depression, sleep disturbance, increased risk of transition from episodic to chronic migraine if untreated
Mx for medication overuse headache?
stop overused medication(s) abruptly (or gradual if strong opioid) for at least 1m, headache may worsen initially and may be other initial withdrawl symptoms
keep headache diary
review 4-8w after withdrawl
advice to prevent relapse= prophylactic meds for underlying primary headache disorder, restrict future use of acute meds
When to refer to neuro/specialist advice in medication overuse headaches?
- uncertain or atypical
- uncertain how to manage
- previous drug withdrawl attempts unsuccessful
- person is overusing strong opioids, barbiturates or tranquilizers, or signif comorbidities needing specialist Mx
How long until headache improves after stopping meds for medication overuse headache?
usually improves 1-2w after but recovery may continue for 2-3m
Advice on acute meds once recovered from medication overuse headache (finished withdrawl)?
if needed 2m following withdrawl, restrict to no more than 2d a w to reduce to risk of recurrence
What drugs are more at risk for medication overuse headache?
opioids and triptans
How quick to withdraw meds in medication overuse headache?
simple analgesics and triptans= abrupt (may initially worsen headache)
opioid= gradually
Withdrawl symptoms in medication overuse headache?
V, hypotension, tachycardia, restlessness, sleep disturbance, anxiety
Subdural haemorrhage: blood is not within the substance of the brain and therefore is called what?
extra-axial or extrinsic lesion
Subdural haemorrhage uni or bilateral?
can be either
Acute subdural haematoma?
Symptoms usually develop within 48 hours of injury, characterised by rapid neurological deterioration
Subacute subdural haematoma?
Symptoms manifest within days to weeks post-injury, with a more gradual progression.
Chronic subdural haematoma?
Common in the elderly, developing over weeks to months. Patients may not recall a specific head injury.
What is common in chronic SDH?
Patients frequently exhibit a lucid interval followed by a gradual decline in consciousness.
Altered Mental Status: Ranging from mild confusion to deep coma. Fluctuations in the level of consciousness are common in what type of haematoma?
subdural
Headache in subdural haematoma?
often localised to one side, worsening over time
What may occur in subdural haemorrhage, esp in acute or expanding haematomas?
seizures
Possible physical exam findings in subdural haematoma?
Papilloedema: Indicates raised intracranial pressure.
Pupil Changes: Unilateral dilated pupil, especially on the side of the haematoma, indicating compression of the third cranial nerve.
Gait Abnormalities: Including ataxia or weakness in one leg.
Hemiparesis or Hemiplegia: Reflecting the mass effect and midline shift.
Possible behavioural and cognitive changes in subdural haematoma?
Memory Loss: Especially in chronic SDH.
Personality Changes: Irritability, apathy, or depression.
Cognitive Impairment: Difficulty with attention, problem-solving, and other executive functions.
3 associated features of subdural haematoma?
Nausea and Vomiting: Secondary to increased intracranial pressure.
Drowsiness: Progressing to stupor and coma in severe cases.
Signs of Increased Intracranial Pressure: Such as bradycardia, hypertension, and respiratory irregularities (Cushing’s triad).
Bradycardia, hypertension and resp abnormalities?
cushings triad= raised ICP
Summary of acute subdural haematoma?
collection of fresh blood
mostly commonly caused by high-impact trauma (extradural more low impact)
often other underlying brain injuries due to trauma
presentation ranges from incidential finding in trauma to severe coma and coning due to herniation
Ix for acute subdural haematoma?
head CT= hyperdense crescent collection not limited by suture lines; large ones will push on the brain (mass effect) and cause midline shift or herniation
Mx of acute subdural haematomas?
small or incidental= observation
surgery= monitoring ICP and decompressive craniectomy
Coning?
serious medical emergency that occurs when the cerebellar tonsils move through the foramen magnum and compress the medulla oblongata
Coning can lead to death when the brain stem stops working. The brain stem controls vital functions like consciousness, awareness, breathing, and heart rate and blood pressure regulation.
Intractable headache
Head tilt
Neck stiffness
Decreased level of consciousness
Flaccid paralysis
Dilated pupils
Drowsiness
Difficulty concentrating
High blood pressure or blood pressure that’s too low
Loss of reflexes or increased reflexes
Seizures
Summarise chronic subdural haemorrhage?
collection of blood in subdural space that has been present for w-m
rupture of small bridging veins rupture and cause slow bleeding
elderly and alcoholics more at risk as have brain atrophy so fragile or taut veins
Presentation is typically a several week to month progressive history of either confusion, reduced consciousness or neurological deficit.
What type of haemorrhage can shaken baby syndrome cause?
subdural
infants have fragile bridging veins that can rupture
Ix for chronic subdural haematoma?
CT= crescentic in shape, not restricted by suture lines and compress the brain (‘mass effect’). Hypodense (dark) compared to the substance of the brain.
Mx of chronic subdural haematoma?
incidental or small with no neuro deficit= conservative with the hope it will dissolve with time
if confused, neuro deficit or severe imaging findings= decompression with burr holes
Giant cell arteritis (GCA)?
chronic vasculitis characterised by granulomatous inflam in walls of medium and large arteries
typically >50yrs
What does GCA stand for?
giant cell arteritis
Cx of GCA?
- vision loss
- large artery Cx (aortic aneurysms, aortic dissection, large artery stenosis)
- CVD eg. stroke
How to prevent Cx of GCA?
Tx with corticosteroids
Can pt with GCA get relapses?
common, occur in 50% of cases
When to suspect GCA?
pt is 50yrs + with:
- new onset headache, unilateral in temporal area and/or
- temporal artery abnormality eg. tenderness, thickening or nodularity
Is GCA serious?
medical emergency
S&S of GCA?
- new onset headache, unilateral, temporal area
- vision loss or diplopia
- scalp tenderness
- intermittent jaw claudication
- fever, fatigue, anorexia, weight loss, depression
- features of polymyalgia rheumatica
- neuro features eg. stroke
- bruits, BP diff between arms or intermittent limb claudication
What condition is associated with GCAA?
polymyalgia rheumatica
Pt with new visual loss or diplopia?
urgent (same day) assessment; may have high dose corticosteroids whilst awaiting opthalmology transfer
?GCA
Suspected GCA?
- same day urgent assessment by opthalmology if new vision loss/diplopia
- other refer to secondary care using local fast track GCA pathway= rheumatologist review same day or within 3d
- strongly suspected= immediately give high dose corticosteroids
- can do FBC, ESR, CRP but don’t delay referral
Prognostic factors and cormorbidities relevant to Tx of GCA?
infection, diabetes, HTN, glaucoma, peptic ulcer, bone fracture risk
consider preventative measures= bone and/or GI protection
Tx for GCA?
- visual symptoms= 60-100mg oral pred for up to 3d
- no visual symptoms= 40-60mg oral pred per day
continue until GCA symptoms and inflam markers resolve
once in clinical remission, tapering dose down; Tx duration usually 1-2yrs
Why regular review in GCA?
to monitor for disease relapse and steroid-related adverse effects
Relapse of GCA?
Depending on the clinical situation an increase in corticosteroid or addition of adjunctive treatment may be indicated.
Relapse with new-onset visual disturbance requires urgent (same day) assessment by ophthalmology.
Headache in GCA?
most common symptom; may not be present; temporal in location but may vary
may have tenderness, thickening or nodularity of temporal artery; sometimes overlying skin is red and pulsation may be reduced or absent
Vision disturbance in GCA?
loss of vision, diplopia or changes in colour vision
may be transient or permanent
What fundoscopic findings that are not specific to GCA but may be present?
pallor and oedema of optic disc, ‘cotton wool’ patches and small haemorrhages in retina (due to vision loss)
Intermittent jaw claudication in GCA?
pain in jaw muscles (masseter after mins of chewing)
intermitted claudication may affect tongue or muscles involved in swallowing
Jaw symptoms in GCA vs MG?
GCA=pain after mins of chewing
MG= fatigue when chewing
Features of polymyalgia rheymatica?
proximal muscle pain, stiffness and tenderness
in 40% pts with GCA
Neuro and resp/ENT features in GCA?
Neuro (30%)= mono/polyneuropathy of arms or legs; upper cranial nerve palsies
Resp/ENT occasionally= audio vestibular symptoms, hoarseness, cough, sore throat
Differential diagnosis of GCA?
- herpes zoster
- migraine/cluster headaches
- acute angle closure glaucoma
- retinal TIA and embolic visual deficits
- temporomandibular joint pain, sinus disease and ear problems
- cervical spondylosis, upper cervical spine disease
- ankylosing spondylitis
- malignancy eg. myeloma, cranial
- connective tissue disease eg. SLE
- vasculitis= grandulomatosis with polyangiitis, Churg-Strauss syndrome, polyarteritis nodosa
Suspected GCA, what MUST you give immediately to prevent vision loss?
oral pred
60-100mg up to 3d if already vision loss (prevent permanent)
no vision loss= 40-60mg per d
Another name for GCA?
temporal arteritis
Main features in GCA?
- typically >60yrs
- rapid onset <1m
- headache
- jaw claudication
- vision loss, diplopia
- tender palpable temporal artery
- 50% will have PMR
- lethargy, low grade fever, night sweats, depression, anorexia
Why is vision testing in GCA key and why?
- anterior ischemic optic neuropathy accounts for the majority of ocular complications. It results from occlusion of the posterior ciliary artery (a branch of the ophthalmic artery) → ischaemia of the optic nerve head. Fundoscopy typically shows a swollen pale disc and blurred margins
- may result in temporary visual loss - amaurosis fugax
- permanent visual loss is the most feared complication of temporal arteritis and may develop suddenly
- diplopia may also result from the involvement of any part of the oculomotor system (e.g. cranial nerves)
Ix for GCA?
-temporal artery biopsy= skip lesions
- raised inflam markers (ESR >50, CRP may be high)
- creatine kinease and EMG normal
Summarise Tx for GCA?
- urgent high dose corticosteroids before temporal artery biopsy
- urgent opthalmology review (same day)= visual damage often irreversible
- bisphosphonates (bone protection as on steroids)
- may need GI protection
In GCA, if there is evolving vision loss what should you give?
= if evolving visual loss give IV methylpred before starting high dose oral pred
Tension-type headache?
most common primary headache disorder, not associated with another underlying condition
ICDH categorises tension headaches according to what?
frequency of attacks
Types of tension headaches?
- infrequent episodic= <1d of headache per month (usually self-limiting)
- frequent episodic= at least 10 episodes of headache occuring on <15d per month, for more than 3m
- chronic= frequent episodic attacks, with 15d+ of headaches per m, for more than 3m, in absence of medication overuse
Cause/mechanism of tension headaches?
peripheral pain mechanisms may play role in episodic TH and central pain mechanisms & heightened sensitivity to pain more important in chronic
Cx of tension headaches?
frequent episodes can impact QOL icl work, home, school
Features of tension headaches?
- recurrent episodes of headache lasting 30mins-7d not associated with N/V
- may be associated with no more than one of photophobia or phonophobia and..
