New Neurology Flashcards
What is presbycusis?
Age related sensorineural hearing loss
What is Otosclerosis?
autosomal dominant replacement of normal bone with spongy vascular bone.
What is menieres disease?
Recurrent episodes of vertigo, tinnitus and sensorineural hearing loss, lasting mins-hours. Vertigo main complaint!
Middle aged adults
Feeling of aural fullness/pressure
Nystagmus/positive romberg test
Investigations in Meniere’s disease
Menieres triad
- Otoscopy - Normal ear drum
- Audiometry - Sensorineural hearing loss
- Tympanometry - normal
Pharmacological mx of menieres
Prochlorperazine (acute vertigo and nausea) [Acute attacks]
Betahistine medication (H1 agonist that acts as a Vestibular sedative) [Prevention]
Intratympanic gentamicin injection if surgical
What is Acoustic Neuroma?
AKA vestibular schwanomma. Tumour arising from schwann cells myelinating CN8. Usually presents between 40-60yo.
Associated with type 2 neurofibromatosis
Presents similar to menieres (vertigo, tinnitus, S hearing loss) BUT also has absent corneal reflex and possible facial paralysis
Affected cranial nerves:
- Men. symptoms (VIII)
- Absent corneal reflex (V)
How can acoustic neuroma present?
Depends on cranial nerves affected
- CN5: Absent corneal relfex
- CN7: Facial palsy
- CN8: Unilateral sensorineural hearing loss and tinnitus, vertigo.
Ix of Acoustic Neuroma
Audiogram and examination show sensorineural hearing loss.
MRI Gold standard imaging for diagnosis and tumour tracking.
Mx and comps of acoustic neuroma
Conservative or
Tumour excision or
Radiotherapy
Permanent hearing loss (CN8), permanent facial weakness (CN7)
What is an essential tremor, give features and management?
Autosomal dominant condition usually affecting both arms.
Postural tremor: worse when arms stretched out
Improved by alcohol and rest
Most common cause of titubation (head tremor)
Managed with propanolol, or primidone second line
Define Extradural Haemorrhage with its main cause and epidemiology
Cranial bleeding above the dura mater.
Usually caused by trauma to pterion of skull, causing rupture of middle meningeal artery in temporo-parietal region. Can associate with temporal bone fracture.
Usually found in young adults
Blood doesnt cross suture lines
Why can extradural stroke present slowly at first before becoming more severe?
If bleeding is slow, symptom onset is slower (lucid interval) before there is a sudden, rapid decline when intracranial pressure increases enough to compress brain
Describe non contrast ct appearance in extradural haemorrhage (3)
Biconvex, hyperdense haematoma
Blood doesnt cross suture lines
Shows midline shift (increased pressure can cause cause brain shifting/herniation)
What are the 2 main herniation complications of haemorrhagic stroke?
Supratentorial herniation (cerebrum against skull, compressing arteries and causing ischaemic stroke)
Infratentorial herniation (Cerebellum pushed against brainstem, compressing area that controls consciousness, respiration, heart rate)
What is cushings triad and how is it treated?
Body’s response to increased intracranial pressure, signifies severe lack of oxygen in brain tissue
- Bradycardia
- Irregular respirations
- Widened pulse pressures (increased systolic, decreased diastolic)
Treated with IV mannitol to reduce ICP
Define Subdural haemorrhage with its main cause and epidemiology
Bleeding below dura mater, caused by bridging vein rupture.
Usually occur in elderly/alcoholic patients but can occur in babies (shaken baby syndrome)
causes of bridging vein rupture
Brain atrophy; with age. Stretches bridging veins, meaning they stretch over gaps unsupported.
Alcohol abuse: Causes walls of vein to thin
Trauma
Falls
Shaken baby syndrome
Acceleration/deceleration injury
non contrast ct appearance of subdural haemorrhage
Bleeding between the dura mater and arachnoid
- Follows contours of brain and crosses suture lines, forming a crescent shape
Acute (hyperdense mass)
Chronic (Hypodense mass)
Acute on Chronic (both)
what gcs requires intubation
8 or below
specific surgery in subdural haemorrhage
Burrhole washout if haemorrhage small
Craniotomy if large haemorrhage
define subarachnoid haemorrhage with main cause
Bleeding below the arachnoid layer, where CSF is located.
Main cause is a ruptured saccular (or Berry) aneurysm, with majority located between anterior communicating artery and anterior cerebral artery
RF for subarachnoid haemorrhage (7)
PKD (Associated with berry aneurysm)
Connective tissue disorders (Ehlers-Danlos, Marfans)
Family history
Increasing age
HTN
Smoking
Alcohol
typical presentation of subarachnoid haemorrhage
Sudden onset occipital “thunderclap” headache, following strenuous activity, with associated neck stiffness and photophobia. Smaller, “Sentinel” headache may have preceded thunderclap
Black, female, 45-70
signs/symptoms of subarachnoid haemorrhage
Thunderclap headache
Meningism (Headache, photophobia + neck stiffness)
Fixed dilated pupil (third nerve palsy - especially in posterior communicating artery rupture)
6th nerve palsy
Kernigs and Brudzinskis due to meningism also
Nausea/vomiting, weakness, confusion, coma, reduced consciousness, speech reduction
ix of subarachnoid haemorrhage
Urgent non contrast CT head (blood in subarachnoid space/basal cisterns)
CT angiography to locate bleed source
ECG to detect arrhythmia/abnormality
If CT non conclusive,
- Lumbar puncture (RBCs in CSF and Xanthochromia) 12 hours after onset.
Define Kernig’s and Brudzinski’s signs
Kernig - Inability to straighten bent leg without pain when hip flexed to 90 degrees
Brudzinski - Passive flexion of neck in supine patient elicits hip and knee flexion
Suggest meningitis/meningism
ct appearance in subarachnoid haemorrhage
Blood in subarachnoid space (hyperdense)
- Star shaped lesion (Blood filling in gyro pattern)
Mx of subarachnoid haemorrhage
Surgical 1st/GOLD
- Endovascular coiling (clipping also possible but more complications)
Nimodipine to prevent vasopasms
IV Mannitol to reduce ICP
Sodium valproate for seizures
Define meningitis
Inflammation of the meninges (specifically leptomeninges - pia and arachnoid). Can be due to viral, bacterial or fungal cause.
Notifiable disease
Viral Causes of meningitis
More common but less severe
- Coxsackie virus
- HSV (Herpes simplex virus)
- Varicella Zoster virus
- Mumps
bacterial causes of meningitis
Most common - S. pneumoniae and N. meningitidis
Children - ^ and H influenzae
Elderly and pregnant - Listeria Monocytogenes (pregnant avoid cheese)
Newborns - ^ and Group B strep
How do n meningitidis, s pneumoniae, group b strep and listeria monocytogenes present on gram film?
N meningitidis - Gram negative diplococci (Only one that causes non blanching rash!)
S pneumoniae/Group B strep - Gram positive cocci in chains
Listeria monocytogenes - Gram positive bacillus
signs/symptoms of meningitis
Signs
- Neck stiffness, headache, photophobia (avoids light)
- Phonophobia (avoid sound)
- Papilloedema (optic disk swelling)
- Kernig sign
- Brudzinski sign
- Non blanching rash (N meningitidis only)
Pyrexia, reduced GCS
ix of meningitis
Blood culture 1st line - Bacterial or negative for viral
Lumbar puncture GOLD
Bacterial
- Cloudy/yellow
- Protein high
- Glucose low (<50% normal)
- WCC high (Neutrophil)
Viral
- Clear appearance
- Protein small raise/normal
- Glucose normal (>60% normal)
- WCC high (lymphocytes)
(Gram stain identifies bacteria and CSF PCR identifies viruses)
fungal appearance of csf in meningitis
Cloudy and fibrous
Protein high
Glucose low
WCC high - Lymphocytes!
mx of bacterial meningitis
Primary care: Immediate IV or IM benzylpenicillin (if suspected meningococcal) and hospital referral
Hospital
- Dexamethasone (steroid)
- Cefotaxime or Ceftriaxone IV
Give Amoxicillin if under 3 months or over 50 to cover listeria
Contact tracing and single dose oral ciprofloxacin for contacts
comps of meningitis
Hearing loss
Seizures
Cognitive impairment
Hydrocephalus
Sepsis
Upper motor neurone lesion signs vs lower motor neurone lesion signs
Type of paralysis
-reflexia
Fasciculations
Babinski sign
Voluntary movement
Muscle tone and power
UMN
Spastic paralysis
Hyperreflexia
No fasciculations
Babinski positive
Voluntary movement slowed
Muscle tone and power kept
LMN
Flaccid paralysis
Hyporeflexia
Fasciculations
Babinski negative
Voluntary movement gone
Muscle tone and power lost
(babinski - toes curl up when bottom of foot is stroked
fasiculations - brief spontaneous contractions under skin)
define ms
Type 4 hypersensitivity reaction in which there is autoimmune attack against oligodendrocytes (which create myelin) in the CNS (Brain/Spinal cord). Causes plaques of demyelination.
