Neurology Flashcards
Describe the management of epilepsy
Most neurologists now start antiepileptics following a second epileptic seizure.
NICE guidelines suggest starting antiepileptics after the first seizure if:
> neurological deficit
> brain imaging: structural abnormality
> EEG: unequivocal epileptic activity
> patient / family / carers seizure risk unacceptable
Drug treatment
- Generalised tonic-clonic seizures
> males: sodium valproate
> females: lamotrigine or levetiracetam
» sodium valproate if under 10 or women unable to have children
» sodium valproate causes weight gain - Focal seizures
> first line: lamotrigine or levetiracetam
> second line: carbamazepine, oxcarbazepine or zonisamide - Absence seizures (Petit mal)
> first line: ethosuximide
> second line:
male: sodium valproate
female: lamotrigine or levetiracetam
carbamazepine may exacerbate absence seizures - Myoclonic seizures
> males: sodium valproate
> females: levetiracetam - Tonic or atonic seizures
> males: sodium valproate
> females: lamotrigine
describe autonomic dysreflexia
caused by excessive sympathetic response below level of injury without coordinated parasympathetic counter response
can only occur if spinal cord injury is above T6
features
- severe hypertension
- flushing and sweating above level of injury
- reflex bradycardia
causes
- urinary retention / catheter blockage
- faecal impaction / loading
complications: stroke
describe Guillain-Barré syndrome
immune-mediated demyelination of peripheral nervous system usually post-diarrhoeal illness e.g. Campylobacter jejuni
clinical features
- back/leg pain
- progressive symmetrical ascending weakness (starting in legs)
- reflexes are reduced or absent
- sensory symptoms usually mild
other features
- respiratory muscle weakness
- cranial nerve involvement: diplopia, bilateral facial nerve palsy, oropharyngeal weakness
- autonomic involvement: urinary retention, diarrhoea
investigations
- LP: rise in CSF protein with normal WCC
> gold-standard investigation
- anti-ganglioside antibodies
- Nerve conduction studies: decreased motor nerve conduction velocity
treatment - plasma exchange, IV immunoglobulins
Which medications can be given for the following problems in MS?
- fatigue
- spasticity
- oscillopsia
- bladder dysfunction
fatigue: amantadine
spasticity: baclofen, gabapentin
oscillopsia: gabapentin
bladder dysfunction: anticholinergics
which medications can be given to reduce the risk of relapse in MS?
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)
medications
- natalizumab: first-line
- ocrelizumab: another first-line option
- fingolimod
- beta-interferon: less effective
- glatiramer acetate: less effective
describe hereditary sensorimotor neuropathy (HSMN) aka Charcot-Marie-Tooth
encompasses Charcot-Marie-Tooth disease (aka peroneal muscular atrophy)
2 common types in clinical practice
- type I: demyelinating
- type II: axonal pathology
HSMN type I
- Autosomal dominant
- due to defect in myelin-coding gene
- features start at puberty
- motor symptoms predominate
- distal muscle wasting, pes cavus, clawed toes
- foot drop and leg weakness often first features
when can anti-epileptic drugs (AED) be stopped?
can be considered if seizure free for >2 years with AEDs being stopped over 2-3 months
describe localising features of focal seizures
temporal lobe
- with/without impairment of consciousness or awareness
- aura
> rising epigastric sensation
> psychic/experiential phenomena e.g. deja vu, jamais vu - less commonly hallucinations (auditory/gustatory/olfactory)
- seizures typically last around one minute
- automatisms (lip smacking/grabbing/plucking of clothes)
- post-ictal dysphasia
- Todd’s paralysis
frontal lobe (motor)
- head/leg movements
- posturing
- post-ictal weakness
- Jacksonian march
parietal lobe (sensory)
- paraesthesia
occipital lobe (visual)
- floaters/flashes
describe subacute combined degeneration of the cord
SCD: spinocerebellar tract, corticospinal tract, dorsal columns
causes
- vitamin B12 & E deficiency
- can result from replacing folate before b12 if b12 deficient (BeFore)
- nitrous oxide inhalation
features
- bilateral spastic paresis: weakness, spasticity
- bilateral loss of proprioception and vibration
- distal tingling, burning and/or sensory loss
- bilateral limb ataxia
- hyperreflexia
- positive Babinski sign
positive Romberg’s test due to sensory ataxia
list driving restrictions for individuals with epilepsy
first unprovoked/isolated seizure: 6 months off if no structural brain abnormalities on imaging or epileptiform activity on EEG
established epilepsy / multiple unprovoked seizures:
> qualify for driving license if they have been seizure free for 12 months
> if no seizures for 5 years, a til 70 license is restored
withdrawal of epilepsy medication: cannot drive whilst being withdrawn and for 6 months after
describe spinal muscular atrophy (SMA)
rare autosomal recessive condition which causes a progressive loss of lower motor neurones leading to progressive muscular weakness
features
- fasciculations
- reduced muscle bulk
- reduced tone
- reduced power
- reduced / absent reflexes
management: supportive
describe myotonic dystrophy
genetic disorder usually presenting in adulthood
features
- progressive muscle weakness
- prolonged muscle contractions
- cataracts
- cardiac arrhythmias
describe the features, investigations and management of a TIA