- at least 2 of: bilateral, pressing, tightening, non-pulsating; mild or moderate intensity; not aggravated by routine physical activity (walking or climbing stairs)
- neuro exam normal and no other cause found
Mx of episodic tension headache?
- reassurance
- risk of medication overuse headache with acute Tx
- simple analgesia= paracetmamol, NSAIDs, aspirin but avoid opioids
- triggers= stress, neck pain, sleep disorders
Mx of frequent episodic or chronic tension type headache?
- acupuncture may help
- physio, exercise, CBT and/or relaxation techniques
- trial amitriptyline drug prophylaxis
- headache diary
What drug could be considered prophylactic for tension headaches (frequent episodic or chronic)?
amitriptyline
10mg every night, increase by 10-25 every 1-2w up to max dose 150mg
trial for 2-3m
if good response for min 6m, consider gradually reducing
When to refer to neuro or specialist advice for tension headaches?
- red flags
- serious underlying cause suspected
- uncertain
- persist despite Tx
Tight band around head type of headache?
tension headache
Tension vs migraine?
M= typically unilateral
T= bilateral
CP of tension headache?
often described as a ‘tight band’ around the head or a pressure sensation.
tends to be of a lower intensity than migraine
not associated with aura, nausea/vomiting or aggravated by routine physical activity
may be related to stress
may co-exist with migraine
Radiculopathy?
neuro state in which conduction is limited or blocked along a spinal nerve or its roots
different to radicular pain but commonly occur together
radicular pain?
caused by compression of nerve root due to cervical disc herniation or degenerative spondylotic changes, can occur without compression (eg. inflam)
Radiculopathy vs radicular pain?
radiculopathy= conduction limited or blocked along spinal nerve or roots
radicular pain= usually due to compression of nerve root eg. due to cervical disc herniation or degenerative spondylotic changes but can occur due to inflam of the nerve
Cervical radiculopathy?
pain and weakness and/or numbness in 1 or both of upper extremities, which corresponds to dermatome of the involved cervical nerve root
Cervical radiculopathy pain?
often occurs alongside neck pain secondary to compression or irritation of nerve roots in cervical spine
Where can pt get pain in cervical radiculopathy?
neck
shoulders
upper back
chest
Most common causes of cervical radiculopathy?
degenerative changes eg. cervical disc herniation and spindylosis
cervical radiculopathy most common in what age group?
50-54yrs
Around 88% of pts with cervical radiculopathy will improve when?
within 4 weeks with non-operative Mx
most will improve regardless of Tx
What tests can be done to help identify cervical radiculopathy?
1) The Spurling test.
2) Arm squeeze test.
3) Axial traction — a combination of a positive Spurling’s test, axial traction test, and arm squeeze test increases the likelihood of cervical radiculopathy.
4) Upper limb neurodynamic tests — a combination of four neurodynamic tests and an arm squeeze test can rule out cervical radiculopathy.
Diagnosis of cervical radiculopathy?
clinical
don’t usually need cervical x-ray or imaging/Ix
cervical radiculopathy: Mx if pt has neck pain for less than 4-6w and no objective neuro signs eg. ?cervical radiculopathy
- reassurance and advice
- oral analgesia
- consider= amitriptyline, duloxetine, pregabalin or gabapentin
- consider referral for physio
cervical radiculopathy: Mx if pt has neck pain 4-6w or more or objective neuro signs?
refer for MRI and consider invasive procedures eg. interlaminar cerical epidural injections, transforaminal injections or spinal surgery
cervical radiculopathy: what to consider examining for if pt presents with neck pain?
Kernig’s sign (painful/resisted extension of leg bent at hip and knee) and Brudzinski’s sign (reflective flexion of the knees when the person is on his/her back and the neck is bent forwards) to demonstrate nuchal rigidity if meningitis is suspected
When may MRI be indicated in pts with cervical radiculopathy?
complex cervical radiculopathy eg. high suspicion of myelopathy or abscess, persistent or progressive neuro findings, failure to improve after 4-6w of conservative Tx
Symptoms of cervical radiculopathy?
- pain in neck, shoulder +/or arm that approximates to a dermatome
- pain may wake pt up at night
- sensory= shooting pains, numbness, hyperaesthesia
sensory>motor
- motor= muscle weakness, spasm
Most common nerve root affected in cervical radiculopathy?
C7 then C6
retro-orbital and temporal pain suggest referral from upper levels (C1 to C3) and can mimic GCA
Signs of cervical radiculopathy?
- postural asymmetry= head to one side or flexed, as decompresses nerve root
- neck movements= restricted, sharp pain may radiate to arms (esp on extension or bending or turning to affected side)
- neuro problems= upper limb weakness, paraesthesia, dermatomal sensory or motor deficit, diminished tendon reflexes at appropriate level
Atypical signs of cervical radiculopathy?
deltoid weakness, scapular winging, weakness of intrinsic muscles of the hand, chest or deep breast pain, headaches
cervical radiculopathy: if postural asymmetry is long-standing, what may be present?
muscle weakness
cervical radiculopathy: nerve root symptoms should normally arise from what?
single nerve root, if there is involvement of more than one it suggests a more widespread neuro disorder
Common cervical radiculopathies…
C5:
- muscle weakness?
- reflex changes?
- sensory changes?
muscle weakness= shoulder abduction and flexion, elbow flexion
reflex changes= biceps
sensory changes= lateral arm
Common cervical radiculopathies…
C6:
- muscle weakness?
- reflex changes?
- sensory changes?
muscle weakness= elbow flexion, wrist extension
reflex changes= biceps, supinator
sensory changes= lateral forearm, thumb, index finger
Common cervical radiculopathies…
C7:
- muscle weakness?
- reflex changes?
- sensory changes?
muscle weakness= elbow extension, writst flexion, finger extension
reflex changes= triceps
sensory changes= middle finger
Common cervical radiculopathies…
C8:
- muscle weakness?
- reflex changes?
- sensory changes?
muscle weakness= finger flexion
reflex changes= none
sensory changes= medial side lower forearm, ring and little fingers
Common cervical radiculopathies…
T1:
- muscle weakness?
- reflex changes?
- sensory changes?
muscle weakness= finger abduction and adduction
reflex changes= none
sensory changes= medial side upper forearm, lower arm
Retro-orbital and temporal pain referral from where?
C1 to C3 cervical radiculopathy but can mimic GCA
Red flags in cervical radiculopathy?
- malignancy, infection or inflam= B symptoms; lymphadenopathy, excruciating pain, tenderness over vertebral body, N&V, visual loss, erythema
- Cervical myelopathy
- headaches, facial pain, ataxia, vertigo, before age 20 or after 55yrs, altered cognitive state
- Hx of ca, TB, arthritis, immunosupression, drug abuse, AIDs
- Hx of violet trauma (minor trauma may fracture spine in pts with osteoporosis
- RFs for oestoporosis
Red flags of cervical radiculopathy in pts under 20yrs and over 50yrs?
under 20:
Altered hair distribution.
Birthmarks.
Congenital abnormalities.
Family history.
Infections related to substance misuse.
Skin tags — these are associated with congenital abnormalities, such as thyroglossal duct cysts or spinal dysraphism.
Over 50:
History of cancer.
Vascular disease.
Mx of cervical radiculopathy if been present for less than 4-6w and no objective neuro signs?
- reassure
- encourage activity and return to normal lifestyle eg. work asap
- do not drive if range of motion of neck is restricted
- firm pillow to support neck; do not use cervical collars as may prolong symptoms (may prolong symptoms)
- oral analgesia= NSAIDs, paracetamol, codeine; consider amitriptyline, duloxetine, pregabalin or gabapentin for neuropathic pain
- consider referral for physio= strengthening and stretching exercises
- follow up review
Mx of cervical radiculopathy if been present for more than 4-6w or objective neuro signs?
- refer for MRI and to consider invasive procedures, eg. interlaminar cervical epidural injections, transforaminal injections, or spinal surgery.
- ?surgery
Indication for surgery in cervical radiculopathy?
unremitting radicular pain despite 6–12 weeks of conservative treatments, symptoms are disabling, or there is progressive motor weakness, and where MRI shows nerve root compression.
Cervical myelopathy vs radiculopathy?
Cervical myelopathy= caused by compression of the spinal cord, this condition can affect the entire spinal cord and can cause weakness, difficulty walking, and problems moving small objects.
Cervical radiculopathy= caused by compression or irritation of a nerve in the neck, this condition is often called a “pinched nerve”. Symptoms include pain that radiates into the shoulder, as well as muscle weakness and numbness that travels down the arm and into the hand
Cervical spondylosis?
degenerative condition affecting the cervical spine
basically osteoarthritis of the cervical vertebral bodies
If cervical spondylosis causes the spinal canal to be narrowed what can happen?
can compress on spinal cord resulting in neuro dysfunction
How common do pts with cervical spondylosis have myelopathy?
5-10% of pts
Features of cervical spondylitic myelopathy?
a variety of motor weakness, sensory loss and bladder/bowel dysfunction may be seen
neck pain
wide-based, ataxic or spastic gait
upper motor neuron weakness in the lower legs - increased reflexes, increased tone and upgoing plantars
bladder dysfunction e.g. urgency, retention
Guillain-Barre syndrome?
immune mediated demyelination of peripheral nervous system often triggered by an infection
What infection most commonly causes Guillain-Barre?
Campylobacter jejuni
Initial symptoms of Guillain-Barre?
65% have back/leg pain in initial stages of illness
Characteristic features of Guillain-Barre?
progressive ascending symmetrical weakness of all the limbs; legs are affected first
- reflexes reduced or absent
- sensory symptoms (distal paraesthesia), very few sensory signs.
progressive ascending symmetrical weakness of all the limbs; legs are affected first; following bout of Campylobacter jejuni infection (eg. gastroenteritis)?
Guillain-Barre
Features of Guillain-Barre except from the weakness, absent/reduced reflexes and mild distal paraestheia?
- may be Hx of gastroenteritis
- Resp muscle weakness
- autonomic invl= urinary retention, diarrhoea
- cranial nerve involvement= diplopia, bilateral facial nerve palsy, oropharyngeal weakness common
Guillain-Barre autonomic involvement?
urinary retention
diarrhoea
Less common findings in Guillain-Barre?
papilloedema= 2 to reduced CSF resorption
Diagnosis for Guillain-Barre?
- clinical= Brighton criteria and supported by…
- LP
- Nerve conduction studies may be done
What does LP show in Guillain-Barre?
rise in protein with normal WCC (albuminocytologic dissociation) - in 66%
What do nerve conduction studies show in Guillain-Barre?
decreased motor nerve conduction velocity (due to demyelination)
prolonged distal motor latency
increased F wave latency
What NS does Guillain-Barre affect?
peripheral
What infections are associated with Guillain-Barre?
Campylobacter jejuni, cytomegalovirus (CMV) and Epstein-Barr virus (EBV)
Pathophysiology of Guillain-Barre?
Due to a process called molecular mimicry.
The B cells of the immune system create antibodies against the antigens on the triggering pathogen.
These antibodies also match proteins on the peripheral neurones. They may target proteins on the myelin sheath or the nerve axon itself.