Lesions vary, meaning plaques are “disseminated in space and time” - affect different areas of CNS at different times/ events.
disease progression in ms
Relapsing remitting (most common) - Episodic flare ups without full recovery in between, meaning flares worsen over time. (Most common and often progress to secondary progressive)
Secondary progressive - Symptoms start getting worse without remission
Primary progressive - Symptoms worsen without remission (/)
Progressive relapsing - Constant attack with superimposed flare ups
what is uhtoffs phenomenon?
Symptoms worsen with heat (e.g. hot bath) or exercise.
New myelin is inefficient, and doesn’t tolerate temperature rise effectively.
what triad is associated with ms?
Charcot’s neurological triad
- Nystagmus (involuntary side-to-side/up-down rapid eye movements)
- Dysarthria (slowed, slurred speech)
- Intention tremor
signs/symptoms of ms
Optic neuritis usually first (Loss of vision, eye pain, pale optic disk, double vision)
Internuclear ophthalmoplegia (eye muscle paralysis which impairs lateral gaze)
Lhermitte’s sign - Electric shock sensation when flexing neck
UPPER motor neurone signs
Bowel, bladder, erectile dysfunction
Ataxia
Sensation loss
(Uhthoff’s and Charcot’s neurological triad already mentioned)
what criteria is used in diagnosis of ms?
McDonald criteria (think McDonald’s M!)
- 2 or more relapses with evidence of 2 or more lesions, or one lesion with reasonable history of relapse
ix in ms (3)
MRI Brain/Spine
- Demyelinating plaques (new enhance with contrast, old don’t - showing dissemination in space and time)
Lumbar puncture
- Oligoclonal IgG bands in CSF
Visual evoked potential studies (responses to visual stimulus)
- Shows delayed nerve conduction
mx of ms
During acute relapse
- Oral/IV methylprednisolone first, cladribine
- Plasma exchange
Maintenance
- Interferon beta
- IV monoclonal antibodies
Cladribine second line in ongoing secondary progressive but causes cancer and is teratogenic
comps of ms and there managements (3)
Spasticity - Baclofen and gabapentin
Neuropathic pain/depression - amitriptyline
Physiotherapy for Spasticity and mobility impairment
what are some disease modifying durgs in ms with their indications?
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)
IV Natalizumab - monoclonal antibody
IV Ocrelizumab
Oral fingolimod
SC beta interferon
SC Glatiramer acetate
what is huntingtons?
Autosomal dominant trinucleotide repeat disorder, which causes deterioration of nervous system and an excess of dopamine.
Also known as Huntington’s chorea (Chorea= involuntary jerky movements)
HTT gene on chromosome 4 - Mutated Huntingtin proteins aggregate in neuronal cells of caudate and putamen. Causes cell death of GABAergic and cholinergic neruones, causing ACh and GABA deficiency, so less dopamine inhibition. Excess dopamine causes excess movement.
Genetic anticipation - The more copies of the protein DNA polymerase adds on in the sperm, the earlier onset and more severe the disease
signs and symptoms of huntingtons?
Usually asymptomatic until 30-50 years
Prodromal - Irritability, depression, cognitive problems
Chorea - Jerky involuntary movements
Eye movement disorders
Dysphagia/dysarthria
Dementia, seizures, death within 15 years
ix in huntingtons
Clinical diagnosis
- Genetic testing GOLD
CT/MRI - Caudate and striatal atrophy - increased size of lateral ventricles
other causes of chorea
Hyperthyroid
Wilson’s
SLE
Dementia
mx of huntingtons
Uncurable
Chorea
- Diazepam and tetrabenazine (Benzodiazepine and dopamine depleting agent)
define parkinsons disease
Neurodegenerative movement disorder characterised by loss of dopaminergic neurones in Substantia Nigra Pars Compacta of basal ganglia.
Misfolded a synuclein proteins called Lewy bodies also present histologically (dark eosinophilic inclusions)
Causes a dopamine deficiency
what are the parkinsonism symptoms
Resting Tremor
Bradykinesia
Rigidity
Postural instability
signs and symptoms of parkinsons (other than parkinsonisms)
Resting tremor
Cogwheel rigidity
Shuffling gait
Reduced arm swing
Non motor:
Loss of smell
Sleep disturbance
Depression, anxiety
Dementia
ix in parkinsons
Clinical diagnosis - bradykinesia and 1 other Parkinsonism sign
(Bradykinesia = slow, difficult movements. Smaller handwriting, shuffling gait, reduced arm swing etc)
Dopamine agent trial shows improvement
mx of parkinsons
If Severe: Levodopa + Decarboxylase inhibitor (boost dopamine and Di prevents L-dopa breakdown)
- Co-careldopa (Levodopa and carbidopa)
Otherwise:
Dopamine agonist
- Ropinirole
Monoamine oxidase B inhibitor (MAOBi) (stop breakdown of circulating dopamine)
- Selegiline
comps of parkinsons
Disease progression and motor fluctuations (off periods when treatment stops working)
- Freezing (sudden stop of movement)
- Dyskinesia
- Dementia
differentials of parkinsonisms
Benign Essential Tremor
Wilson’s disease
Encephalitis causing degeneration of substantia nigra
Trauma
define myasthenia gravis
Type 2 hypersensitivity reaction causing autoimmune destruction of the post synaptic membrane at the neuromuscular junction of skeletal muscle. Antibodies to acetylcholine receptors in 85% of cases. (Anti-AChR)
2x in women.
Mostly affects facial muscles.
Strong association with thymoma/ thymic hyperplasia
signs/symptoms of myasthenia gravis (6)
Mostly affects proximal and small muscles of head and neck
- Muscle weakness with fatigability, worse with exertion better with rest (e.g. patient counting to 50 will struggle in later numbers)
- Ptosis (eyelid droop) and diplopia (double vision)
- Jaw weakness and weak swallow (dysphagia)
- Head drop
- Facial paresis and slurred speech
- Snarl when attempting to smile (myasthenic snarl)
how to check for muscle fatigability on examination (3) and what should you check? (myasthenia)
Repeated blinking causing ptosis
Counting to 50, speech becomes slurred and quieter towards end
Repeated abduction of one arm will result in weakness in said arm compared to other
Forced Vital Capacity should also be checked
ix in myasthenia gravis?
Antibodies
- AchR antibodies (anti-MuSK and anti-LRP4 less sensitive)
- Anti-MUSK
- Anti LRP4
CT thorax - look for thymus growth/thymoma (rule out)
Tensilon test - Used to be done but causes arrhythmia so dont even think about it
drugs that exacerbate MG muscle fatigability
penicillamine
quinidine, procainamide
beta-blockers
lithium
phenytoin
antibiotics: gentamicin, macrolides, quinolones, tetracyclines
Most common factor is exertion!
mx of myasthenia gravis
Long acting acetylcholinesterase inhibitor
- Pyridostigmine first line
Immunosuppression (not started at diagnosis, usually started later)
- Prednisolone
Thymectomy may be needed (as many patients also have thymic hyperplasia)
myasthenia gravis brief pathophysiology
Unexplained destruction of Only UMNs and LMNs.