brief period of neurological deficit due to a vascular cause, typically lasting less than an hour (always <24h)
Clinical features
- unilateral weakness or sensory loss
- aphasia or dysarthria
- ataxia, vertigo, or loss of balance
- visual problems
- sudden transient loss of vision in one eye (amaurosis fugax)
- diplopia
- homonymous hemianopia
ABCD2: prognostic score to risk stratify patients
Management
- immediate antithrombotic therapy: > aspirin 300 mg immediately
unless:
1. bleeding disorder / anticoagulated (needs immediate admission for imaging to exclude a haemorrhage)
2. already taking low-dose aspirin regularly: continue the current dose of aspirin until reviewed by a specialist
3. Aspirin is contraindicated: discuss management urgently with the specialist team
Advise the person not to drive until they have been seen by a specialist
Investigations
- diffusion-weighted MRI first-line
- Carotid imaging
> all patients should have an urgent carotid doppler unless they are not a candidate for carotid endarterectomy
Further management
> Secondary prevention
- clopidogrel is recommended first-line (as for patients who’ve had a stroke)
- aspirin + dipyridamole should be given to patients who cannot tolerate clopidogrel
- lipid modification
- high-intensity statin (such as atorvastatin 20–80 mg daily)
carotid artery endarterectomy if carotid stenosis > 70%
Describe the symptoms of stroke depending on the affected vessel
ACA
> contralateral hemiparesis and sensory loss
> lower extremity > upper
MCA
> contralateral hemiparesis and sensory loss
> upper extremity > lower extremity
> aphasia
> contralateral homonymous hemianopia
PCA
> Contralateral homonymous hemianopia with macular sparing
> Visual agnosia
Weber’s syndrome (branches of PCA supplying midbrain)
> contralateral upper and lower limb weakness
> ipsilateral CN III palsy
PICA (lateral medullary syndrome, Wallenberg syndrome)
> ipsilateral facial pain and temperature loss
> contralateral limb/torso pain and temperature loss
> ataxia, nystagmus
> ipsilateral Horner’s syndrome
AICA (lateral pontine syndrome)
> similar to Wallenberg’s but ipsilateral facial paralysis and deafness also
> reduced GCS, paralysis, bilateral pinpoint pupils
Retinal/ophthalmic artery: amaurosis fugax
Basilar artery: locked in syndrome
Lacunar strokes present with either isolated hemiparesis, hemisensory loss or hemiparesis with limb ataxia
> strong association with hypertension
> common sites include the basal ganglia, thalamus and internal capsule
Describe bacterial meningitis and its management
Clinical features
- meningism: neck stiffness, photophobia
- nausea / vomiting
- reduced GCS
- non-blanching rash
- fever
- tachycardia, tachypnoea, hypotension
- Brudzinski / kernig’s sign +ve
attempt LP unless
- severe sepsis / rapidly evolving rash
- severe respiratory / cardiac compromise
- significant bleeding risk
- signs of raised ICP (focal neurological signs, papilloedema, continuous/uncontrolled seizures)
CT head prior to LP ONLY if risk of evolving SOL / signs of increased ICP
Management
- IV antibiotics
> <3 months: IV cefotaxime + amoxicillin
> 3 months-50 years: cefotaxime or ceftriaxone
> >50 years: cefotaxime (or ceftriaxone) + amoxicillin (increased risk of Listeria meningitis)
- IV dexamethasone 0.15mg/kg QDS for 4 days
> avoid in septic shock, meningococcal septicaemia, immunocompromised or meningitis following surgery - if allergic to penicillin / cephalosporins, use chloramphenicol
- if Listeria monocytogenes: IV amoxicillin + gentamicin
Describe a post-LP headache and its management
Low pressure headache
can occur up to a week later from when the LP was taken
alleviated by lying down
> if headache lasts more than 3 days, consider subdural haematoma
management
- supportive analgesia - analgesia, rest
- consider blood patch, epidural saline and IV caffeine
Describe the diagnostic criteria for migraine
- headache attacks lasting 4-72h
- at least 2 of the following characteristics
> unilateral location but may be bilateral
> pulsating quality
> moderate or severe pain
> aggravation by routine physical activity - during headache at least one of the following
> nausea or vomiting
> photophobia, phonophobia - not attributed to another disorder
in children, attacks may be shorter-lasting, headache is bilateral and GI disturbance is more prominent
migraine with aura
> aura is progressive in nature and occurs hours prior to headache
> typical aura: transient hemianopic disturbance / spreading scintillating scotoma (jagged crescent)
> sensory symptom
auras may occur with/without headache and are fully reversible, develop over at least 5 minutes and last 5-60 minutes
atypical symptoms
> motor weakness
> double vision
> visual symptoms affecting only one eye
> poor balance / decreased GCS
Name the most common cause of
- bacterial meningitis <60
- viral meningitis
- viral encephalitis
bacterial meningitis <60: Neisseria meningitidis, streptococcus pneumoniae
Viral meningitis: enterovirus
> Coxsackie, Echovirus
Viral encephalitis: HSV
Describe viral meningitis and its management
Presents similarly to bacterial meningitis
> less likely to have a non-blanching purpuric rash
> less severe than bacterial meningitis
management
- supportive, usually self-limiting course of 7-14 days
- aciclovir if thought to be caused by HSV
- IV aciclovir if concern regarding encephalitis
in the context of meningitis, under which circumstances should LP be delayed?