Timeline of Guillain-Barre presentation?
typically within 4w of the triggering infection
begin in feet and ascend up
peak within 2-4w
recovery period that can last m-yrs
Mx of Guillain-Barre?
- IV immunoglobulins (IVIG) 1st line
- Plasmapheresis alternative to IVIG
- supportive
- VTE prophylaxis (PE leading cause of death)
Severe cases of Guillain-Barre may develop what?
resp failure= intubation, ventilation and admission to ICU
Prognosis of Guillain-Barre?
recovery can take months to yrs
pts can continue regaining function 5yrs after the acute illness.
Most patients eventually make either a full recovery or are left with minor symptoms.
Some are left with significant disability.
Mortality is around 5%, mainly due to respiratory or cardiovascular complications.
Peripheral neuropathy can be divided into what?
conditions which predominately cause motor or sensory loss
Peripheral neuropathy: conditions that predominately cause motor loss?
Guillain-Barre syndrome
porphyria
lead poisoning
hereditary sensorimotor neuropathies (HSMN) - Charcot-Marie-Tooth
chronic inflammatory demyelinating
polyneuropathy (CIDP)
diphtheria
Peripheral neuropathy: conditions that predominately cause sensory loss?
diabetes
uraemia
leprosy
alcoholism
vitamin B12 deficiency
amyloidosis
Alcoholic neuropathy?
secondary to both direct toxic effects and reduced absorption of B vitamins
sensory symptoms typically present prior to motor symptoms
Peripheral neuropathy caused by vit B12 def?
subacute combined degeneration of spinal cord
dorsal column usually affected first (joint position, vibration) prior to distal paraesthesia
Subacute combined degeneration of the spinal cord?
due to vit B12 def resulting in impairment of the dorsal columns, lateral corticospinal tracts and spinocerebellar tracts
What can cause subacute degeneration of the spinal cord?
B12 def
Recreational nitrous oxide inhalation may also result in vitamin B12 deficiency → subacute combined degeneration of the spinal cord.
Features of subacute degeneration of the spinal cord?
- dorsal column involvement
- lateral corticospinal tract involvement
- spinocerebellar tract involvement
Features of subacute degeneration of the spinal cord: dorsal column involvement?
distal tingling/burning/sensory loss is symmetrical and tends to affect the legs more than the arms
impaired proprioception and vibration sense
Features of subacute degeneration of the spinal cord: lateral corticospinal tract involvement?
muscle weakness, hyperreflexia, and spasticity
upper motor neuron signs typically develop in the legs first
brisk knee reflexes
absent ankle jerks
extensor plantars
Features of subacute degeneration of the spinal cord: spinocerebellar tract involvement?
sensory ataxia → gait abnormalities
positive Romberg’s sign
UMN travel where from where?
from brain to spinal cord/brainstem
Signs of UMN lesion?
- hypertonia
- hyperreflexia (brisk reflexes)
- positive Babinski’s skign
Causes of UMN lesions?
any damage to brain/brain stem/white matter of spinal cord eg. stroke, infection, tumour
LMNs travel from where to where?
from brainstem/spinal cord to skeletal muscles
Signs of LMN lesions?
- hypotonia
- decreased or absent tendon reflexes
- muscle wasting
- fasiculations
Causes of LMN lesions?
any damage to axons leaving spinal cord or anterior horn of spinal cord eg. peripheral neuropathy, spinal cord injury
Ascending (sensory) spinal tracts carry sensory info where?
from periphery, up spinal cord and to the brain
Ascending (sensory) spinal tracts?
1) Dorsal column-medial lemniscus pathway
2) Spinothalamic tract (part of anterolateral system)
3) Spinocerebellar tracts
Ascending (sensory) spinal tracts: dorsal column-medial lemniscus pathway…
function?
Fine touch (tactile discrimination).
Vibration sense.
Proprioception (sense of body position).
Ascending (sensory) spinal tracts: dorsal column-medial lemniscus pathway…
location?
posterior (dorsal) column of spinal cord
Ascending (sensory) spinal tracts: dorsal column-medial lemniscus pathway…
pathway?
Fasciculus gracilis: Carries information from the lower body (below T6).
Fasciculus cuneatus: Carries information from the upper body (above T6).
Fibers cross in the medulla and ascend to the thalamus before reaching the somatosensory cortex.
Ascending (sensory) spinal tracts: spinothalamic tract (part of anterolateral system)…
function?
pain
temp
crude touch
Ascending (sensory) spinal tracts: spinothalamic tract (part of anterolateral system)…
location?
anterolateral aspect of the spinal cord
Ascending (sensory) spinal tracts: spinothalamic tract (part of anterolateral system)…
pathway?
Sensory fibers synapse in the dorsal horn.
Second-order neurons cross within the spinal cord (anterior white commissure) and ascend to the thalamus, then the sensory cortex.
Ascending (sensory) spinal tracts: spinocerebellar tracts…
function?
proprioception for muscle coordination
Ascending (sensory) spinal tracts: spinocerebellar tracts…
location?
lateral portion of spinal cord
Ascending (sensory) spinal tracts: spinocerebellar tracts…
pathway?
Posterior spinocerebellar tract: Transmits information from the lower body to the cerebellum (ipsilateral).
Anterior spinocerebellar tract: Transmits information from the lower body, crossing in the spinal cord and cerebellum (mostly ipsilateral overall).
Descending (motor) tracts carry motor signals where?
from brain to muscles
Descending (motor) tracts?
Pyramidal tracts (direct pathways):
1) cortiospinal= lateral and anterior
2) corticobulbar
Extrapyramidal (indirect):
1) rubrospinal
2) reticulospinal
3) vestibulospinal
4) tectospinal
What are the extrapyramidal spinal tracts?
involved in involuntary, automatic and reflexive motor control
includes= rubrospinal, reticulospinal, vestibulospinal and tectospinal
What are pyramidal spinal tracts (descending)?
involved in voluntary, precise and skilled movements
includes= corticospinal (lateral and anterior) and corticobulbar tracts
Descending (motor) tracts- Pyramidal:
Corticospinal tracts:
- Function?
- Subdivisions?
Function: Control voluntary movements, especially fine motor skills.
Subdivisions:
Lateral Corticospinal Tract::
- Pathway: Originates in the motor cortex → decussates (crosses) at the medullary pyramids → descends in the contralateral spinal cord.
- Function: Controls movements of the distal limbs (e.g., hands, fingers).
Anterior Corticospinal Tract:
- Pathway: Originates in the motor cortex → descends uncrossed until the spinal cord level, where some fibers decussate.
- Function: Controls axial (trunk) and proximal muscles.
Descending (motor) tracts- Pyramidal:
Corticobulbar tract:
- Function?
- Pathway?
- Controls?
Function: Controls voluntary movements of the face, head, and neck by influencing cranial nerve nuclei.
Pathway: Originates in the motor cortex → travels through the brainstem → synapses with cranial nerve motor nuclei.
Controls muscles for speaking, swallowing, and facial expressions.
Descending (motor) tracts- Extrapyramidal:
Rubrospinal tract:
- Function?
- Pathway?
- Role?
Function: Facilitates flexor muscle tone and inhibits extensors.
Pathway: Originates in the red nucleus (midbrain) → crosses immediately → descends contralaterally to the spinal cord.
Role: Fine motor control (less significant in humans).
Descending (motor) tracts- Extrapyramidal:
Reticulospinal tract:
- Function?
- Pathway?
- Origin?
Function: Regulate posture, locomotion, and reflexes.
Pathway:
1) Medial (Pontine) Reticulospinal Tract: Facilitates extensor reflexes and muscle tone.
2) Lateral (Medullary) Reticulospinal Tract: Inhibits extensor reflexes, reducing muscle tone.
Origin: Reticular formation of the brainstem.
Descending (motor) tracts- Extrapyramidal:
Vestibulospinal tract:
- Function?
- Pathway?
- Origin?
Function: Maintain balance and posture.
Pathway:
1) Lateral Vestibulospinal Tract: Descends ipsilaterally to control extensor muscles in the limbs.
2) Medial Vestibulospinal Tract: Descends bilaterally to control head and neck muscles.
Origin: Vestibular nuclei in the brainstem.
Descending (motor) tracts- Extrapyramidal:
Tectospinal tract:
- Function?
- Pathway?
- role?
Function: Coordinates head and neck movements in response to visual and auditory stimuli.
Pathway: Originates in the superior colliculus (midbrain) → crosses immediately → descends contralaterally in the spinal cord.
Role: Reflexive turning of the head.
Lateral corticospinal tract:
- decussation
- target muscles
Medullary pyramids
Distal limbs
Anterior corticospinal tract:
- decussation
- target muscles
at spinal cord level
axial/trunk muscles
Corticobulbar:
- decussation
- target muscles
brainstem (varies)
cranial nerves
Reticulospinal:
- decussation
- target muscles
none or bilateral
proximal muscles
Rubrospinal:
- decussation
- target muscles
midbrain (red nucleus)
upper limb flexors
Vestibulospinal:
- decussation
- target muscles
none (lateral) or bilateral (medial)
axial/proximal muscles
Tectospinal:
- decussation
- target muscles
midbrain (superior colliculus)
neck muscles
Dorsal column-medial lemniscus:
- decussation
- pathway location
- target
Medulla (internal arcuate fibers)
Posterior (dorsal) column
Somatosensory cortex (via thalamus)
Dorsal column-medial lemniscus= fasciculus gracilis:
- decussation
- pathway location
- target
Medulla
Medial dorsal column
Lower body (below T6)
Dorsal column-medial lemniscus= fasciculus cuneatus:
- decussation
- pathway location
- target
Medulla
Lateral dorsal column
Upper body (above T6)
Spinothalamic tract:
- decussation
- pathway location
- target
Spinal cord (anterior white commissure)
Anterolateral column
Somatosensory cortex (via thalamus)
Spinocerebellar tracts:
- decussation
- pathway location
- target
Varies (mostly ipsilateral)
Lateral column
Cerebellum
Spinocerebellar tracts- posterior:
- decussation
- pathway location
- target
None
Lateral column (posterior)
Cerebellum (ipsilateral)
Spinocerebellar tracts- anterior:
- decussation
- pathway location
- target
Spinal cord and cerebellum
Lateral column (anterior)
Cerebellum (mostly ipsilateral)
Summarise Dorsal column-medial lemniscus tract?
Transmits highly precise sensory information (fine touch, vibration, proprioception).
Crosses in the medulla, travels via the medial lemniscus, and terminates in the somatosensory cortex.
Summarise spinothalamic tract?
Part of the anterolateral system, responsible for pain, temperature, and crude touch.
Crosses in the spinal cord at the level of entry.
Summarise the spinocerebellar tracts?
Transmit unconscious proprioceptive input for coordination.
Primarily ipsilateral, except for some fibers in the anterior spinocerebellar tract that cross twice.
Summarise pyramidal tracts (descending)?
Control voluntary, skilled movements.
Decussation occurs in the medulla (corticospinal) or brainstem (corticobulbar).