- No effect on sensory neurones (distinguishing point from MS etc)
- No effect on eyes (distinguishing from myasthenia gravis)
- No cerebellar involvement
- SOD1 mutation association in ALS
Define guillain barre syndrome with causes
Acute autoimmune demyelination of the peripheral nervous system, following an upper resp tract or GI infection (e.g. gastroenteritis)
Acute, symmetrical, ascending weakness!
Can be caused by:
Bacteria:
- Campylobacter Jejuni
- M. pneumoniae
Viral:
- Cytomegalovirus
- EBV
guillaine barre disease course (4)
Initial GI or URT infection
Symptoms start after 2 weeks
Symptoms peak 2-4 weeks further
Recovery period of months to years
pathophysiology of guillain barre syndrome
Molecular mimicry.
Pathogenic antigens resemble Schwan cell proteins so when immune response is launched, there is also destruction of myelin sheath. Demyelination occurs in patches down length of axon (segmental demyelination). Schwann cells can remyelinate so patients recover over time. Affects sensory and motor nerves.
signs and symptoms of guillain barre syndrome
Symptom onset 2-3 weeks after preceding infection. Proximal muscles affected first
Symmetrical ascending weakness beginning in legs/feet.
Areflexia
Reduced sensation
Paraesthesia
Sensory loss
Respiratory distress if lungs affected
Autonomic dysfunction (bowel/bladder, sweating, raised BP/pulse, arrhythmia)
ix of guillain barre syndrome
Brighton criteria used to make clinical diagnosis, can be supported by:
- Nerve conduction studies (reduced conduction)
- Lumbar puncture (high protein in CSF, normal cell count and glucose)
Antibodies: subtype of GBS
AIDP (90%) - Anti ganglioside
Miller fisher syndrome (eyes affected first) - anti GQ1b
Do spirometry to assess risk of resp failure
mx of guillain barre syndrome (2 tx + 2 complication tx)
IV immunoglobulin 5 days (CI if IgA deficiency)
Plasma exchange
VTE Prophylaxis (LMWH)
Ventilation if low FVC
umn lesion area
anywhere from pre central gyrus to anterior spinal cord
lmn lesion area
anwhere from anterior spinal cord to innervated muscle
how is muscle power affected in umn lesions?
In arms flexors>extensors
In legs extensors>flexors
types of mnd
Amyotrophic lateral sclerosis (50% of patients)
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
Primary lateral sclerosis
UMN signs only
Progressive muscular atrophy
LMN signs only
affects distal muscles before proximal
carries best prognosis
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
presentation of mnd
asymmetric limb weakness is the most common presentation of ALS
the mixture of lower motor neuron and upper motor neuron signs
wasting of the small hand muscles/tibialis anterior is common
fasciculations
the absence of sensory signs/symptoms
vague sensory symptoms may occur early in the disease (e.g. limb pain) but ‘never’ sensory
mx of mnd (2)
what is prognosis?
Riluzole (protects neurones from glutamate induced damage)
Respiratory support (non invasive at home)
50% die in 3 years
gene associations with alzheimers?
APoE e4 - APoE usually helps break down beta amyloid but e4 version less effective.
Down’s (Trisomy 21) - Increased APP (amyloid precursor protein) production (APP gene also on C21). APP broken down incorrectly becomes beta amyloid.
PSEN1, PSEN2
key histological findings in alzheimers
Senile plaques of beta amyloid proteins (APP incorrectly broken down into sticky, insoluble b amyloid) (extracellular)
Neurofibrillary tangles of hyperphosphorylated tau proteins (intracellular)
signs/symptoms of alzheimers (4)
Insidious onset and slow progressive decline
Poor memory (short term early, long term late)
Speech problems (receptive and expressive dysphagia)
Loss of executive function (planning/problem solving)
Disorientation/lack of recognition of places, people or objects
how would alzheimers affect behaviour? (4)
Emotional instability
Depression/anxiety
Withdrawal/apathy
Disinhibition (Socially/sexually inappropriate behaviour)
how would alzheimers affect daily living? (3)
Loss of independence
Early on loss of higher level function (finances, difficulties working)
Later loss of basic function (washing, eating, walking)
how is alzheimers diagnosed?
Based on DSM-V criteria and MMSE (Mini mental state examination) (25+ normal, <17 severely impaired)
MRI - Generalised brain atrophy with medial temporal then later parietal predominance
Brain biopsy is GOLD but can only be done after death
Mx of alzheimers
Supportive: Improve cognitive function
- Exercise
- Music
- Board games
- Cognitive stimulation program
ACh-esterase inhibitor (Donepezil)
NMDA receptor antagonist (Memantine)
Define Vascular dementia
Dementia caused by cerebrovascular damage causing hypoperfusion of neuronal cells.
Presents in patients with Stroke/TIA history, UMN signs and general condition decline.
Shows a stepwise decline with symptoms worsening after each cerebrovascular event.
Investigations and treatment of vascular dementia
Mini mental state exam
CT/MRI of brain
- Multiple cortical and subcortical infarcts
- Atrophy of brain cortex
Treated with management of risk factors (lower BP, cholesterol, diabetes etc)
define lewy body dementia
Dementia with Parkinsonism (Resting tremor, bradykinesia, rigidity, postural instability).
Alpha synuclein misfolds in neurones and aggregates to form Lewy Bodies, which deposit in cortex and substantia nigra causing neuron death.
sx of lewy body dementia
Presents with dementia symptoms first
(memory, focus, speech, understanding issues)
Parkinsonism develops later
Sleep disorders like sleep walking/talking, and hallucinations are also very common in LBD
If Parkinsons first, it is Parkinson Dementia
mx of lewy body dementia
Dopamine analogue - Levodopa
ACh-esterase inhibitor - Donepezil
Define fronto temporal dementia
Focal degeneration of frontal and temporal lobes. Pick’s disease is most common type. Loss of over 70% of spindle neurones.
Frontotemporal dementia (Pick’s disease)
Progressive non fluent aphasia (Chronic progressive aphasia)
Semantic dementia
common features of frontotemporal lobar dementias
Onset before 65
Insidious onset
Relatively preserved memory and visuospatial skills
What is Pick’s disease, how does it present and what macroscopic and microscopic changes are found
AKA Frontotemporal dementia.
Personality change and impaired social conduct! Hyperorality, disinhibition, increased appetite, perserveration beahviours.
Focal gyral atrophy with knife-blade appearance
Macro
- Atrophy of frontal and temporal lobes
Micro
- Pick bodies - spherical aggregations of tau protein (silver staining)
- Gliosis
- Senile plaques and neurofibrillary tangles
What is cpa? (chronic progressive aphasia?
Clues in the name.
Short utterances that are agrammatic. Comprehension preserved
what is semantic dementia?
Fluent progressive aphasia. Speech fluent but empty, not much meaning. Memory better for recent rather than remote events
what are pick bodies?
3R isoform of tau proteins
These become hyperphosphorylated and form tangles, causing atrophy in frontoteporal lobes
(In alzheimers, the isoform is 3R+4R)
Give primary and secondary causes of headache
Primary
- Migraine
- Tension
- Cluster
Secondary (to other pathology)
- GCA
- Cerebrovascular disease
- Subarachnoid haemorrhage
- Truma
define migraine
Episodes of recurrent, unilateral throbbing headache. May or may not have an aura and often has visual changes (e.g. photophobia, diplopia etc).
Can last up to 72 hours, and classically preceded by an aura
- Visual, progressive, lasting 5-60 minutes.
Common migraine triggers
CHOCOLATE
Chocolate
Hangover
Orgasms
Cheese
Oral contraceptive
Lie ins (tiredness)
Alcohol
Tumult (loud noise)
Exercise
Signs/symptoms of typical/atypical aura
Typical: Lasts 5-60 mins and fully reversible.