- signs of severe sepsis
- rapidly evolving rash
- severe respiratory / cardiac compromise
- significant bleeding risk
- signs of increased ICP
> focal neurology
> GCS <=12
> Papilloedema
> seizures
describe CSF results depending on the cause of meningitis
bacterial
- appearance: cloudy
- glucose: low (<1/2 plasma)
- protein: high (>1g/l)
- WCC: 10-5000 polymorphs/mm3
viral
- appearance: clear/cloudy
- glucose: 60-80% of plasma glucose
- protein: normal/raised
- WCC: 15-1000 lymphocytes/mm3
TB
- appearance: slight cloudy, fibrin web
- glucose: low (<1/2 plasma)
- protein: high (>1 g/l)
- WCC: 30-300 lymphocytes/mm3
fungal
- appearance: cloudy
- glucose: low
- protein: high
- WCC: 20-200 lymphocytes/mm3
describe the management of status epilepticus
- ABCDE assessment
- airway: lateral decubitus, nasal trumpets, O2, suction
- IV access
0-5 mins (give benzos at 5 mins)
> lorazepam 0.1mg/kg IV, max 4mg, repeat once in 4min
> OR midazolam 10mg IM once
10-15 mins
> levetiracetam 60mg/kg IV
> OR fosphenytoin or phenytoin 20mg/kg IV
> OR valproate 40mg/kg IV
> advanced airway management
> RSI
> Preoxygenation
> Induction: propofol or ketofol
> paralytics: rocuronium, succinylcholine
15-20 mins: refractory medications
- propofol
- midazolam
- ketamine
- lacosamide
- phenobarbital
describe herpes simplex encephalitis
infection of brain parenchyma
features
- fever
- headache
- psychiatric symptoms
- seizures
- vomiting
- focal features e.g. aphasia, weakness, limping
investigations
- LP
> elevated protein
> lymphocytosis
> PCR for HSV, VZV, enterovirus
- CT head: temporal lobe changes e.g. asymmetrical low density areas
- EEG
management
- start aciclovir promptly in all suspected cases of viral encephalitis
> treats HSV and VZV
> ganciclovir is needed for CMV
how would you check if fluid is truly CSF?
check a glucose level: bedside test
beta-2-transferrin level: gold standard
describe the different types of aphasia
Wernicke’s (receptive) aphasia
> lesion of superior temporal gyrus
> usually supplied by inferior division of left MCA
> features
- sentences that make no sense
- word substitution
- neologism
- speech remains fluent
- “word salad”
- comprehension is impaired
Broca’s (expressive) aphasia
> lesion of inferior frontal gyrus
> usually supplied by superior division of left MCA
> speech is non-fluent, laboured and halting
> repetition is impaired
> comprehension is normal
Conduction aphasia
> usually due to stroke affecting arcuate fasciculus
> speech is fluent but repetition is poor
> aware of errors being made
> comprehension is normal
Global aphasia
> severe expressive and receptive aphasia
> may still be able to communicate using gestures
describe chronic fatigue syndrome
aka myalgic encephalomyelitis (ME)
diagnosed after at least 3 months of disabling fatigue affecting mental and physical function >50% of the time in the absence of other disease which can explain symptoms
features
- fatigue
- sleep problems
- muscle/joint pains
- headaches
- painful lymph nodes
- cognitive dysfunction
- physical or mental exertion makes symptoms worse
management
- energy management
- CBT
What is the mechanism of controlled hyperventilation in raised ICP?