Summarise extrapyramidal tracts (descending)?
Control involuntary and reflexive movements like posture and balance.
Originate in brainstem nuclei (red nucleus, reticular formation, vestibular nuclei, and superior colliculus).
Decussation varies, with some remaining ipsilateral (e.g., lateral vestibulospinal).
What does decussation mean?
In sensory pathways, such as the dorsal column-medial lemniscus and spinothalamic tracts, decussation ensures that sensory input from one side of the body is processed by the opposite side of the brain.
In motor pathways, such as the corticospinal tract, decussation ensures that motor commands from one hemisphere of the brain control muscles on the opposite side of the body.
Examples of decussation?
Medullary Pyramids (Motor): The corticospinal tract crosses at the pyramids in the medulla, which is why a stroke in one hemisphere affects voluntary movements on the opposite side.
Spinal Cord (Sensory): The spinothalamic tract crosses at the level of entry into the spinal cord, carrying pain and temperature signals to the opposite side of the brain.
Brainstem (Sensory): The fibers of the dorsal column-medial lemniscus cross at the medulla via the internal arcuate fibers.
Give an example of the clinical significance of decussation?
The location of decussation determines whether a lesion in the CNS causes ipsilateral (same side) or contralateral (opposite side) effects.
For example:
A lesion above the corticospinal tract decussation in the medulla leads to contralateral weakness.
A lesion below the decussation causes ipsilateral weakness.
Third nerve palsy features?
eye is deviated ‘down and out’
ptosis
pupil may be dilated (sometimes called a ‘surgical’ third nerve palsy)
Causes of third nerve palsy?
diabetes mellitus
vasculitis e.g. temporal arteritis, SLE
false localizing sign* due to uncal herniation through tentorium if raised ICP
posterior communicating artery aneurysm= pupil dilated, often associated pain
cavernous sinus thrombosis
Weber’s syndrome: ipsilateral third nerve palsy with contralateral hemiplegia -caused by midbrain strokes
other possible causes: amyloid, multiple sclerosis
False localising sign?
Third nerve palsy (usually 6th nerve but can be used for variety of neuro presentation) due to uncal herniation through tentorium if raised ICP
Aphasia vs dysarthria?
Aphasia occurs due to brain damage that affects the ability to express and understand speech.
Dysarthria, on the other hand, is a condition that affects the muscles necessary for speech. It does not affect the ability to understand language.
4 types of aphasia?
- Wernicke’s (receptive)
- Broca’s (expressive)
- Conduction aphasia
- Global aphasia
Wernicke’s (receptive) aphasia is due to what?
Due to a lesion of the superior temporal gyrus. It is typically supplied by the inferior division of the left MCA
Wernicke’s (receptive aphasia)?
Comprehension is impaired.
This area ‘forms’ the speech before ‘sending it’ to Broca’s area. Lesions result in sentences that make no sense, word substitution and neologisms but speech remains fluent - ‘word salad’
Broca’s (expressive) aphasia is due to what?
Due to a lesion of the inferior frontal gyrus. It is typically supplied by the superior division of the left MCA
Broca’s (expressive) aphasia?
Comprehension is normal.
Speech is non-fluent, laboured, and halting. Repetition is impaired
Conduction aphasia?
Speech is fluent but repetition is poor. Aware of the errors they are making
Comprehension is normal
Conduction aphasia is due to what?
Classically due to a stroke affecting the arcuate fasiculus - the connection between Wernicke’s and Broca’s area
Connection between Broca’s and Wernicke’s area?
arcuate fasiculus
Global aphasia?
Large lesion affecting all 3 of the above areas (wernicke, broca and conduction) resulting in severe expressive and receptive aphasia
May still be able to communicate using gestures
What lobes are Broca’s and Wernicke’s in?
Wernicke’s area is located in the temporal lobe, while Broca’s area is located in the frontal lobe
Speech non fluent:
- comprehension relatively intact?
- comprehension impaired?
intact= Broca’s aphasia
impaired= global aphasia
Speech fluent:
- comprehension relatively intact?
- comprehension impaired?
intact= conduction aphasia
impaired= Wernicke’s aphasia
Arnold-Chiari malformation?
downward displacement, or herniation, of the cerebellar tonsils through the foramen magnum
malformations may be congenital or acquired through trauma
Features of Arnold-Chiari malformation?
non-communicating hydrocephalus may develop as a result of obstruction of cerebrospinal fluid (CSF) outflow
headache
syringomyelia
What cause peripheral (‘finger-nose ataxia’)?
cerebellar hemisphere lesions
What lesion causes gait ataxia?
cerebellar vermis
Ataxia telangiectasia?
autosomal recessive disorder caused by defect in ATM gene which encodes for DNA repair enzymes
one of the inherited combined immunodeficiency disorders
When does Ataxia telangiectasia typically present?
early childhood with abnormal movements
Ataxia telangiectasia features?
cerebellar ataxia
telangiectasia (spider angiomas)
IgA deficiency resulting in recurrent chest infections
10% risk of developing malignancy, lymphoma or leukaemia, but also non-lymphoid tumours
Ataxia telangiectasia vs Fredreich’s ataxia?
F= trinucleotide repeat disorder (but no anticipation); kyphoscoliosis; optic atrophy; HOCM; DM; onset at 10-15yrs
A= telangiectasia (spider angiomas); IgA def so recurrent infections; increased risk of lymphoma and leukaemia; onset at 1-5yrs
Both= autosomal recessive; cerebellar ataxia; onset in childhood
Autonomic dysreflexia?
occurs in pts who have had a spinal cord injury at, or above T6 spinal level
Briefly, afferent signals, most commonly triggered by faecal impaction or urinary retention (but many other triggers have been reported) cause a sympathetic spinal reflex via thoracolumbar outflow. The usual, centrally mediated, parasympathetic response however is prevented by the cord lesion.
Features of autonomic dysreflexia?
the result is an unbalanced physiological response, characterised by extreme HTN
flushing and sweating above the level of the cord lesion
agitation
in untreated cases, severe consequences of extreme hypertension have been reported, e.g. haemorrhagic stroke.
Mx of autonomic dysreflexia?
removal/control of the stimulus and treatment of any life-threatening hypertension and/or bradycardia.
Brachial plexus: origin?
anterior rami of C5 to T1
Brachial plexus: sections of the plexus?
- roots, trunks, divisions, cords, branches
- Mneumonic= Real Teenagers Drink Cold Beer
Brachial plexus: roots?
- located in the posterior triangle
- pass between scalenus anterior and medius
Brachial plexus: trunks?
- located posterior to middle third of clavicle
- upper and middle trunks related superiorly to subclavian artery
- lower trunk passes over 1st rib posterior to subclavian artery
Brachial plexus: divisions?
apex of axilla
Brachial plexus: cords?
related to axillary artery
Parietal lobe lesions?
- sensory inattention
- apraxias
- astereognosis (tactile agnosia)
- inferior homonymous quadrantanopia
- Gerstmann’s syndrome (lesion of dominant parietal): alexia, acalculia, finger agnosia and right-left disorientation
Occipital lobe lesions?
- homonymous hemianopia (with macula sparing)
- cortical blindness
- visual agnosia
Temporal lobe lesion?
- Wernicke’s aphasia: this area ‘forms’ the speech before ‘sending it’ to Brocas area. Lesions result in word substituion, neologisms but speech remains fluent
- superior homonymous quadrantanopia
- auditory agnosia
- prosopagnosia (difficulty recognising faces)
Frontal lobe lesions?
expressive (Broca’s) aphasia: located on the posterior aspect of the frontal lobe, in the inferior frontal gyrus. Speech is non-fluent, laboured, and halting
disinhibition
perseveration
anosmia
inability to generate a list
Cerebellum lesions?
midline lesions: gait and truncal ataxia
hemisphere lesions: intention tremor, past pointing, dysdiadokinesis, nystagmus
Brain lesion causing= Wernicke and Korsakoff syndrome?
Medial thalamus and mammillary bodies of the hypothalamus
Brain lesion causing= hemiballism?
subthalamic nucleus of the basal ganglia
Brain lesion causing= Huntington chorea
striatum (caudate nucleus) of basal ganglia
Brain lesion causing= parkinson’s disease
substantia nigra of basal ganglia
Brain lesion causing= Kluver-Bucy syndrome (hypersexuality, hyperorality, hyperphagia, visual agnosia)
amygdala
Hemiballism
rare movement disorder that causes involuntary, violent, and wide-amplitude limb movements on one side of the body
Tumours that most commonly spread to the brain?
lung (most common)
breast
bowel
skin (namely melanoma)
kidney
Glioblastoma multiforme?
- Glioblastoma is the most common primary tumour in adults and is associated with a poor prognosis (~ 1yr).
- On imaging they are solid tumours with central necrosis and a rim that enhances with contrast. Disruption of the blood-brain barrier and therefore are associated with vasogenic oedema.
- Treatment is surgical with postoperative chemotherapy and/or radiotherapy. Dexamethasone is used to treat the oedema.
Glioblastoma multiforme histology?
- Histology: Pleomorphic tumour cells border necrotic areas
Meningioma?
The second most common primary brain tumour in adults
- Meningiomas are typically benign, extrinsic tumours of the central nervous system. Cause symptoms by compression rather than invasion.
- They typically are located at the falx cerebri, superior sagittal sinus, convexity or skull base.
- Investigation is with CT (will show contrast enhancement) and MRI, and treatment will involve either observation, radiotherapy or surgical resection.
Meningioma arise from what?
arise from the arachnoid cap cells of the meninges and are typically located next to the dura and cause symptoms by compression rather than invasion.
Meningioma histology?
Spindle cells in concentric whorls and calcified psammoma bodies
Vestibular schwannoma?
- A vestibular schwannoma (previously termed acoustic neuroma) is a benign tumour arising from the eighth cranial nerve (vestibulocochlear nerve). Often seen in the cerebellopontine angle. It presents with hearing loss, facial nerve palsy (due to compression of the nearby facial nerve) and tinnitus.
- Neurofibromatosis type 2 is associated with bilateral vestibular schwannomas.
- Treatment may involve observation, radiotherapy or surgery.
Vestibular schwannoma histology?
Antoni A or B patterns are seen. Verocay bodies (acellular areas surrounded by nuclear palisades)
Pilocytic astrocytoma?
- The most common primary brain tumour in children
Medulloblastoma?
A medulloblastoma is an aggressive paediatric brain tumour that arises within the infratentorial compartment. It spreads through the CSF system. Treatment is surgical resection and chemotherapy.
Medulloblastoma histology?
- Histology: Small, blue cells. Rosette pattern of cells with many mitotic figures
Pilocytic astrocytoma histology?
Rosenthal fibres (corkscrew eosinophilic bundle)
Ependymoma?
- Commonly seen in the 4th ventricle
- May cause hydrocephalus
Ependymoma histology?
perivascular pseudorosettes
Oligodendroma?
benign, slow growing tumour common in frontal lobes
Oligodendroma histology?
Calcifications with ‘fried-egg’ appearance
Haemangioblastoma?