- Visual changes (zigzag lines, distortion etc)
- Smell changes
- Paraesthesia
Atypical: >60 mins
- Diplopia
- Motor weakness (hemiplegic migraine!)
- Poor balance
- Reduced consciousness
Ix in migraine
Clinical diagnosis:
Migraine with/without aura
(at least 2/4 symptoms, 1 associated symptom, no attribution to another disorder)
CT/MRI to exclude secondary haemorrhage
ESR exclude GCA
tx of migraine
Acute prevention
- Oral Sumatriptan (5-HT receptor agonist (mimic serotonin))
with/without aspirin
Prophylaxis
- Propanolol
- Amitriptyline
- Topirimate (antiemetic)
AVOID Opiates
define tension headache
Most common type of headache.
Bilateral “pressing/tight” headache. Lasts minutes to hours. No associated symptoms except photo OR phonophobia
Define cluster headache
Severe, unilateral periorbital headache, with associated autonomic features, affecting same side face/eyes. Lasts 15-180 mins.
AKA Trigeminal Autonomic Cephalalgia
Signs/symptoms of cluster headache
Severe unilateral, periorbital, crescendo headache, lasting 15mins to 3 hours. Clusters of headaches,
(Boring/hot poker pain “worst pain ever”)
Ipsilateral autonomic symptoms
- Ptosis (eyelid droop)
- Miosis (excessive constriction of pupil of eye)
- Teary, bloodshot eye
- Nasal congestion/rhinorrhoea
diagnosis and mx of cluster headache
Clinical diagnosis (5+ similar headaches)
Acute
- Triptans
- High flow oxygen
(AVOID paracetamol, NSAID, Opioids)
Prophylaxis
- Verapamil (CCB)
- Prednisolone, 2-3 weeks
define trigeminal neuralgia
Severe, unilateral “electric” pain along distribution of trigeminal nerve lines. Extremely increased risk in demyelinating disease. Attacks last seconds.
Pathophysiology of trigeminal neuralgia
Vascular loop (MC superior cerebellar artery) compresses nerve near nerve root entry zone. Compression causes poor conduction along nerve root, causing pain.
common triggers of trigeminal neuralgia pain
Light touch (washing, shaving, brushing teeth)
- Talking
- Cold weather
- Spicy food
- Caffeine and citrus
What are the branches of the trigeminal nerve
Ophthalmic
Maxillary
Mandibular
Signs/Symptoms of trigeminal neuralgia?
Facial pain
- Electric/stabbing pain
- Very severe
- Trigeminal distribution
- Unilateral
- Provoked (touch, cold etc)
Ix and Mx of trigeminal neuralgia
Clinical but can MRI brain (space occupying lesion, demyelination etc)
Carbamazepine first line
Surgery (microvascular decompression)
define cauda equina
Compression of the bundle of nerves below the end of the spinal cord (known as cauda equina). Causes bilateral lower limb weakness/saddle anaesthesia
Medical emergency that requires immediate decompression
Signs/Symptoms of cauda equina
Severe lower back pain, bilateral lower limb weakness and reduced sensation. LMN signs!
Saddle anaesthesia (numbness/reduced tone in perianal region, groin, inner thigh)
Decreased reflexes and leg weakness/paralysis
Erectile dysfunction
Bladder/bowel dysfunction.
Ix and tx of cauda equina
URGENT MRI spine GOLD
Emergency decompressive laminectomy (vertebra removal) within 24-48 hours, or permanent weakness/dysfunction
brief overview of anatomy of spine
Originates at base of medulla oblongata, exiting through foramen magnum, ending at conus medullaris at L2
Consists of 5 sections of vertebrae, with 31 spinal nerves arising from this
- Cervical (7)
- Thoracic (12)
- Lumbar (5)
(Spinal cord ends at L1, conus medullaris begins at L2)
- Sacrum (5 - fused)
- Coccyx (4 - fused)
Beyond L2 are bundle of nerves called “cauda equina”
causes of spinal cord compression
Vertebral body neoplasms (thoracic most likely)
- disc herniation
- disc prolapse
- infection
- Trauma
- Spinal stenosis
how do spinal cord lesions secondary and motor symptoms present?
Motor - Contralaterally
Sensory - Ipsilaterally (same side)
what nerve roots are implicated in knee jerk, big toe jerk and ankle jerk reflexes?
Knee jerk - L3/4
Big toe - L5
Ankle - S1
signs/symptoms of spinal cord compression
Progressive (Hours-weeks/months) back pain and progressive leg weakness. Motor signs contralateral
UMN signs above level of lesion
LMN below level of lesion
Sensory loss 1-2 cord segments below lesion level
bladder/sphincter involvement is a late, bad signs
what levels are lesions in l5 nerve root compression and sciatica?
L5 nerve root compression - L4/L5
Sciatica - L5/S1
specific causes of peripheral neuropathy
DAVID
Diabetes
Alcoholism
Vitamin B12 deficiency
Infective/inherited (GBS/Charcot-Marie-Tooth)
Drugs e.g. isonazid
what is brown sequard syndrome?
Damage to one half of the spinal cord, resulting in a specific pattern of symptoms:
- Ipsilateral motor weakness
Ipsilateral loss of proprioception (position sense), light touch, vibration at level of lesion
Contralateral loss of pain and temperature sense below level of lesion
gold ix and mx of brown sequard
Investigations
- EMG (electromyography)
- MRI Spinal cord
Management
- Treatment of underlying condition
- High dose steroids
Define/ explain pathophysiology of Charcot Marie Tooth syndrome
Group of inherited diseases (autosomal dominant) that cause axonal/myelin dysfunction. CMT1 and CMT2 most common
CMT 1 - loss of myelin sheath (onion bulb myelin due to schwann cell repair)
CMT 2 - Neuronal mitochondrial dysfunction = neurone death
Causes atrophy of muscle when motor neurones affected
Signs/symptoms of charcot marie tooth
Weakness in lower legs and hands.
Loss of muscle tone and reflexes
Foot drop and claw hand
Tingling/burning in hands and feet
Thickened palpable nerves and hammer toes
“Inverted champagne bottle” legs due to distal muscle wasting
Define Duchenne Muscular Dystrophy
X linked recessive condition characterised by severe muscle dystrophy due to absence of Dystrophin protein
Signs/symptoms of duchenne muscular dystrophy
Usually presents 3-5 years.
- Weakness in pelvic muscles
- Waddling gait
- Gowers sign: due to inability to get up normally, they get into downward dog position then climb their hands up their legs to stand.
- Fat calves due to buildup of fat and fibrotic tissue rather than muscle
Nerves implicated in wrist drop and claw hand
WD - Radial
Cl - Ulnar (4th and 5th fingers claw)
What is Bells palsy
Acute, unilateral, idiopathic facial nerve paralysis.
Aetiology unknown, but suspected HSV
How does Bells palsy present
LMN Facial nerve palsy -> Forehead affected
(UMN spares upper face)
- Post auricular pain
- Altered taste
- Dry eyes
- Hyperacusis
How is Bell’s palsy treated
Prednisolone within 72 hours, maybe add antiviral
most people with Bell’s palsy make a full recovery within 3-4 months
What is temporal arteritis
AKA Giant cell arteritis. Branches of carotid artery (
Occurs in >50, usually ~70. Strong association with Polymyalgia Rheumatica.
Early recognition and treatment can minimise risk of complications e.g. permanent sight loss.
How does temporal arteritis present
Rapid <1 month onset
Unilateral headache around temple/forehead
Diminished/absent temporal artery
Jaw claudication
Blurred/double vision
Optic disc pallor
Scalp tenderness (Painful to comb)
Fever, muscle aches, weight loss, loss of appetite
GCA typical presentation
50+ white female with unilateral temple headache, scalp tenderness (painful to comb), jaw claudication and vision changes.