Reduced blood carbon dioxide to induce cerebral vasoconstriction
describe the Barthel index
measure of disability or dependence in activities of daily living in stroke patients
describe neurogenic shock
causes
- trauma e.g. spinal cord transection
features
- bradycardia
- hypotension
management
- vasopressors
Describe the management of migraines
Acute:
- analgesia
NSAIDs or paracetamol + triptans
> avoid opioids - antiemetic: metoclopramide
> prokinetic agent that helps to relieve gastric stasis that occurs during acute migraine attack
> consider even in cases without nausea
Prophylaxis:
- topiramate (teratogenic) or propranolol
What are the antibiotics of choice for contacts of patients with meningococcal meningitis
PO ciprofloxacin or rifampicin
> one dose for close contacts within last 7 days
Describe idiopathic intracranial hypertension
classically seen in young overweight females
features
- headache
- blurred vision
- papilloedema
- enlarged blind spot
- sixth nerve palsy
- high pressure features: worse on coughing/sneezing/lying flat/leaning forwards
associated with tetracycline antibiotics
investigations
- lumbar puncture: opening pressure values >25 cmH2O
- CT brain: enlarged arachnoid outpouchings, prominent perivascular spaces
management
- weight loss - semaglutide and topiramate
- carbonic anhydrase inhibitors e.g. acetazolamide
- repeated LP - temporary measure
- surgery:
> optic nerve sheath decompression and fenestration
> lumboperitoneal / ventriculoperitoneal shunt
Describe an Arnold-Chiari malformation
downward displacement or herniation of cerebellar tonsils through foramen magnum
malformation may be congenital or acquired through trauma
features
- non-communicating hydrocephalus may occur as a result of obstruction of CSF flow
- headache
- syringomyelia
describe essential tremor
autosomal dominant condition which usually affects both upper limbs
Features
- postural tremor: worse if arms outstretched
- improved by alcohol and rest
- most common cause of titubation (head tremor)
Management
- propranolol is first-line
- primidone is sometimes used
describe a myasthenic crisis and its management
myasthenic crisis
- acute respiratory failure characterised by FVC <1L
- accessory muscle use
Management
> plasmapheresis
> intravenous immunoglobulins
> ventilatory support: low threshold for intubation
Describe myasthenia gravis and its management (as well as exacerbating factors)
autoimmune disorder due to autoantibodies against ACh receptors
Features
- muscle fatiguability - muscles become progressively weaker during periods of activity and slowly improve after periods of rest
- extraocular muscle weakness: diplopia
- proximal muscle weakness: face, neck, limb girdle
- ptosis
- dysphagia
Associations
> thymomas in 15%
> autoimmune disorders: pernicious anaemia, autoimmune thyroid disorders, rheumatoid, SLE
> thymic hyperplasia in 50-70%
Exacerbating factors
> penicillamine
> beta-blockers
> lithium
> phenytoin
> antibiotics: gentamicin, macrolides, quinolones, tetracyclines
> quinidine, procainamide
Investigations
> single fibre electromyography
> CT thorax - exclude thymoma
> antibodies to acetylcholine receptors
> Tensilon test: IV edrophonium reduces muscle weakness temporarily
Management
> long-acting acetylcholinesterase inhibitors: pyridostigmine is first-line
> immunosuppression: prednisolone initially
> azathioprine, cyclosporine, mycophenolate mofetil
> thymectomy
Describe Wernicke’s encephalopathy and its management
Wernicke’s encephalopathy is a neuropsychiatric disorder caused by thiamine deficiency which is most commonly seen in alcoholics
Rarer causes; persistent vomiting, stomach cancer, and dietary deficiency
Classic triad: ophthalmoplegia/nystagmus, ataxia and encephalopathy
Features
> oculomotor dysfunction
> nystagmus (the most common ocular sign)
> ophthalmoplegia: lateral rectus palsy, conjugate gaze palsy
> gait ataxia
> encephalopathy: confusion, disorientation, indifference, and inattentiveness
> peripheral sensory neuropathy
Investigations
> decreased red cell transketolase
> MRI
Treatment: urgent replacement of thiamine