- Vascular tumour of the cerebellum
- Associated with von Hippel-Lindau syndrome
Haemangioblastoma histology?
foam cells and high vascularity
Pituitary adenoma?
- Pituitary adenomas are benign tumours of the pituitary gland. They are either secretory (producing a hormone in excess) or non-secretory. They may be divided into microadenomas (smaller than 1cm) or macroadenoma (larger than 1cm).
How will pts with pituitary adenoma present?
will present with the consequences of hormone excess (e.g. Cushing’s due to ACTH, or acromegaly due to GH) or depletion. Compression of the optic chiasm will cause a bitemporal hemianopia due to the crossing nasal fibers.
Pituitary adenoma Ix and Tx?
Investigation requires a pituitary blood profile and MRI. Treatment can either be hormonal or surgical (e.g. transphenoidal resection).
Craniopharyngioma?
- Most common paediatric supratentorial tumour
- A craniopharyngioma is a solid/cystic tumour of the sellar region that is derived from the remnants of Rathke’s pouch. It is common in children, but can present in adults also. It may present with hormonal disturbance, symptoms of hydrocephalus or bitemporal hemianopia.
- Investigation requires pituitary blood profile and MRI. Treatment is typically surgical with or without postoperative radiotherapy.
Craniopharyngioma histology?
Derived from remnants of Rathke pouch
Brown-Sequard syndrome?
caused by lateral hemisection of the spinal cord
Features of Brown-Sequard syndrome?
ipsilateral weakness below lesion
ipsilateral loss of proprioception and vibration sensation
contralateral loss of pain and temperature sensation
Cataplexy?
Cataplexy describes the sudden and transient loss of muscular tone caused by strong emotion (e.g. laughter, being frightened). Around two-thirds of patients with narcolepsy have cataplexy.
Features range from buckling knees to collapse.
Cavernous sinus?
The cavernous sinuses are paired and are situated on the body of the sphenoid bone. It runs from the superior orbital fissure to the petrous temporal bone.
Cavernous sinus relations?
medial= pituitary fossa, sphenoid sinus
lateral= temporal lobe
Cavernous sinus contents?
Lateral wall components= (from top to bottom:)
Oculomotor nerve
Trochlear nerve
Ophthalmic nerve
Maxillary nerve
Contents of the sinus=
(from medial to lateral:)
Internal carotid artery (and sympathetic plexus)
Abducens nerve
Cavernous sinus blood supply?
Blood supply
Ophthalmic vein, superficial cortical veins, basilar plexus of veins posteriorly.
Drains into the internal jugular vein via: the superior and inferior petrosal sinuses
Unilateral cerebellar lesions cause what signs?
ipsilateral signs
Mnemonic to remember cerebellar disease?
D - Dysdiadochokinesia, Dysmetria (past-pointing), patients may appear ‘Drunk’
A - Ataxia (limb, truncal)
N - Nystamus (horizontal = ipsilateral hemisphere)
I - Intention tremour
S - Slurred staccato speech, Scanning dysarthria
H - Hypotonia
Causes of cerebellar syndrome?
Friedreich’s ataxia, ataxic telangiectasia
neoplastic: cerebellar haemangioma
stroke
alcohol
multiple sclerosis
hypothyroidism
drugs: phenytoin, lead poisoning
paraneoplastic e.g. secondary to lung cancer
Cerebral perfusion pressure (CPP)?
net pressure gradient causing blood flow to the brain. The CPP is tightly autoregulated to maximise cerebral perfusion.
What happens if there is changes in cerebral perfusion pressure (CPP)?
sharp rise in CPP may result in a rising ICP, a fall in CPP may result in cerebral ischaemia.
How to calculate cerebral perfusion pressure?
CPP=
Mean arterial pressure - Intra cranial pressure
Following trauma, the cerebral perfusion pressure has to be…
carefully controlled and may require invasive monitoring of the ICP and MAP
CSF fills what space?
space between arachnoid mater and pia mater (covering surface of the brain)
Total volume of CSF in brain?
approx 150ml
Where is CSF produced and reabsorbed?
Approx 500 ml is produced by the ependymal cells in the choroid plexus (70%), or blood vessels (30%). It is reabsorbed via the arachnoid granulations which project into the venous sinuses.
there is approx 150ml in brain
CSF circulation?
- Lateral ventricles (via foramen of Munro)
- 3rd ventricle
- Cerebral aqueduct (aqueduct of Sylvius)
- 4th ventricle (via foramina of Magendie and Luschka)
- Subarachnoid space
- Reabsorbed into the venous system via arachnoid granulations into superior sagittal sinus
CSF composition?
Glucose: 50-80mg/dl
Protein: 15-40 mg/dl
Red blood cells: Nil
White blood cells: 0-3 cells/ mm3
Charcot-Marie-Tooth disease?
most common hereditary peripheral neuropathy. It results in a predominantly motor loss. There is no cure, and management is focused on physical and occupational therapy.
Charcot-Marie-Tooth disease features?
There may be a history of frequently sprained ankles
Foot drop
High-arched feet (pes cavus)
Hammer toes
Distal muscle weakness
Distal muscle atrophy (upside down champagne bottle legs)
Hyporeflexia
Stork leg deformity
CNS tumours?
- 60% = Glioma and metastatic disease
20% = Meningioma
10% = Pituitary lesions
Most common CNS tumours in children?
astrocytomas
Symptoms of CNS tumours?
Tumours arising in right temporal and frontal lobe may reach considerable size before becoming symptomatic. Whereas tumours in the speech and visual areas will typically produce early symptoms.
Diagnosis of CNS tumours?
MRI Scanning provides the best resolution.
Tx for CNS tumours?
Usually surgery, even if tumour cannot be completely resected conditions such as rising ICP can be addressed with tumour debulking and survival and quality of life prolonged.
Curative surgery can usually be undertaken with lesions such as meningiomas. Gliomas have a marked propensity to invade normal brain and resection of these lesions is nearly always incomplete.
Cranial nerve I?
olfactory
CN II?
optic
CN III?
oculomotor
CN IV?
trochlear
CN V?
trigeminal
CN VI?
abducens
CN VII?
facial
CN VIII?
vestibulocochlear
CN IX?
glossopharyngeal
CN X?
vagus
CN XI?
accessory
CN XII?
hypoglossal
CN I (olfactory)= functions?
smell
CN I (olfactory)= pathway/foramen?
cribriform plate
CN II (optic)= functions?
sight
CN II (optic)= pathway/foramen?
optic canal
CN III (oculomotor)= functions?
Eye movement (MR, IO, SR, IR)
Pupil constriction
Accomodation
Eyelid opening
CN III (oculomotor)= clinical?
Palsy results in:
- ptosis
- ‘down and out’ eye
- dilated, fixed pupil
CN III (oculomotor)= pathway/foramen?
superior orbital fissure (SOF)
CN IV (trochlear)= functions?
eye movement (SO)
CN IV (trochlear)= clinical?
palsy results in defective downward gaze -> vertical diplopia
CN IV (trochlear)= pathway/foramen?
SOF
CN V (trigeminal)= functions?
facial sensation
mastication
CN V (trigeminal)= clinical?
Lesions may cause:
- trigeminal neuralgia
- loss of corneal reflex (afferent)
- loss of facial sensation
- paralysis of mastication muscles
- deviation of jaw to weak side
CN V (trigeminal)= pathway/foramen?
V1: SOF, V2: Foramen rotundum,
V3: Foramen ovale
CN VI (abducens)= functions?
eye movement (LR)
CN VI (abducens)= clinical?
palsy results in defective abduction -> horizontal diplopia
CN VI (abducens)= pathway/foramen?
SOF
CN VII (facial)= functions?
Facial movement
Taste (anterior 2/3rds of tongue)
Lacrimation
Salivation
CN VII (facial)= clinical?
Lesions may result in:
- flaccid paralysis of upper + lower face
- loss of corneal reflex (efferent)
- loss of taste
- hyperacusis
CN VII (facial)= pathway/foramen?
internal auditory meatus
CN VIII (vestibulocochlear)= functions?
hearing, balance
CN VIII (vestibulocochlear)= clinical?
Hearing loss
Vertigo, nystagmus
Acoustic neuromas are Schwann cell tumours of the cochlear nerve
CN VIII (vestibulocochlear)= pathway/foramen?
internal auditory meatus
CN IX (glossopharyngeal)= functions?
Taste (posterior 1/3rd of tongue)
Salivation
Swallowing
Mediates input from carotid body & sinus
CN IX (glossopharyngeal)= clinical?
Lesions may result in;
- hypersensitive carotid sinus reflex
- loss of gag reflex (afferent)
CN IX (glossopharyngeal)= pathway/foramen?
jugular foramen
CN X (vagus)= features?
phonation
swallowing
innervates viscera
CN X (vagus)= clinical?
lesions may result in:
- uvula deviates away from site of lesion
- loss of gag reflex (efferent)
CN X (vagus)= pathway/foramen?
jugular foramen
CN XI (accessory)= functions?
head and shoulder movement
CN XI (accessory)= clinical?
lesions may result in:
- weakness turning head to contralateral side
CN XI (accessory)= pathway/foramen?
jugular foramen
CN XII (hypoglossal)= functions?
tongue movement
CN XII (hypoglossal)= clinical?
tongue deviates towards side of lesion
CN XII (hypoglossal)= pathway/foramen?
hypoglossal canal
Mneumonic to remember which CN are motor or sensory or both?
Some Say Marry Money But My Brother Says Big Brains Matter Most
S = Sensory, M = Motor, B = Both
CN reflexes= corneal?
Afferent limb= opthalmic nerve (V1)
Efferent limb= facial nerve (VII)
CN reflexes= jaw jerk?
Afferent limb= mandibular nerve (V3)
Efferent limb= mandibular nerve (V3)
CN reflexes= gag?
Afferent limb= glossopharyngeal nerve (IX)
Efferent limb= vagal nerve (X)
CN reflexes= carotid sinus?
Afferent limb= glossopharyngeal nerve (IX)
Efferent limb= vagal nerve (X)
CN reflexes= pupillary light?
Afferent limb= optic nerve (II)
Efferent limb= oculomotor nerve (III)
CN reflexes= lacrimation?
Afferent limb= opthalmic nerve (V1)
Efferent limb= facial nerve (VII)
Creutzfeldt-Jakob disease (CJD)?
rapidly progressive neurological condition caused by prion proteins. These proteins induce the formation of amyloid folds resulting in tightly packed beta-pleated sheets resistant to proteases.
no cure, usually die within 1yr
Features of Creutzfeldt-Jakob disease (CJD)?
dementia (rapid onset)
myoclonus
Ix for Creutzfeldt-Jakob disease (CJD)?
CSF usually normal
EEG= biphasic, high amplitude sharp waves (only in sporadic)
MRI= hyperintense signals in basal ganglia and thalmus
Creutzfeldt-Jakob disease (CJD)= sporadic?
accounts for 85% of cases
10-15% of cases are familial
mean age of onset is 65 years
Creutzfeldt-Jakob disease (CJD)= new variant CJD?
younger patients (average age of onset = 25 years)
psychological symptoms such as anxiety, withdrawal and dysphonia are the most common presenting feature
s
the ‘prion protein’ is encoded on chromosome 20 - it’s role is not yet understood
methionine homozygosity at codon 129 of the prion protein is a risk factor for developing CJD - all patients who have so far died have had this
median survival = 13 months
Prion diseases?