Pathophys of GCA
Granulomatous vasculitis of large/medium arteries. Arteries become inflamed, intima is thickened and vascular lumen is narrowed. Usually cerebral (temporal) arteries affected:
- Superficial temporal artery: Headache/scalp tenderness
- Mandibular artery: jaw claudication
- Ophthalmic artery: visual loss (retinal ischaemia)
Diagnostic criteria in GCA
3 of:
- Over 50
- New headache
- Temporal artery tenderness/diminished pulse
- ESR Raised
- Abnormal temporal artery biopsy
Management of GCA
High dose prednisolone
Sight loss (amaurosis fugax!)
Should be dealt with ASAP or could lead to permanent blindness
Ischaemic cranial complications (Visual loss/stroke)
Aortic aneurysm
In cases where long term steroids are given, what 2 systems should be protected and how is this done?
GCA
GI (stomach and oesophagus) and Bones
- PPI (omeprazole)
- Alendronate (bisphosphonate)
- Ca2+ and vitamin D
Define Polymyalgia Rheumatica
symptoms
ix
mx
Condition that causes pain stiffness and inflammation in neck, shoulders and hips. Limits range of motion. Occurs alongside GCA often.
Morning pain/stiffness in shoulders etc. Leads to fatigue, fever, weight loss, anorexia and depression
Raised ESR but CK and EMG normal
Managed with prednisolone
What is Broca’s area, and what does an injury of it cause?
Located in the frontal lobe of person’s dominant side (left in right handed people) and influences motor production of speech.
Causes expressive aphasia - Patients can understand speech but can’t produce it themselves
What is Wernickes area and what does injury of it cause
Located in parietal and temporal lobe of person’s dominant side (left in right handed people) , influences understanding of speech and using correct words to express thoughts
Causes receptive aphasia - Patients can produce speech but don’t understand the meaning of spoken words
What are Wernickes and Brocas areas supplied by?
Middle Cerebral Artery
What is a watershed area?
Areas furthest from blood supply, most susceptible to infarction
Define ischaemic stroke
Reduced cerebral blood flow due to arterial occlusion or stenosis. Account for 85% of all strokes.
Consists of rapidly developing signs of cerebral dysfunction, lasting more than 24 hours, with no apparent cause.
Causes of ischaemic stroke
Disruption of blood supply secondary to:
- Thrombus formation/ embolus
- Atherosclerosis
- Shock
- Vasculitis
- Hypercoagulability (thrombophilia)
Risk factors for stroke (9)
Male
Old (>55)
Black or Asian
History of Ischaemic stroke or TIA
Atrial fibrillation
Sickle cell disease
Combined contraceptive pill
Carotid artery stenosis
Smoking, obesity, HTN, T2DM
Define hemiparesis
Weakness or paralysis on one side of the body
Manifestations of an Anterior Cerebral Artery ischaemic stroke
Contralateral hemiparesis and sensory loss, affecting lower limbs>upper limbs
Manifestations of middle cerebral artery stroke
Contralateral hemiparesis and sensory loss affecting upper limbs>lower limbs.
Aphasia if affecting Broca or Wernickes areas in dominant hemisphere
Homonymous hemianopia (visual field defect in same side of both eyes)
Manifestations of Posterior Cerebral Artery stroke
Contralateral homonymous hemianopia with macular sparing (preservation of central visual field)
Contralateral loss of pain and temperature
Manifestations of Vertebrobasilar artery stroke
Cerebellar signs (intention tremor, nystagmus, hypotonia)
Reduced consciousness
Quadriplegia
Manifestations of Webers syndrome (midbrain infarct)
Oculomotor palsy and contralateral hemiplegia
Manifestations of Lateral medullary syndrome (posterior inferior cerebellar artery occlusion)
Ipsilateral facial loss of pain and temperature
Ipsilateral Horner’s syndrome: miosis, ptosis, anhidrosis
Ipsilateral cerebellar signs
Contralateral loss of pain and temperature
What is lacunar stroke?
Stroke affecting small branches of middle cerebral artery.
Causes one of the following:
Pure motor loss
pure sensory loss,
ataxic hemiparesis
What is pronator drift
Ask patient to raise arms. On the affected side, palm and arm will face inward and downwards. Suggests muscle weakness.
(Symptoms contralateral to affected brain side)
What assessment system is used in acute strokes
ROSIER (Recognition of Stroke In Emergency Room)
Uses symptoms as + points and mimics (syncope, seizure activity) as - points
Investigations on stroke presentation
Assessment using ROSIER scale
(stroke possible if >0)
Non contrast CT head (rule out haemorrhagic stroke)
CT Angiogram (identify occlusion - hypoattenuation of brain parenchyma, loss of matter differentiation)
How do strokes appear on CT
Ischaemic - Darkness of brain parenchyma
Haemorrhagic - Brightness surrounded by darkness (blood surrounded by oedema)
Management of ischaemic stroke
Once haemorrhagic ruled out:
- IV Alteplase if presents within 4.5 hours
- Mechanical thrombectomy if after 4.5 hours
Then: 300mg Oral aspirin daily for 2 weeks then clopidogrel lifelong daily
What are the driving rules in ischaemic stroke
Patients must not drive car for 1 month after TIA or stroke, or 1 year for HGV
What classification system is used in stroke?
Bamford classification - categorises stroke based on area of circulation affected.
Total anterior circulation stroke - Anterior/middle cerebral artery (all 3 - Unilateral weakness of face,arm or leg, homonymous hemianopia, higher cerebral dysfunction)
Partial anterior circulation stroke - only part of anterior circulation (2 out of 3 symptoms)
Lacunar - Either: All sensory, all motor or ataxic hemiparesis
Posterior circulation syndrome (1 of cranial nerve palsy, bilateral motor/sensory defecit, eye movement disorder, homonymous hemianopia)
Scoring system for risk of stroke after Atrial Fibrillation
CHA2DS2 VASc
DEFINE TIA
Transient Ischaemic Attack. Acute neurological dysfunction that has a sudden onset and resolves in less than 24 hours.
NOT a stroke as involves ischaemia not infarction
Symptoms of TIA
Contralateral numbness, face drooping, dysphasia, vision loss
Amaurosis Fugax
Same as stroke but lasts less than 24 hours and no lasting effects
What acronym helps identify stroke in public
FAST
Face
Arms
Speech
Time
Define amaurosis fugax with pathophysiology and causes
Short lived blindness in one eye described as “curtain coming down over vision”. Due to temporary reduction in internal carotid or central retinal artery leading to ischaemia of the retina.
Occurs in GCA, Stroke, AF
What risk score should be completed after TIA
ABCD2 - risk of stroke after TIA
Age >60
BP >140/90
Clinical features (unilateral weakness =2, just speech disturbance =1)
Duration >60mins =2, 10-59mins =1
> 6 predicts stroke, immediate referral
4 requires referral
MX OF TIA
1st line - 300mg Aspirin daily for 2 weeks
- Clopidogrel daily long term
- Atorvastatin
ABCD2 score to assess stroke risk
What is it called when 2 TIAs happen in close proximity
Crescendo TIAs.
Requires urgent referral
What are the layers over the brain called
Meninges of the brain
Dura mater
Arachnoid
Pia mater (innermost layer)
What are the types of haemorrhagic stroke
Extradural haemorrhage - bleeding above dura mater
Sudural haemorrhage - bleeding between dura and arachnoid
Subarachnoid haemorrhage - bleeding between arachnoid and pia mater
Intracerebral haemorrhage - Bleeding within cerebrum
Risk factors for haemorrhagic stroke (6)
Head injury
Hypertension
Aneurysms
Brain tumour
Connective tissue disorder
Family history
General symptoms of haemorrhagic stroke
Reduced GCS
Headache
Vomiting
Seizures
One sided arm/leg/face weakness/paralysis
Give the scoring system for unconsciousness
Glasgow Coma Scale - assessment of eye opening, verbal and motor response.
Minimum score 1 per category
Give the scoring system for consciousness
Glasgow Coma Scale - assessment of eye opening, verbal and motor response.