> IV Pabrinex (vitamin B/C)
If not treated Korsakoff’s syndrome may develop
> antero- and retrograde amnesia
> confabulation
Describe cluster headaches and their management
occur in clusters lasting 4-12 weeks
> clusters typically occur once a year
More common in men, smokers and alcohol drinkers
Features
-intense sharp, stabbing pain around one eye
- pain once or twice a day, each episode lasting 15 mins - 2 hours
- restless / agitatated during attack
- clusters typically last 4-12 weeks
- ipsilateral autonomic features
> conjunctival injection (redness)
> orbital oedema
> lacrimation
> miosis and ptosis
> nasal congestion
> facial sweating
Investigations
- neuroimaging - underlying brain lesions are sometimes found
> MRI with gadolinium contrast: investigation of choice
Management
- acute:
> 100% oxygen
> subcutaneous triptan
- prophylaxis: verapamil
list the components of the Glasgow Coma Scale (GCS)
Eye-opening
> eyes open spontaneously (4)
> eyes open to voice (3)
> eyes open to pain (2)
> no eye-opening (1)
Verbal response
> orientated (5)
> confused (4)
> words (3)
> incomprehensible sounds (2)
> no verbal response (1)
Motor resopnse
> obeys commands (6)
> localises pain (5)
> normal flexion (4)
> abnormal flexion (3)
> abnormal extension (2)
> no motor response (1)
describe cerebral venous sinus thrombosis (CVST)
clinical features
- headache (insidious or sudden onset)
- signs of increased ICP
> vomiting
> papilloedema
> focal / generalised seizures
risk factors: thrombophilia, female, pregnancy, COCP, infections (mastoiditis, otitis, sinusitis)
diagnosis: MR venogram
management
- anticoagulation: heparin or warfarin
- treat underlying cause if possible
List contraindications to thrombolysis
Absolute
- Previous intracranial haemorrhage
- Seizure at onset of stroke
- Intracranial neoplasm
- Suspected subarachnoid haemorrhage
- Stroke / traumatic brain injury in preceding 3 months
- LP in preceding 7 days
- GI bleed in preceding 3 weeks
- Active bleeding
- Pregnancy
- Oesophageal varices
- Uncontrolled hypertension
Relative
- Concurrent anticoagulation
- Active diabetic haemorrhagic retinopathy
- Suspected intracardiac thrombus
- Major surgery / trauma in the preceding 2 weeks
Describe the secondary prevention of stroke
clopidogrel 75mg daily lifelong (first-line)
aspirin plus MR dipyridamole (if clopidogrel is contraindicated)
Carotid artery endarterectomy if patient has suffered stroke / TIA in the carotid territory and is not severely disabled
Describe the management of ischaemic stroke
aspirin 300mg orally or rectally - once haemorrhagic stroke has been excluded
> continue aspirin 300mg daily for 2 weeks then clopidogrel 75mg daily long-term
If AF - anticoagulants should not be started until 14 days have passed from the onset of an ischaemic stroke
if cholesterol is > 3.5 mmol/l - commence statin
> delay treatment until after 48 hours due to risk of haemorrhagic transformation
Thrombolysis with alteplase for acute ischaemic stroke
> if within 4.5 hours of onset of stroke symptoms and
haemorrhage definitively excluded
Thrombectomy for acute ischaemic stroke
> within 6 hours of symptom onset, PLUS intravenous thrombolysis (if within 4.5 hours)
> consider extended 6-24h if potential to salvage tissue as shown by CT perfusion or diffusion-weighted MRI showing limited infarct core volume
ROSIER score used in medical settings
List causes of subarachnoid haemorrhage and describe its diagnosis and management
Clinical features
- thunderclap headache
> maximal intensity <5mins
> sudden onset severe occipital headache
- meningism (photophobia, neck stiffness)
- nausea and vomiting
- coma
- seizures
- neurological symptoms: visual changes, dysphasia, focal weakness, reduced consciousness
- ECG changes - ST elevation
risk factors: female sex, smoker, hypertension, black ethnic origin, excessive alcohol
Diagnosis
- Non-contrast CT head <6h after onset: if negative, consider alternative diagnosis
- if CT >6h after onset is negative do LP after 12h (confirm xanthochromia)
- after spontaneous SAH is confirmed
> CT intracranial angiogram +/- digital subtraction angiogram
Management