CJD
kuru
fatal familial insomnia
Gerstmann Straussler-Scheinker disease
Nerve root= C2
Landmark?
posterior half of skull (cap)
Nerve root= C3
Landmark?
high turtleneck shirt
Nerve root= C4
Landmark?
low-collar shirt
Nerve root= C5
Landmark?
ventral axial line of upper limb
Nerve root= C6
Landmark?
thumb and index finger
(make a 6 with ur left hand by touching tip of thumb and index finger)
Nerve root= C7
Landmark?
middle finger and palm of hand
Nerve root= C8
Landmark?
ring and little finger
Nerve root= T4
Landmark?
nipples
(T4 at the Teat Pore)
Nerve root= T5
Landmark?
inframammary fold
Nerve root= T6
Landmark?
Xiphoid process
Nerve root= T10
Landmark?
umbilicus
(BellybuT-TEN)
Nerve root= L1
Landmark?
inguinal ligament
(L for ligament, 1 for 1nguinal)
Nerve root= L4
Landmark?
knee caps
(don on aLL fours- L4)
Nerve root= L5
Landmark?
big toe, dorsum of foot (except lateral aspect)
(L5= Largest of the 5 toes)
Nerve root= S1
Landmark?
lateral foot, small toe
(S1= small toe)
Nerve root= S2, S3
Landmark?
genitalia
Dystrophinopathies inheritance?
X-linked recessive
Dystrophinopathies due to?
due to mutation in the gene encoding dystrophin, dystrophin gene on Xp21
dystrophin is part of a large membrane associated protein in muscle which connects the muscle membrane to actin, part of the muscle cytoskeleton
Dystrophinopathies: Duchenne vs Becker mutations?
in Duchenne muscular dystrophy there is a frameshift mutation resulting in one or both of the binding sites are lost leading to a severe form
in Becker muscular dystrophy there is a non-frameshift insertion in the dystrophin gene resulting in both binding sites being preserved leading to a milder form
Summary of Duchenne muscular dystrophy?
progressive proximal muscle weakness from 5 years
calf pseudohypertrophy
Gower’s sign: child uses arms to stand up from a squatted position
30% of patients have intellectual impairment
Summary of Becker muscular dystrophy?
develops after the age of 10 years
intellectual impairment much less common
What does EMG stand for?
electromyography
What is EMG used for?
used to evaluate the physiological properties of muscles at rest and whilst contracting
EMG findings in neuropathy?
↑ action potential duration
↑ action potential amplitude
EMG findings in myopathy?
↓ action potential duration
↓ action potential amplitude
EMG findings in MG?
Diminished response to repetitive stimulation
EMG findings in Lambert-Eaton syndrome?
Incremental response to repetitive stimulation
EMG findings in myotonic syndromes?
extended series of repetitive discharges lasting up to 30 seconds
What muscle does the trochlear nerve (CN IV) supply?
superior oblique muscle= rotates eye down and out
enables eye to look down when adducted (towards nose)- important for going downstairs or reading
Features of fourth cranial nerve palsy?
vertical diplopia= when reading book or going downstairs
torsional diplopia (subjective tilting of objects
pt may develop head tilt, may not be aware of
when looking straight ahead, affected eye deviated up and rotated out
Pt looking straight ahead, affected eye deviates up and rotated out?
trochlear (CN IV) palsy (superior oblique muscle)
Diplopia when reading a book or going downstairs?
CN IV palsy
Friedreich’s ataxia?
most common early-onset hereditary ataxias
gait ataxia and kyphoscoliosis
Friedreich’s ataxia genetics?
autosomal recessive trinucleotide repeat disorder characterised by GAA repeat in X25 gene on chromosome 9 (frataxin)
Why is Friedreich’s ataxia an unusual trinucleotide repeat disorder?
doesn’t demonstrate anticipation
Friedreich’s ataxia main features?
gait ataxia and kyphoscoliosis
Friedreich’s ataxia typical age of onset?
10-15yrs
Friedreich’s ataxia neuro features?
absent ankle jerks/extensor plantars
cerebellar ataxia
optic atrophy
spinocerebellar tract degeneration
Friedreich’s ataxia other features?
hypertrophic obstructive cardiomyopathy (90%, most common cause of death)
diabetes mellitus (10-20%)
high-arched palate
Hereditary sensorimotor neuropathy (HSMN)?
term which encompasses Charcot-Marie-Tooth disease (aka peroneal muscle atrophy)
over 7 types have been characterised but only 2 are common
Hereditary sensorimotor neuropathy (HSMN)= 2 types?
HSMN type I: primarily due to demyelinating pathology
HSMN type II: primarily due to axonal pathology
Hereditary sensorimotor neuropathy (HSMN) type 1?
autosomal dominant
due to defect in PMP-22 gene (which codes for myelin)
features often start at puberty
motor symptoms predominate
distal muscle wasting, pes cavus, clawed toes
foot drop, leg weakness often first features
Idiopathic intracranial hypertension also known as what?
pseudotumour cerebri and formerly benign intracranial hypertension
Idiopathic intracranial hypertension?
condition classically seen in young overweight females
RFs of Idiopathic intracranial hypertension?
obesity
female
pregnany
drugs
What drugs are RFs for Idiopathic intracranial hypertension?
combined oral contraceptive pill
steroids
tetracyclines
retinoids (isotretinoin, tretinoin) / vitamin A
lithium
Idiopathic intracranial hypertension features?
headache
blurred vision
papilloedema
enlarged blind spot
may be 6th nerve palsy
Mx of Idiopathic intracranial hypertension?
- weight loss
- carbonic anhydrase inhibitors eg. acetazolamide
- repeated LP temporary but not for long term Mx
- surgery may be needed to prevent damage to optic nerve
Idiopathic intracranial hypertension: what drugs may be considered by specialist alongside diet and exercise to aid weight loss for Mx?
semaglitide and topiramate
topiramate also inhibits carbonic anhydrase
Idiopathic intracranial hypertension: when may surgery be needed?
optic nerve sheath decompression and fenestration may be needed to prevent damage to the optic nerve. A lumboperitoneal or ventriculoperitoneal shunt may also be performed to reduce intracranial pressure
headache, blurred vision, papilloedema, enlarged blind spot, 6th nerve palsy, overweight female?
Idiopathic intracranial HTN
Lambert-Eaton myasthenic syndrome?
repeated muscle contractions lead to increased muscle strength (in contrast to MG)
Lambert-Eaton syndrome is often seen in association with what?
small cell lung ca and lesser extent breast & ovarian ca
may also occur independently as an autoimmune disorder
Cause of Lambert-Eaton syndrome?
caused by an antibody directed against presynaptic voltage-gated calcium channel in the peripheral NS
can be autoimmune or in association with small cell lung ca
Features of Lambert-Eaton syndrome?
repeated muscle contractions lead to increased muscle strengt
(this is seen in only 50% of patients and following prolonged muscle use muscle strength will eventually decrease)
limb-girdle weakness (affects lower limbs first)
hyporeflexia
autonomic symptoms: dry mouth, impotence, difficulty micturating
ophthalmoplegia and ptosis not commonly a feature (unlike in myasthenia gravis)
Autonomic symptoms of Lambert-Eaton syndrome?
dry mouth, impotence, difficulty micturating
Ix in Lamber-Eaton syndrome?
EMG= incremental response to repetitive electrical stimulation
Mx of Lambert-Eaton syndrome?
- Tx underlying ca
- immunosupression= pred +/or azathioprine
- IVIG and plasma exchange may be beneficial
What is currently being trialled for the Mx of Lambert-Eaton syndrome?
3,4-diaminopyridine (Amifampridine)
works by blocking potassium channel efflux in the nerve terminal so that the action potential duration is increased. Calcium channels can then be open for a longer time and allow greater acetylcholine release to the stimulate muscle at the end plate
Intranuclear opthalmoplegia?
a cause of horizontal disconjugate eye movement
due to a lesion in the medial longitudinal fasciculus (MLF):
- controls horizontal eye movements by interconnecting the IIIrd, IVth and VIth cranial nuclei
- located in the paramedian area of the midbrain and pons
Features of internuclear opthalmoplegia?
impaired adduction of the eye on the same side as the lesion
horizontal nystagmus of the abducting eye on the contralateral side
Causes of internuclear ophthalmoplegia?
multiple sclerosis
vascular disease
Intracranial venous thrombosis?
can cause cerebral infarction, much lesson common than arterial causes
50% of patients have isolated sagittal sinus thromboses - the remainder have coexistent lateral sinus thromboses and cavernous sinus thromboses
Intracranial venous thrombosis common features?
headache (may be sudden onset)
N&V
reduced consciousness
Ix for Intracranial venous thrombosis?
GOLD= MRI venography
non-contrast CT normal in 70%
D-dimer levels may be elevated
Mx for Intracranial venous thrombosis?
anticoag:
- LMWH acute
- then warfarin long term
Intracranial venous thrombosis: specific syndromes?
- sagittal sinus thrombosis
- cavernous sinus thrombosis
- lateral sinus thrombosis
Intracranial venous thrombosis: sagittal sinus thrombosis?
may present with seizures and hemiplegia
parasagittal biparietal or bifrontal haemorrhagic infarctions are sometimes seen
‘empty delta sign’ seen on venography
Intracranial venous thrombosis: cavernous sinus thrombosis?
other causes of cavernous sinus syndrome: local infection (e.g. sinusitis), neoplasia, trauma
periorbital erythema and oedema
ophthalmoplegia: 6th nerve damage typically occurs before 3rd & 4th
trigeminal nerve involvement may lead to hyperaesthesia of upper face and eye pain
central retinal vein thrombosis
Intracranial venous thrombosis: lateral sinus thrombosis?
6th and 7th cranial nerve palsies
Median nerve pathway?
formed by the union of a lateral and medial root respectively from the lateral (C5,6,7) and medial (C8 and T1) cords of the brachial plexus; the medial root passes anterior to the third part of the axillary artery. The nerve descends lateral to the brachial artery, crosses to its medial side (usually passing anterior to the artery). It passes deep to the bicipital aponeurosis and the median cubital vein at the elbow.
It passes between the two heads of the pronator teres muscle, and runs on the deep surface of flexor digitorum superficialis (within its fascial sheath).
Near the wrist it becomes superficial between the tendons of flexor digitorum superficialis and flexor carpi radialis, deep to palmaris longus tendon. It passes deep to the flexor retinaculum to enter the palm, but lies anterior to the long flexor tendons within the carpal tunnel.
Median nerve: branch in upper arm?
No branches, although the nerve commonly communicates with the musculocutaneous nerve
Median nerve: branches in forearm?
Pronator teres
Flexor carpi radialis
Palmaris longus
Flexor digitorum superficialis
Flexor pollicis longus
Flexor digitorum profundus (only the radial half)
Median nerve: branches in distal forearm?
palmar cutaneous branch
Median nerve: branches in hand (motor)?