Eye out of 4
Verbal out of 5
Motor out of 6
Minimum score 1 per category
Glasgow coma scale scoring system in detail (not sure if need to know but probably helpful to have decent idea)
Eye opening
4 - Spontaneous
3 - To speech
2 - To pain
1 - None
Verbal response
5 - Orientated
4 - Confused conversation
3 - Inappropriate words
2 - Incomprehensible sounds
1 - None
Motor response
6 - Obeys command
5 - Localises to pain
4 - Withdraws to pain
3 - Abnormal flexion to pain
2 - Extension of upper and lower limbs to pain
1 - No response
why might someone get a brain abscess?
extension of sepsis from middle ear or sinuses
trauma or surgery to scalp
penetrating head injury
embolic event from endocarditis
presenting sx of brain abscess
depend on site of abscess - if motor cortex will present earlier
mass effect on brain and raised icp
headache: dull persistent
fever: poss absent. not swinging pyrexia though
focal neurology: oculomotor nerve palsy or abducens nerve palsy secondary to raised icp
nausea
papilloedema
seizures
how would you investigate for brain abscess
ct scanning
how would you manage for brain abscess
surgery - craniotomy - debride abscess cavity.
abscess can reform because head is closed after abscess drainage
iv abx: 3rd gen cephalosporin+ metronidazole
icp manage: dexamethasone
features of encephalitis
fever headache psychiatric sx , seizures, vomiting
focal features: aphasia
altered cognition/conciousness. unusual behaviour.
peripheral lesions: like cold sores no relationship to presence of HSV encephalitis
pathophysiology of encephalitis
hsv-1 - 95% adult cases
affects temporal and inferior frontal lobes
ix encephalitis
csf - lymphocytosis, elevated protein
pcr: HSV,VSV,ENTEROVIRUSES
neuroimaging:
CT - medial temporal and inferior frontal changes - eg petechial haemorrhages
normal in 1/3 of patients
MRI is better
hiv test
swabs - causative organism, throat and vesicle swab
EEG: lateralised periodic discharges at 2Hz
mx encephalitis
intravenous aciclovir start in all suspected encephalitis cases - hsv and vsv
ganciclovir - cmv
comps of encephalitis
lasting fatigue/prolonged recovery
headache, chronic pain, learning disability, change to memory, personality, mood , cognition.
movement disorder
sensory disturbance
siezure
hormonal imbalance
ci of lp in encephalitis in kids
gcs below 9
haemodynamiccaly unstable
active seizures
post-ictal
causes of encephalitis in kids
non infective: autoimmune
mc : viral
bacterial and fungal - rare
mc: hsv. hsv-1 from cold sores.
in neonates: hsv-2 from genital herpes from birth.
vzv: chicken pox,cmv associated with immunodeficiency.
ebv: infectious monucleosis, adenovirus, influenza virus.
polio, mumps, rubella, measles - all can cause it too.
hsv encephalitis - typically affects with lobes
temporal
what is shingles herpes zoster?
acute unilateral painful blistering rash
caused by reactivation of varicella-zoster virus - vzv.
(following vzv infection, chickenpox)
virus lies dormant in dorsal root or cranial nerve ganglia.
risk factors of shingles
increasing age
hiv: strong rf 15* more common
other immunosuppressive conoditions: steroids, chemo
most common affected dermatomes in shingles
t1-l2
features of shingles
prodromal period:
- burning pain over affected dermatome for 2-3 days.
-pain might be severe and sleep
- 20% pts experience fever, headache, lethargy
rash
- erythematous, macular rash over affected dermatome
-quickly becomes vesicular
- characteristically well demarcated by dermatome dont cross midline. “ bleeding” in adjacent areas might be seen.
clinical diagnosis
how would you manage shingles - herpes zoster
avoid pregnant women and immunosuppresed - contagious
infectious until vesicles crusted over, 5-7 days after onset. covering lesions reduces risk.
analgesia:
- 1st line para and nsaids
- if not : neuropathic agent like amitriptyline
- oral corticosteroids - in 1st 2 weeks in immunocompetent adults with localised shingles if pain severe.
antivirals
- within 72 hrs for most patients, unless pt under 50 and mild truncal rash with mild pain
- aciclovir , famciclovir, valaciclovir
benefit of prescribing antiviral in shingles - herpes zoster
reduced incidence of post-herpetic neuralgia
complications of shingles -herpes zoster
postherpetic neuralgia
herpes zoster opthalmicus - ocular division of trigeminal nerve
herpes zoster oticus - ear lesions and facial paralysis
malaria is caused by?
plasmodium protozoa spread by female anopheles mosquito:
4 diff :
plasmodium falciparum - most.
plasmodium vivax - 2nd - benign malaria
plasmodium ovale
plasmodium malariae
malaria protects from what diseases?
sickle-cell trait
g6pd deficiency
hla b53
absence of duffy antigens
falciparum malaria - mc - most severe type: features - classic triad
why do they occur every 48 hrs?
paroxysms of fever chills and sweating.
every 48 hrs because erythrocytic cycle of plasmodium falciparum parasite.
fever high intermittent. - possible rigors too.
non specific:
malaise,headache,myalgia
general features of falciparum malaria
fever - cyclical - sweating and sometimes rigors.
gi:
-anorexia,n+v, abdo pain. diarhoea poss , mc in kids. poss mild jaundice and occasional pruritus
resp: cough, poss mild tachypnoea
msk: generalised body aches and joint pain
neuro: headache. dizziness and sleep disturbance.
cv: tachy, hypotension more typical of severe malaria.
haem: thrombocytopenia most significant haematological finding. mild anaemia poss
renal: aki associated with severe malaria. non severe : mild-moderate increase in creatinine or blood urea nitrogen levels
features of severe falciparum malaria
how to treat
schizonts on blood film
parasitaemia >2%
hypogly
acidosis
temp over 39
severe anaemia
iv artesunate
if parasite count over 10% then exchange tranfusion
shock might show bacterial septicaemia
comps of malaria falciparum
cerebral malaria: seizures, coma
acute renal failure: blackwater fever, secondary to intravascular haemolysis
ards
hypogly
dic
how would you manage falciparum malaria?
uncomplicated falciparum:
1st line: artemisinin-based combo therapy (acts) - artemether+ lumefantrine, artesunat+ amodiaquine, artesunate+ mefloquine, artesunate + sulfadoxine-pyrimethamine, dihydroartemisinin + piperaquine
mc non falciparum malaria
vivax - central america and indian subcontinent.
then ovale (africa) and malariae.
knowlesi - south east asia
features of non falciparum malaria
fever headache splenomegaly
vivax/ovale: cyclical fever every 48 hrs.
malariae: cyclical fever every 72 hours. associated with nephrotic syndrome.
ovale and vivax: hypozoite stage : relapse after tx.
how would you treat non falciparum malaria?
artemisinin-based combo therapy (act) or chloroquine
if chloroquine resistant.
act avoid in pregnancy.
ovale/vivax: primaquine after acute tx with chloroquine to destroy liver hypnozoites and prevent relapse
incubation period of malaria
1-4 weeks after exposure.
vivax and ovale can lie dormant upto 4 yrs
how do you diagnose malaria
blood film.
edta bottle.
3 negative samples over 3 consecutive days needed to exclude.
side effect of primaquine
can cause severe haemolysis in g6pd pts
doxycline - use in malaria when to give etc
side effedcts
broad spect abx
se: diarrhoea , thrush. skin sensitivity to sun
take 2 days before until 4 weeks after endemic area travel.
antimalarial meds
proguanil with atovaquone
doxycycline
mefloquine - psych side effects. - anxiety,dep,abnormal dreams
chloroquine with proguanil
general advice preventing malaria
mosquito spray 50% DEET spray
nets and barriers
antimalarial meds
metastatic brain cancer can spread from?
lung - MC
breast
bowel
skin - namely melanoma
kidney
features of brain tumour
poss asx
progressive focal neurological symptoms
raised icp
features of raised icp
headache contant worse at night or on waking or coughing/straining.
vomiting
papilloedema on fundoscopy (paton lines)
altered mental state
ptosis unilateral
visual field defect
3rd and 6th nerve palsy
with papilloedema what do u see on fundoscopy?
blurring of optic disc margin
engorged retinal veins
haemorrhages around optic disc
paton lines - creases in retina around optic disc
loss of venous pulsation
elevated optic disc
what is a glioma?
tumour of glial cells in brain/spinal cord.
support/surround neurones.
include: astrocytes,oligodendrocytes, ependymal cells.
grade 1-4.