> supportive: bed rest, analgesia, VTE prophylaxis, discontinuation of antithrombotics
> nimodipine for prevention of vasospasm
- neurosurgical referral due to risk of aneurysm rebleeding
> endovascular coiling by interventional neuroradiologist (most common)
> if severe, neurosurgical clipping or craniotomy
list complications of subarachnoid haemorrhage
- Re-bleeding
- hydrocephalus (treat with LP, external ventricular drain or long-term ventriculoperitoneal shunt)
- vasopasm
> prevention with nimodipine - hyponatraemia (due to SIADH)
- Seizures
> treat with anti-epileptics - Torsades de Pointes
describe the management of Parkinson’s disease
- Levodopa (co-careldopa contains levodopa and carbidopa)
> reduced effectiveness with time - Carbidopa: DOPA decarboxylase inhibitor
- dopamine receptor agonists e.g. bromocriptine, cabergoline, apomorphine
> highest chance of inhibition disorders - COMT inhibitors e.g. entacapone
- MAO-B inhibitors e.g. selegiline
- amantadine
List side-effects of levodopa
- dry mouth
- anorexia
- CVS: palpitations, postural hypotension, arrhythmias
- psychosis, hallucinations
- on-off phenomenon
- end-of-dose dyskinesia
describe the features of Parkinson’s disease
clinical features
- Bradykinesia
> test with finger-tap test and pronation-supination test
- Unilateral pill-rolling tremor
> asymmetrical, 4-6 Hz, enhanced by anxiety - shuffling gait with reduced arm swing
- Postural instability
- Leadpipe rigidity or cogwheeling
Neuropsychiatric
- Anxiety, depression
- Hallucinations
- REM sleep disorders
- Cognitive impairment (Parkinson’s disease dementia)
> motor symptoms ongoing at least a year before the emergence of dementia
Autonomic
- Altered sense of smell (hyposmia)
- Constipation
Genitourinary
- Incontinence
- Nocturia, frequency
- Erectile dysfunction
Myalgia
Hyperhidrosis (sweating)
Hypomimia
Infrequency of eye blinking
Hypophonia
List lower motor neurone signs
- Reduced tone
- Hyporeflexia
- Fasciculations
- Paresis
- Weakness in a root innervated pattern
- muscle atrophy
Describe medication overuse headaches and their treatment
Features
- present for 15 days or more per month
- developed or worsened whilst taking regular symptomatic medication
- patients using opioids and triptans are at most risk
- may be psychiatric co-morbidity
Management
- simple analgesics and triptans should be withdrawn abruptly (may initially worsen headaches)
- opioid analgesics should be gradually withdrawn
which medications can lead to drug-induced parkinsonism?
typical antipsychotics e.g. haloperidol
antiemetics e.g. metoclopramide, prochlorperazine
> symptoms are likely to be bilateral in drug-induced parkinsonism
describe the management of acute confusional state
treat the underlying cause and modify environment
if not working then haloperidol / olanzapine can be used
if Parkinson’s use atypical antipsychotics like quetiapine or clozapine
describe vertebrobasilar ischaemia
features
- vertigo upon neck extension (looking up)
- exacerbated by changes in head position
- falls in elderly patients
risk factors - CVD, atherosclerosis
in a painful third nerve palsy where would the lesion be located?
posterior communicating artery aneurysm
after trauma an oculomotor nerve palsy + hemiparesis can be a sign of transtentorial / uncal herniation (medial aspect of temporal lobe compressing CN III and cerebral peduncle)
describe the following conditions affecting the spinal cord
- Brown-Sequard syndrome
- friedrich’s ataxia
- anterior spinal artery occlusion
- neurosyphilis
Brown-Sequard syndrome
- aka spinal cord lateral hemisection
- ipsilateral spastic paresis below lesion
- ipsilateral loss of proprioception and vibration sensatoin
- contralateral loss of pain and temperatures sensation
friedrich’s ataxia
- same as above + cerebellar ataxia, intention tremor
anterior spinal artery occlusion
- bilateral spastic paresis
- bilateral loss of pain and temperature sensation
neurosyphilis (tabes dorsalis)
- loss of proprioception and vibration sense as dorsal columns are affected
which diet is used in children with epilepsy which is unresponsive to treatment?