Motor supply (LOAF)
Lateral 2 lumbricals
Opponens pollicis
Abductor pollicis brevis
Flexor pollicis brevis
Median nerve: branches in hand (sensory)?
Over thumb and lateral 2 ½ fingers
On the palmar aspect this projects proximally, on the dorsal aspect only the distal regions are innervated with the radial nerve providing the more proximal cutaneous innervation.
Median nerve damage at wrist?
e.g. carpal tunnel syndrome
paralysis and wasting of thenar eminence muscles and opponens pollicis (ape hand deformity)
sensory loss to palmar aspect of lateral (radial) 2 ½ fingers
Median nerve damage at elbow?
same as wrist +
unable to pronate forearm
weak wrist flexion
ulnar deviation of wrist
Median nerve: damage of anterior interosseous nerve (branch of median nerve)?
leaves just below the elbow
results in loss of pronation of forearm and weakness of long flexors of thumb and index finger
Types of multiple system atrophy?
2 predominant:
1) MSA-P - Predominant Parkinsonian features
2) MSA-C - Predominant Cerebellar features
Shy-Drager syndrome is a type of multiple system atrophy.
Features of multiple system atrophy?
parkinsonism
autonomic disturbance
erectile dysfunction: often an early feature
postural hypotension
atonic bladder
cerebellar signs
Neurofibromatosis?
There are two types of neurofibromatosis, NF1 and NF2. Both are inherited in an autosomal dominant fashion
Neurofibromatosis type 1 (NF1)?
also known as von Recklinghausen’s syndrome. It is caused by a gene mutation on chromosome 17 which encodes neurofibromin and affects around 1 in 4,000
NF2?
caused by gene mutation on chromosome 22 and affects around 1 in 100,000
Features of NF1?
Cafe-au-lait spots (>= 6, 15 mm in diameter)
Axillary/groin freckles
Peripheral neurofibromas
Iris hamatomas (Lisch nodules) in > 90%
Scoliosis
Pheochromocytomas
Features of NF2?
Bilateral vestibular schwannomas
Multiple intracranial schwannomas, mengiomas and ependymomas
Neuroleptic malignant syndrome?
rare but dangerous condition seen in pts taking antipsychotic meds
mortality of up to 10%
can occur also with atypical antipsychotics
What drugs can Neuroleptic malignant syndrome occur with?
- antipsychotics (incl atypicals)
- dopaminergic drugs eg. levodopa for Parkinson’s= usually when drug is suddenly stopped or dose reduced
Pathophysiology of Neuroleptic malignant syndrome?
unknown but thought that the dopamine blockade induced by antipsychotics trigger massive glutamate release and subsequent neurotoxicity and muscle damage
When does Neuroleptic malignant syndrome occur?
within hrs to days of starting antipsychotics (antipsychotics also known as neuroleptics hence the name)
Typical features in Neuroleptic malignant syndrome?
- pyrexia
- muscle rigidity
- autonomic lability= HTN, tachycardia, tachypnoea
- agitated delirium with confusion
What may also be seen in Neuroleptic malignant syndrome on top of the S&S?
raised creatine kinase
AKI (secondary to rhabdomyolysis) in severe cases
leukocytosis
Mx of Neuroleptic malignant syndrome?
- stop antipsychotics
- often transferred to ICU
- IV fluids to prevent renal failure
- dantrolene in some cases
- may also use bromocriptine (dopamine agonist)
What drugs may be used in Neuroleptic malignant syndrome and how do they work?
dantrolene= decreasing excitation-contraction coupling in skeletal muscle by binding to the ryanodine receptor, and decreasing the release of calcium from the sarcoplasmic reticulum
bromocriptine= dopamine agonist
Serotonin syndrome vs neuroleptic malignant syndrome?
SS= caused by SSRIs, MAOIs, ecstacy/novel psychoactive stimulants; faster onset (hrs); increased reflexes, clonus, dilated pupils; Mx of severe cases= cyproheptadine, chlorpromazine
NMS= caused by antipsychotics (and stopping dopaminergic drugs eg. levodopa in Parkinsons); slower onset (hrs-ds); decreased reflexes, lead-pipe rigidity, normal pupils; Mx of severe= dantrolene
Both= ‘drug’ reaction often in younger pts; tachycardia, HTN; pyrexia, diaphoresis; rigidity; IV fluids + benzodiazepines
Normal pressure hydocephalus?
reversible cause of dementia seen in elderly patients.
It is thought to be secondary to reduced CSF absorption at the arachnoid villi. These changes may be secondary to head injury, subarachnoid haemorrhage or meningitis.
Normal pressure hydocephalus triad?
- urinary incontinence
- dementia and bradyphrenia
- gait abnormality (may be similar to Parkinson’s)
may develop over months
Normal pressure hydocephalus Ix?
CT head= hydrocephalus with ventriculomegaly, in absence of or out of proportion to sulcal enlargement
Mx of Normal pressure hydocephalus?
- ventriculoperitoneal shunting
- around 10% with shunts have signif Cx= seizures, infection, intracerebral haemorrhage
Paroxysmal hemicrania (PH)?
defined by attacks of severe, unilateral headache, usually in the orbital, supraorbital or temporal region. These attacks are often associated with autonomic features, usually last less than 30 minutes and can occur multiple times a day.
Paroxysmal hemicrania (PH) is within a group of disorders called what?
trigeminal autonomic cephalgias which also contains cluster headache, a condition which shares many features with PH.
Mx of paroxysmal hemicrania?
completely responsive to indomethacin
- urinary incontinence
- dementia
- gait abnormality (may be similar to Parkinson’s)
Normal pressure hydrocephalus
Pituitary apoplexy?
Sudden enlargement of a pituitary tumour (usually non-functioning macroadenoma) secondary to haemorrhage or infarction.
Pituitary apoplexy precipitating factors?
HTN
pregnancy
trauma
anticoag
Features of Pituitary apoplexy?
- sudden onset headache (like in SAH)
- vomiting
- neck stiffness
- visual field defects= bilateral superior quadrantic defect
- extraocular nerve palsies
- features of pituitary insufficiency
Features of Pituitary apoplexy: examples of features of pituitary insufficiency?
hypotension/hyponatraemia secondary to hypoadrenalism
Ix for Pituitary apoplexy?
MRI diagnostic
Pituitary apoplexy Mx?
- urgent steroid replacement due to loss of ACTH
- careful fluid balance
- surgery
Post-LP headache?
headache following LP, occurs in 1/3 pts
Post-LP headache pathophysiology?
may relate to a leak of CSF during dural puncture
Post-LP headaches are more common in who?
females with low BMI
Post-LP headache typical features?
- usually 24-48hrs after LP but may be up to 1w later
- may last several days
- worsens with upright position
- improves with recumbent position
Headache in female with low BMI, improves lying down and worse upright?
Post-LP headache
Post-LP headache: factors which may contribute to headache?
Needle type: conventional ‘cutting’ needles increase the risk compared to pencil point (atraumatic) needles
Increased needle size
Direction of bevel
Not replacing the stylet
Increased number of LP attempts
Post-LP headache: factors which do not contribute to headache?
Increased volume of CSF removed
Bed rest following procedure
Increased fluid intake post procedure
Opening pressure of CSF
Position of patient
Mx of post-LP headache?
- supportive (analgesia, rest)
- if pain >72hrs then specific Tx to prevent subdural haematoma:
- includes= blood patch, epidural saline and IV caffeine
Progressive supranuclear palsy?
aka Steele-Richardson-Olszewski syndrome
a ‘Parkinson Plus’ syndrome
Progressive supranuclear palsy features?
postural instability and falls
- patients tend to have a stiff, broad-based gait
impairment of vertical gaze (down gaze worse than up gaze - patients may complain of difficultly reading or descending stairs)
parkinsonism
- bradykinesia is prominent
cognitive impairment
- primarily frontal lobe dysfunction
Mx of progressive supranuclear palsy?
poor response to L-dopa
Psychogenic nonepileptic seizures sometimes referred to as what?
pseudoseizures
Factors which favour psychogenic non-epileptic seizures (pseudoseizures)?
pelvic thrusting
family member with epilepsy
much more common in females
crying after seizure
don’t occur when alone
gradual onset
Factors that favour true epileptic seizures?
tongue biting
raised serum prolactin
What is helpful to differentiate psychogenic non-epileptic seizures (pseudoseizures) from true epileptic seizures?
video telemetry
Why is prolactin raised following true epileptic seizures?
hypothesised that there is spread of electrical activity to the ventromedial hypothalamus, leading to release of a specific prolactin regulator into the hypophyseal portal system
Pathophysiology behind raised intracranial pressure?
As the brain and ventricles are enclosed by a rigid skull, they have a limited ability to accommodate additional volume. Additional volume (e.g. haematoma, tumour, excessive CSF) will therefore lead to a rise in ICP
the normal ICP is 7-15 mmHg in adults in the supine position
cerebral perfusion pressure (CPP) is the net pressure gradient causing cerebral blood flow to the brain
CPP = mean arterial pressure - ICP
Causes of raised ICP?
idiopathic intracranial hypertension
traumatic head injuries
infection (meningitis)
tumours
hydrocephalus
Features of raised ICP?
headache
vomiting
reduced levels of consciousness
papilloedema
Cushing’s triad
- widening pulse pressure
- bradycardia
- irregular breathing
Ix and monitoring for raised ICP?
Neuroimaging (CT/MRI)= underlying cause
invasive ICP monitoring:
- catheter placed into the lateral ventricles of the brain to monitor the pressure
- may also be used to take collect CSF samples and also to drain small amounts of CSF to reduce the pressure
- a cut-off of > 20 mmHg is often used to determine if further treatment is needed to reduce the ICP
Cut of used to determine if further Tx is needed to reduce ICP (eg. after treating underlying cause and monitoring eg. using intraventricular monitor)?
> 20 mmHg
Mx for raised ICP?
- treat underlying cause
- head elevation to 30º
- IV mannitol may be used as an osmotic diuretic
- controlled hyperventilation
- removal of CSF
Mx for raised ICP: controlled hyperventillation?
aim is to reduce pCO2 → vasoconstriction of the cerebral arteries → reduced ICP
leads to rapid, temporary lowering of ICP. However, caution needed as may reduce blood flow to already ischaemic parts of the brain
Mx for raised ICP: removal of CSF?
drain from intraventricular monitor
repeated lumbar puncture (e.g. idiopathic intracranial hypertension)
ventriculoperitoneal shunt (for hydrocephalus)
Ankle reflex: root?
S1-S2
Knee reflex: root?
L3-L4
Biceps reflex: root?
C5-C6
Triceps reflex: root?
C7-C8
Reye’s syndrome?
severe, progressive encephalopathy affecting children that is accompanied by fatty infiltration of the liver, kidneys and pancreas.
Aetiology not fully understood but known association with aspirin use and a viral cause has been postulated
Reye’s syndrome features?