4 (glioblastoma multiforme)
3 types of glioma
astrocytoma - mc and aggressive is glioblastoma
oligodendroglioma
ependymoma
gliobastoma multiforme
prognosis
imaging
histology
tx
solid tumour with central necrosis and a rim that enhances contrast.
pleomorphic tumour cells border necrotic areas
surgery and postop chemo and/or radio.
dex: oedema
what is a meningioma?
histology
ix
tx
tumour of meninges.
benign
mass effect. = raised icp
arise from arachnoid cap and typically located next to dura.
spindle cells in concentric whirls and calcified psammoma bodies.
CT: contrast enhancement
MRI
tx: RADIO/ SURGERY
tell me about pituitary tumours.
press on what?
sx?
mx
benign
optic chiasm press - bitemporal hemianopia
cause hormone def or release excessive hormones lead to:
- acromegaly
-hyperprolactinaemia
- cushings disease
- thyroxicosis
transphenoidal surgery
radio
bromocriptine - block excess prolactin
somatostatin analogue - block excess GH
symptoms of cerebellar disease
D - dysdiadokinesia, dysmetria, “drunk”
A - ataxia - limb,truncal
N - nystagmus - horizantal = ipsilateral hemisphere
I - intention tremor
S - slurred staccato speech, scanning dysarthria
H - hypotonia
unilateral cerebellar lesions cause what signs?
ipsilateral
causes of cerebellar disease
friedreichs ataxia
neoplastic: cerebellar haemangioma
stroke
alcohol
ms
hypothyroid
drugS: phenytoin, lead poisoning
paraneoplastic: 2 to lung cancer
What is normal pressure hydrocephalus?
reversible cause of dementia in elderly.
2 to reduced csf absorption at arachnoid villi.
could be due to head injury, subarachnoid haemorrhage or meningitis
classic triad of normal pressure hydrocephalus
urinary incontinence
dementia and bradyphrenia
gait abnormality
sx develop over a few months
imaging for normal pressure hydrocephalus
hydrocephalus with ventriculomegaly in absence of ,or out of proportion to , sulcal enlargement
how would you manage normal pressure hydrocephalus
comps of this tx
ventriculoperitoneal shunting
seizures
infection
intracerebral haemorrhage
What is Cerebral Palsy?
non-progressive lesion motor pathways in developing brain.
disorder of movement and posture.
causes of cerebral palsy
antenatal
intrapartum
postnatal
antenatal 80%
- cerebral malformation
-congenital infection (rubella, toxoplasmosis, CMV)
intrapartum 10% =
- birth asphyxia/trauma
-preterm birth
postnatal 10%
- intraventricular haemorrhage
-meningitis
-head trauma
classifying cerebral palsy
4 different types
spastic 80%
- hemiplegia, diplegia or quadriplegia
-increased tone (hypertonia) and reduced function resulting from damage to UMN
dyskinetic:
- damage to basal ganglia and substantia nigra
-athetoid movements and oro-motor problems . muscle tone problems (hypertonia/hypotonia) causing the athetoid etc.
ataxic:
- caused by damage to the cerebellum with typical cerebellar signs
- problems with coordinated movement due to above.
mixed: mixed of spastic,dyskinetic and/or ataxic features.
patterns of spastic cerebral palsy
monoplegia: 1 limb affected
hemiplegia: one side of body affected
diplegia: 4 limbs affected by mostly legs
quadriplegia: neck down all 4 limbs - with seizures, speech disturbance and other impairements
what non-motor problems might a cerebral palsy patient have?
learning difficulties - 60%
epilepsy 30
squints 30%
hearing impairement 20
How would you manage cerebral palsy?
tx spasticity:
-oral diazepam
-oral and intrathecal baclofen (muscle relaxant), botulinum toxin type A , orthopaedic surgery and selective dorsal rhizotomy.
anticonvulsants, analgesia prn
physio - stretch and strength muscles prevent muscle contractures.
OT
SLT
DIETICIAN - peg feeding poss?
ORTHO SURGEON - release contractures or length tendons (tenotomy)
glycopyrronium bromide: excessive drooling.
anti-epileptic - seizures
what condition makes children at risk of developing cerebral palsy?
hypoxic-ischaemic encephalopathy
signs and symptoms of cerebral palsy
failure to meet milestones
learning difficulty
feeding or swallowing problems
problems with coordination, speech or walking
increased/decreased tone, generally or in specific limbs
hand preference below 18 months .
based on neuro exam what to each of these indicate:
hemi/di plegic gait
broad based gait/ataxic gait
high stepping gait
waddling gait
antalgic gait
UMN lesion
cerebellar lesion
foot drop/lmn lesion
pelvic muscle weakness due to myopathy
indicates localised pain
with a UMN lesion tell me about:
inspection
tone
power
reflexes
muscle bulk preserved
hypertonia
slightly reduced
brisk
with a lmn tell me about:
inspection
tone
power
reflex
reduced muscle bulk with fasciculations
hypotonia
dramatically reduced
reduced
complications and associated conditions of cerebral palsy
learning disability
epilepsy
kyphoscoliosis
muscle contractures
hearing and visual impairment
gastro-oesophageal reflux
what is hypoxic ischaemic encephalopathy?
neonates.
hypoxia during birth.
causes of HIE
anything that leads to asphyxia (deprivation of oxygen) to the 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
grading HIE
mild
moderate
severe
sarnat Staging
mild:
- poor feeding, generally irritability and hyper-alert
-resolves within 24 hours
-normal prognosis
moderate:
- poor feeding, lethargic, hypotonic, seizures
-can take weeks to resolve
-up to 40% develop cerebral palsy
severe:
- reduced gcs, apnoeas, flaccid and redcued or absent reflexes
-upto 50% mortality
-upto 90% develop cerebral palsy
when to suspect HIE
neonate
acidosis (ph under 7) on umbilical abg
poor Apgar score
features of mild,mod,severe HIE
evidence of multi organ failure.
how would you manage HIE
supportive care with neonatal resus
optimal ventilation, circulatory support, nutrition, acid base balance and seizure tx.
therapeutic hypothermia - protect brain from hypoxic injury.
what is horners syndrome?
combo of sx that happen when group of nerves known as sympathetic trunk is damaged.
eye and surrounding area on 1 side of face.
features of horners syndrome
miosis - small pupil
ptosis
enophthalmos - sunken eye
anhidrosis - loss of sweating one side
using anhidrosis to decipher where the lesion is for horners syndrome
face arm and trunk: central lesion
face: pre-ganglionic
no anhidrosis: post-ganglionic
central lesion causes of horners
pre ganglionic
post ganglionic
stroke, syringomyelia, ms, tumour, encephalitis
pancoasts tumour , thryoidectomy, trauma, cervical rib
carotid artery dissection, carotid anerusym, cavernous sinus thrombosis, cluster headache
heterochronic - difference in iris colour is seen in what?
congenital horners
horners - what are apraclonidine drops
alpha adrenergic agonist
cause pupillary dilation
produces mild pupillary construction in normal pupil.
What is epilepsy?
types
seizure tendency.
transient episodes of abnormal electrical activity in brain.
generalised tonic-clonic
focal seizures
absence
atonic
myoclonic
infantile spasms
febrile convulsions
What are febrile convulsions?
age of onset
mc type of seizure here
clinical features
seizures provoked by fever in otherwise normal kids.