ketogenic diet
describe the diagnosis of multiple sclerosis
diagnosis requires dissemination of demyelinating lesions in time and space
- MRI brain + spinal cord with contrast
> high signal T2 lesions
> periventricular plaques
> Dawson fingers - +ve Hoffman’s sign
- CSF
> oligoclonal bands
> increased intrathecal synthesis of IgG - Visual evoked potentials
> delayed but well-preserved waveform
risk factors: EBV infection, multiple genes, low vitamin D, smoking, obesity
Describe the symptoms of stroke depending on the affected vessel
ACA
> contralateral hemiparesis and sensory loss
> lower extremity > upper
MCA
> contralateral hemiparesis and sensory loss
> upper extremity > lower extremity
> aphasia
> contralateral homonymous hemianopia
PCA
> Contralateral homonymous hemianopia with macular sparing
> Visual agnosia
Weber’s syndrome (branches of PCA supplying midbrain)
> contralateral upper and lower limb weakness
> ipsilateral CN III palsy
PICA (lateral medullary syndrome, Wallenberg syndrome)
> ipsilateral facial pain and temperature loss
> contralateral limb/torso pain and temperature loss
> ataxia, nystagmus
> ipsilateral Horner’s syndrome
AICA (lateral pontine syndrome)
> similar to Wallenberg’s but ipsilateral facial paralysis and deafness also
> reduced GCS, paralysis, bilateral pinpoint pupils
Retinal/ophthalmic artery: amaurosis fugax
Basilar artery: locked in syndrome
Lacunar strokes present with either isolated hemiparesis, hemisensory loss or hemiparesis with limb ataxia
> strong association with hypertension
> common sites include the basal ganglia, thalamus and internal capsule
describe multiple sclerosis and its clinical features
Idiopathic inflammatory demyelinating disease of the CNS
Acute episodes of inflammation are associated with focal neurological deficits
visual symptoms
- optic neuritis
- optic atrophy
- Uhthoff’s phenomenon: worsening of vision following rise in body temperature
- internuclear ophthalmoplegia
sensory symptoms
- pins/needles
- numbness
- trigeminal neuralgia
- Lhermitte’s syndrome: paraesthesiae in limbs on neck flexion
motor symptoms
- spastic weakness (most commonly in legs)
cerebellar symptoms
- ataxia
- tremor
others symptoms
- urinary incontinence
- sexual dysfunction
- intellectual deterioration
describe Miller Fisher syndrome
subtype of Guillain-Barré syndrome (GBS)
characterised by areflexia, ataxia and ophthalmoplegia
descending weakness is a classic features as opposed to ascending weakness in more common forms of GBS
anti-GQ1b antibodies present in most cases
describe osmotic demyelination syndrome
aka central pontine myelinolysis
- can occur due to overcorrection of severe hyponatraemia
- Na levels should only be raised by 4-6 mmol/l in 24h
symptoms usually occur after 2 days and are irreversible
- dysarthria
- dysphagia
- paraparesis or quadriparesis
- seizures
- confusion
- coma
patients are awake but unable to move or verbally communicate - locked-in syndrome
from high to low your brain will blow - cerebral oedema
from low to high your pons will die - central pontine myelinolysis
Describe the subtypes of multiple sclerosis
- Relapsing remitting Multiple Sclerosis (RRMS)
> Unpredictable attacks which may or may not leave permanent deficits followed by periods of remission - Primary progressive multiple sclerosis (PPMS)
> At least 1 year of disease progression
> Steady increase in disability without attacks - Secondary progressive multiple sclerosis (SPMS)
> Initial relapsing-remitting multiple sclerosis
> Suddenly begins to decline without periods of remission - Benign multiple sclerosis (BMS)
describe the management of multiple sclerosis
- disease-modifying therapies e.g. natalizumab for RRMS or ocrelizumab for PPMS
- relapses: high dose steroids e.g. IV methylprednisolone
- symptomatic
> strength: exercise
> fatigue: amantadine, modafinil, SSRIs
> neuropathic pain: amitriptyline, gabapentin
> depression: SSRIs
> urge incontinence: solifenacin
> spasticity: baclofen, gabapentin
> oscillopsia: gabapentin, memantine
describe the diagnosis of motor neurone disease (MND)
diagnosis is clinical
- features
> asymmetric limb weakness
> mixture of LMN and UMN signs
> wasting of small hand muscles / tibialis anterior
> fasciculations
> emotional lability
> absence of sensory signs/symptoms
> sparing of eye movements
> nerve conduction studies: normal motor conduction, exclude neuropathy
> electromyography: reduced number of action potentials with increased amplitude
> MRI - exclude cervical cord compression and myelopathy
usually pathological TDP-43 proteins
describe the different types of motor neurone disease (MND) and their management
- amyotrophic lateral sclerosis (ALS)
> most common - progressive bulbar palsy
> second most common
> affects talking/swallowing
> causes tongue wasting and fasciculations - progressive muscular atrophy
- primary lateral sclerosis
management
- riluzole: slow progression of disease
- symptomatic
> spasticity: baclofen
> antimuscarinics: excessive salivation
> NIV to support breathing
- advanced directives, end-of-life care
describe Lambert-Eaton myasthenic syndrome
usually a paraneoplastic syndrome occurring alongside small-cell lung cancer (SCLC)
> can occur as a primary autoimmune disorder
caused by antibodies against voltage-gated calcium channels
clinical features