- peak incidence is 2yrs
- may be Hx of preceding viral illness or aspirin use
- encephalopathy= confusion, seizures, cerebral oedema, coma
- fatty infiltration of liver, kidneys and pancreas
- hypoglycaemia
Mx for Reye’s syndrome?
supportive
prognosis: mortality 15-25%
Spastic paraparesis?
UMN pattern of weakness in lower limbs
Causes of spastic paraparesis?
demyelination e.g. MS
cord compression: trauma, tumour
parasagittal meningioma
tropical spastic paraparesis
transverse myelitis e.g. HIV
syringomyelia
hereditary spastic paraplegia
osteoarthritis of the cervical spine
Spinal cord lesions: example of motor lesions?
Amyotrophic lateral sclerosis(MND):
- affects both upper (corticospinal tracts) and lower motor neurons
- results in a combination of upper and lower motor neuron signs
Poliomyelitis:
affects anterior horns resulting in lower motor neuron signs
Combined motor and sensory spinal cord lesions=
Brown-Sequard syndrome (spinal cord hemisection):
-tracts affected?
- features?
- Lateral corticospinal tract
- Dorsal columns
- Lateral spinothalamic tract
- Ipsilateral spastic paresis below lesion
- Ipsilateral loss of proprioception and vibration sensation
- Contralateral loss of pain and temperature sensation
Combined motor and sensory spinal cord lesions=
Subacute combined degeneration of spinal cord (vit B12 and E def):
-tracts affected?
- features?
- Lateral corticospinal tracts
- Dorsal columns
- Spinocerebellar tracts
- Bilateral spastic paresis
- Bilateral loss of proprioception and vibration sensation
- Bilateral limb ataxia
Combined motor and sensory spinal cord lesions=
Friedrich’s ataxia:
-tracts affected?
- features?
Same as subacute combined degeneration of the spinal cord
Same as subacute combined degeneration of the spinal cord
In addition cerebellar ataxia → other features e.g. intention tremor
Combined motor and sensory spinal cord lesions=
Anterior spinal artery occlusion:
-tracts affected?
- features?
- Lateral corticospinal tracts
- Lateral spinothalamic tracts
- Bilateral spastic paresis
- Bilateral loss of pain and temperature sensation
Combined motor and sensory spinal cord lesions=
Syringomyelia:
-tracts affected?
- features?
- Ventral horns
- Lateral spinothalamic tract
- Flacid paresis (typically affecting the intrinsic hand muscles)
- Loss of pain and temperature sensation
Combined motor and sensory spinal cord lesions=
Syringomyelia:
-tracts affected?
- features?
Asymmetrical, varying spinal tracts involved
Combination of motor, sensory and ataxia symptoms
Example of sensory spinal cord lesion?
Neurosyphilis (tabes dorsalis)
Tracts affected= Dorsal columns
Loss of proprioception and vibration sensation
Examples of disorders with combined motor and sensory spinal cord lesions?
- Brown-Sequard
- Subacute combined degeneration of spinal cord
- Friedrich’s ataxia
- Anterior spinal artery occlusion
- Syringomyelia
- MS
Spontaneous intracranial hypotension?
very rare cause of headaches that results from a CSF leak
leak typically from thoracic nerve root sleeves
Spontaneous intracranial hypotension RFs?
connective tissue disorders eg. Marfan’s
Spontaneous intracranial hypotension key features?
trong postural relationship with the headache generally much worse when upright. Patients may, therefore, be bed-bound
Spontaneous intracranial hypotension Ix?
MRI with gadolinium (contrast): typically shows pachymeningeal enhancement
Spontaneous intracranial hypotension Mx?
usually conservative
if this fails an epidural blood patch may be tried
Syringomyelia (‘syrinx)?
collection of CSF within the spinal cord
Syringomyelia vs syringobulbia?
Syringobulbia is a similar phenomenon in which there is a fluid-filled cavity within the medulla of the brainstem. This is often an extension of the syringomyelia but in rare cases can be an isolated finding.
Causes of syringomyelia?
strong association with Chiari malformation
trauma
tumours
idiopathic
Features of syringomyelia?
‘cape like’ (neck, shoulders and arms):
- loss of temp sens but preserved light touch, proprioception and vibration
- eg. pts accidentally burn hands without realising
spastic weakness (predom of lower limbs)
neuropathic pain
upgoing plantars
autonomic features= Horner’s, bowel and bladder dysfunction
What will occur in a matter of yrs if syringomyelia is not treated?
scoliosis
Horner’s in syringomyelia?
due to compression of sympathetic chain but this is rare
Why do pts with syringomyelia get a cape like (neck, shoulders and arms) loss of sensation to temp but preservation of light touch, proprioception and vibration?
due to the crossing of the spinothalamic tracts in the anterior commissure of the spinal cord being the first tracts to be affected
Pt accidentially burnt their hand without realising, but still have light touch, proprioception and vibration sensation. Loss of sensation to temp affecting neck, shoulders and arms. Also spastic weakness of lower limbs, neuropathic pain and bowel & bladder dysfunction?
syringomyelia
Ix for syringomyelia?
- MRI full spine with contrast= exclude tumour or tethered cord
- brain MRI= exclude Chiari malformation
Tx for syringomyelia?
treating the cause of the syrinx
pts with persistent or symptomatic syrinx, a shunt into the syrinx can be placed
Causes of tremor?
- parkinsonism
- essential tremor= postural tremor (worse if arms outstretched, improved by alcohol and rest, titubation and often strong FHx)
- anxiety
- thyrotoxicosis (usually thyroid signs eg. weight loss, feeling hot, tachycardia)
- hepatic encephalopathy (Hx of chronic liver disease)
- CO2 retention (Hx of COPD)
- Cerebellar disease= intention tremor + cerebellar signs eg. past-pointing, nystagmus
- drug withdrawl= alcohol, opiates
Summary of parkinsonism?
Resting, ‘pill-rolling’ tremor
Bradykinesia
Rigidity
Flexed posture, short, shuffling steps
Micrographia
‘Mask-like’ face
Depression & dementia are common
May be history of anti-psychotic use
Tuberous sclerosis?
genetic condition of autosomal dominant inheritance. Like neurofibromatosis, the majority of features seen in TS are neurocutaneous.
Cutaneous features of tuberous sclerosis?
depigmented ‘ash-leaf’ spots which fluoresce under UV light
roughened patches of skin over lumbar spine (Shagreen patches)
adenoma sebaceum (angiofibromas): butterfly distribution over nose
fibromata beneath nails (subungual fibromata)
cafe-au-lait spots may be seen (but MORE COMMON IN NEUROFIBROMATOSIS)
Neuro features of tuberous sclerosis?
developmental delay
epilepsy (infantile spasms or partial)
intellectual impairment
Other features of tuberous sclerosis (besides neuro and cutaneous)?
retinal hamartomas: dense white areas on retina (phakomata)
rhabdomyomas of the heart
gliomatous changes can occur in the brain lesions
polycystic kidneys, renal angiomyolipomata
lymphangioleiomyomatosis: multiple lung cysts
Left homoymous hemianopia means what?
visual field defect to the left i.e. lesion of right optic tract
Mnemonic for hemonymous quadrantanopias?
PITS (Parietal= Inferior; Temporal= Superior)
Visual field defects: incongruous defects vs congruous?
incongruous defects = optic tract lesion; congruous defects = optic radiation lesion or occipital cortex
A congruous defect simply means complete or symmetrical visual field loss and conversely an incongruous defect is incomplete or asymmetric.
Visual field defects: hemonymous hemianopia?
incongruous defects: lesion of optic tract
congruous defects: lesion of optic radiation or occipital cortex
macula sparing: lesion of occipital cortex
Visual field defects: homonymous quadrantanopias?
superior: lesion of the inferior optic radiations in the temporal lobe (Meyer’s loop)
inferior: lesion of the superior optic radiations in the parietal lobe
mnemonic = PITS (Parietal-Inferior, Temporal-Superior)
(in reality most actually caused by occipital lobe lesions, above is exam answers)
Visual field defects: bitemporal hemianopia?
lesion of optic chiasm
upper quadrant defect > lower quadrant defect = inferior chiasmal compression, commonly a pituitary tumour
lower quadrant defect > upper quadrant defect = superior chiasmal compression, commonly a craniopharyngioma
Von Hippel-Lindau (VHL) syndrome?
autosomal dominant condition predisposing to neoplasia. It is due to an abnormality in the VHL gene located on short arm of chromosome 3
Von Hippel-Lindau (VHL) syndrome features?
cerebellar haemangiomas: these can cause subarachnoid haemorrhages
retinal haemangiomas: vitreous haemorrhage
renal cysts (premalignant)
phaeochromocytoma
extra-renal cysts: epididymal, pancreatic, hepatic
endolymphatic sac tumours
clear-cell renal cell carcinoma
Hypoxic ischaemic encephalopathy (HIE)?
occurs in neonates as a result of hypoxia during birth
hypoxia- lack of O2
ischaemia- restriction of blood flow to brain
encephalopathy- malfunctioning of brain
Hypoxic ischaemic encephalopathy (HIE): is hypoxia normal?
some hypoxia at birth is normal but prolonged or severe leads to ischaemic damage
HIE can lead to permanent damage to brain causing cerebral palsy or death
Suspect HIE in neonates when there are events that could lead to hypoxia when?
perinatal or intrapartum period, acidosis (pH < 7) on the umbilical artery blood gas, poor Apgar scores, features of mild, moderate or severe HIE (see below) or evidence of multi organ failure.
Causes of HIE?
anything that leads to asphyxia (deprivation of O2) to brain eg.
- Maternal shock
- Intrapartum haemorrhage
- Prolapsed cord, causing compression of the cord during birth
- Nuchal cord, where the cord is wrapped around the neck of the baby
Mild HIE (Sarnat Staging)?
Poor feeding, generally irritability and hyper-alert
Resolves within 24 hours
Normal prognosis
Moderate HIE (Sarnat staging)?
Poor feeding, lethargic, hypotonic and seizures
Can take weeks to resolve
Up to 40% develop cerebral palsy
Severe HIE (Sarnat Staging)?
Reduced consciousness, apnoeas, flaccid and reduced or absent reflexes
Up to 50% mortality
Up to 90% develop cerebral palsy
Mx of HIE?
- supportive on neonatal unit= resus, ventilation, circulatory support, nutrition, acid base balance and Tx for seizures
- Therapeutic hypothermia is option sometimes to protect brain from hypoxic injury
- children need MDT and paeds follow up to assess development and support
HIE: therapeutic hypothermia?
For babies near or at term considered to have HIE.
Involves actively cooling the core temperature of the baby according to a strict protocol. The baby is transferred to neonatal ICU and actively cooled using cooling blankets and a cooling hat. The temperature is carefully monitored with a target of between 33 and 34°C, measured using a rectal probe. This is continued for 72 hours, after which the baby is gradually warmed to a normal temperature over 6 hours.
HIE: therapeutic hypothermia aim?
reduce the inflammation and neurone loss after the acute hypoxic injury. It reduces the risk of cerebral palsy, developmental delay, learning disability, blindness and death.