6 months and 5 years.
tonic-clonic
usually occur early in viral infection as temp rises rapidly
seizures brief: last less than 5 mins
types of febrile convulsion
simple
complex
febrile status epilepticus
simple:
- less than 15 mins
-generalised seizure
-typically no recurrence within 24 hours
-should be complete recovery within an hr
complex:
- 15-30 mins
-focal seizure
-may have repeat seizures within 24 hours
febrile status epilepticus :
- over 30 mins
Mx of Febrile Convulsion
following a seizure
ongoing
admit if first or features of complex seizure
phone ambulance if seizure over 5 mins.
benzodiazepine rescue med: rectal diazepam or bvuccal midazolam
regular antipyretics: dont reduce chance of them occuring
prognosis of febrile convulsions
risk of another one is 1 in 3. depends on:
age of onset under 18months
fever under 39
shorter duration of fever before seizure
fhx of them
rf for developing epilepsy post febrile convulsions
fhx of epilepsy
complex febrile seizures.
background of neurodevelopmental disorder
if no rf@ 2.5% risk of developing it.
if kids have all 3 features 50% risk
what is benign rolandic epilepsy?
how to ix and what does it show
prognosis
features
form of childhood epilepsy occurs between 4 and 12.
EEG - centrotemporal spikes
excellent prognosis - seizures stop by adolescence
seizures at night.
partial seizures (paresthesia affect face) but secondary generalisation may occur (parents may only report tonic-clonic movements)
Using Contraception in epilepsy what factors are to be considered?
effect of contraceptive on effectiveness of anti-epileptic
effect of anti-epileptic on effectiveness of contraceptive
potential teratogenic effects of anti-epileptic if women becomes pregnant
if women is taking phenytoin carbamazepine barbiturates primidone topiramate oxcarbazepine what to do?
for contraception in epileptic pt
ukmec 3 : the COCP and POP
ukmec 2 : implant
ukmec 1 : depo-provera, IUD,IUS
use condom
if COCP : min of 30 ug of ethinylestradiol
for lamotrigine if women takes what to do?
contraceptionin epileptic pt
use condom
ukmec 3 : cocp
ukmec 1: pop, implant, depo-provera, Iud, ius
if COCP: min or 30 ug of ethinylestradiol
what conditions are associated with epilepsy?
cerebral palsy: 30% have it
tuberous sclerosis
mitochondrial diseases
what is an alcohol withdrawal seizure?
mechanism
tx
pt hx of alcohol excess suddenly stopped drinking.
36 hours after drink cessation.
give benzodiazepine after stopping.
chronic alcohol enhances gaba mediated inhibition in cns and inhibits nmda-type glutamate receptors.
alcohol withdrawal dose the opposite
what are psychogenic non-epileptic seizures?
pseudoseizures
epileptic-like seizures but no electrical discharges.
hx of mental health or personality disorder
define a focal seizure
partial seizure.- start in temporal lobe.
start in a specific area , one 1 side of brain.
varied awareness.
30 yr old women told by her husband she wakes at night, grunting sound, lip smacking, non responsive during ep. after minute falls asleep again.
focal impaired awareness - explained above.
classified into
motor: jacksonian march
non-motor : deja vu, jamais vu
or other features like aura
sx: affect hearing speech memory and emotions.
hallucinations
-memory flashbacks
-deja vu
doing strange things on autopilot
generalised seizure define
involve networks on both sides of the brain at onset.
lose conciousness immediately.
motor: tonic clonic
non motor: absence
specific types:
-tonic clonic - grand mal
- tonic
-clonic
-typical absence - petit mal
- myoclonic: brief,rapid muscle jerks
- atonic
unknown onset seizure - what is it?
unknown origin of seizure
focal to bilateral seizure - what is it
starts on 1 side of brain in specific area before spreading to both lobes
previously called secondary generalised seizure
tell me about typical absence seizures: petit mal
onset
duration
eeg
tx
prognosis
onset: 4-8 years
duration : few-30 seconds. no warning, quick recovery: often many per day
eeg: 3Hz, generalised, symmetrical
sodium valproate, ethosuximide
good prognosis: 90-95% become seizure free in adolescence
if a focal seizure happens starts in occipital lobe (visual) what happens?
floaters/flashes
if a focal seizure happens in parietal lobe what happens ? (sensory)
paresthesia
if a focal seizure happens in frontal lobe? (motor)
head/leg movements, posturing
post-ictal weakness (todd’s paresis),
jacksonian march (clonic movements travelling proximally)
if a focal seizure starts in temporal lobe what can i expect?
last around 1 min - automatisms (lip smacking/grabbing/plucking) common
with/without impairement of conciousness or awareness
aura in most:
- rising epigastric sensation
-psychic or experiental phenomena like deja vu , jamais vu
-less common: hallucination (auditory,gustatory,olfactory)
sodium valproate is associated with what issue for maternal kids?
neural tube defects
risk of neurodevelopmental delay in kids after maternal use of it.
DONT USE IN PREGNANCY AND WOMEN OF CHILDBEARING AGE UNLESS NECESSARY.
which of the epileptic drugs is seen as least teratogenic or older antiepileptics?
carbamazepine
phenytoin is associated with what issue?
antiepileptic
what to give with it. this is to prevent what?
cleft palate
vit k
last month of pregnancy
prevent clotting disorders in newborn
is breast feeding safe for mothers taking anti-epileptics?
is there an exception to the rule?
yes
NO BARBITURATES
women want to get pregnant epileptic what should they take and why?
folic acid 5mg per day well before pregnancy
minimise risk of neural tube defects
briefly explain what an absence seizure is?
tx
in kids.
pt becomes blank stares into space and abruptly returns to normal.
unaware of surroundings, wont respond.
10-20 seconds.
stop when they get older
1st line: ethosuximide
2nd line:
m - sodium valproate
f: lamotrigine or levetiracetam
carbamazepine - exacerbate absence seizures
how would you tx focal seizures ?
1st line : lamotrigine or levetiracetam
2nd line: carbamazepine, oxcarbazepine or zonisamide
the rules are you start antiepileptics after second seizure.
in what case would you start after first?
pt has neuro deficit
brain imaging shows structural abnormality
EEG shows unequivocal epileptic activity.
pt or their fam or carer consider the risk of having a further seizure unacceptable
generalised tonic clonic seizure tx?
male: sodium valproate
female: lamotrigine or levetiracetam
under 10, unlikely to need tx when old enough to have kids or women unable to have children can be given sodium valproate first line.
how to atonic seizures present?
tx?
drop attacks.
brief lapse in muscle tone.
no more than 3 minutes.
childhood begin.
indicative of lennox-gastaut syndrome.
mx:
- m: sodium valproate
- f: lamotrigine
how do myoclonic seizures present?
tx?
sudden brief muscle contractions - sudden jump.
awake during.
happen in kids as part of juvenile myoclonic epilepsy.
tx:
- m - sodium valproate
- f : levetiracetam
rescue medication for epilepsy if they dont terminate spontaneously ie after 5-10 mins
benzodiazepines like diazepam
administer rectally or intranasally/under tongue.
if still fits: status epilepticus. - medical emergency - hospital tx required.
mx: further benzodiazepine med, infusion of antiepileptic or general anesthetic.
ix for epilepsy
good history
eeg: typical patterns of it. after the second simple tonic clonic do.
MRI brain: structure of brain.
ecg - exclude heart problems
blood electrolytes - sodium, potassium, calcium , magnesium
bg: hypoglycaemia and diabetes
blood cultures, urine cultures and lp where sepsis, encephalitis or meningitis is suspected
general advice for epileptic patients
take showers not baths
cautious with heights, traffic, heavy/hot/electric equipment
be very catious with swimming unless seizures well controlled and they are closely supervised.
mx of seizures physically
pt in safe position - carpeted floor
recovery position if poss
something soft under head
remove obstacles that could lead to injury
note the time at start and end
call ambulance if more than 5 mins or 1st seizure.
what is status epilepticus?
mx:
medical options in community ?
med emergency
seizure lasting more than 5 mins or 2 or more seizures without regaining consciousness in interim.
ABCDE approach
secure airway
high conc ox
assess cardiac and resp function
check bg levels
iv access - insert cannula
iv lorazepam - repeated after 10 mins if the seizure continues.
if persist final step is iv phenobarbital or phenytoin. intubation and ventilation to secure airway- transfer to icu.
med options in community:
- buccal midazolam
- rectal diazepam
