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