- proximal muscle weakness (difficulty climbing stairs/rising from chair)
- autonomic dysfunction (dry mouth, blurred vision, impotence, dizziness)
- reduced / absent tendon reflexes
symptoms IMPROVE after periods of muscle contraction, as opposed to myasthenia gravis (fatiguability)
management
- exclude malignancy
- amifampridine: voltage-gated potassium channel blocker
- pyridostigmine, immunosuppressants
- IV immunoglobulins, plasmapheresis
describe Huntington’s disease
aka Huntington’s chorea
AD trinucleotide repeat disorder
> demonstrates anticipation: successive generations have earlier age of onset / increased severity of disease
clinical features
- chorea: involuntary, random, irregular and abnormal body movements
- dystonia: abnormal muscle tone leading to abnormal postures
- rigidity
- eye movement disorders
- dysarthria, dysphagia
- cognitive, psychiatric or mood problems
investigations
> genetic testing
> MRI: caudate atrophy
management
- genetic counselling
- SSRIs if depressed
- tetrabenazine for chorea
- supportive: physio, SALT, advanced directives, end-of-life care
describe tuberous sclerosis
AD condition
cutaneous features
- depigmented ash-leaf spots which fluoresce under UV light
- roughened patches of skin over lumbar spine (Shagreen patches)
- adenoma sebaceum (angiofibromas): butterfly distribution over nose
- subungal fibromata
- cafe-au-lait spots may be seen
- angiomyolipomas
neuro features
- developmental delay
- epilepsy (infantile spasms or partial)
- intellectual impairment
also: retinal hamartomas, rhabdomyomas of the heart, gliomatous changes in brain lesions, polycystic kidneys, lymphangioleiomyomatosis
describe neurofibromatosis
2 types: neurofibromatosis type 1 is more common
> NF2 is associated with BILATERAL acoustic neuromas
AD inheritance
clinical features: CRABBING
- cafe au lait spots
- relative with NF1
- axillary or inguinal freckling
- bony dysplasia e.g. bowing of a long bone
- iris hamartomas (Lisch nodules)
- neurofibromas: skin-coloured raised nodules
- glioma of optic pathway
management: monitor for complications
complications
- migraines
- epilepsy
- renal artery stenosis
- multiple tumours (e.g. spinal cord, GIST…)
- vision loss due to optic nerve glioma
describe a generalised tonic clonic seizure
clinical features
- body stiff, rigid, crashes to the ground
- from rigid to rhythmical jerking (not tremor)
- may have urinary incontinence / lateral tongue biting
- cyanotic
- eyes often open (rolled back)
usually last 1-2 minutes
post-seizure
- non-responsive
- heavy breathing/snoring
- post-ictal confusion
- patient may: not remember events, have headache, myalgia, and go to sleep it off
describe seizure classification
generalised
- tonic
- clonic
- atonic
- tonic-clonic
- myoclonic
- absence
focal
- depends on origin of seizure
- +/- impaired consciousness
- +/- becoming generalised
Describe spontaneous intracranial hypotension
Low CSF headaches caused by spontaneous intracranial hypotension
> worse on standing, improved lying flat
more common in connective tissue disorder e.g. Marfan’s syndrome
Conservative management. Epidural blood patch if this fails.
Describe trigeminal neuralgia
Trigeminal neuralgia: pain syndrome characterised by severe unilateral pain in trigeminal distribution
> brief electric shock-like pains, abrupt in onset and termination with a refractory period
triggers
> light touch, washing, shaving, smoking, talking, and brushing the teeth (trigger factors), and frequently occurs spontaneously
idiopathic but compression of trigeminal roots by tumours or vascular problems may occur
Management
- carbamazepine is first-line
- failure to respond to treatment or atypical features (e.g. < 50 years old) should prompt referral to neurology
> surgery: microvascular decompression
Describe the diagnosis of creutzfeldt jakob disease
Rapidly progressive neurodegenerative prion disorder, usually sporadic but can be inherited
clinical features
- rapid cognitive decline
- myoclonic jerks
- behavioural change
- visual disturbance
- ataxia
Key investigations
- MRI
- lumbar puncture
- EEG
> bi- or tri-phasic periodic sharp wave complexes appearing with a frequency of around 1-2 per second.
Definitive diagnosis: post-mortem biopsy
describe the investigations used in stroke
- ROSIER score: asymmetric weakness, speech disturbance, visual disturbance
- Non-contrast CT head:
> acute ischaemic strokes
» areas of low density in the grey and white matter; changes may take time to develop
> > other signs include the ‘hyperdense artery’ sign corresponding with the responsible arterial clot - this tends to visible immediately
> acute haemorrhagic strokes
>hyperdense material (blood) surrounded by low density (oedema)
describe chronic traumatic encephalopathy (CTE)
sequelae of chronic repeated head injury
usually in athletes e.g. rugby / boxing
clinical features
- behavioural change
- cognitive difficulties
- mood disturbance
explain the use of prolactin in seizures
Rise in serum prolactin can help to differentiate a seizure from a pseudoseizure
pseudoseizures also tend to have a gradual onset while true seizures have sudden onset
Other features suggestive of seizure
> Tongue biting
> Urinary incontinence
> Post-ictal phase
> Memory loss
> Todd’s paresis (post-ictal weakness)
Describe the clinical presentation of subdural haemorrhage
caused by rupture of bridging veins
risk factors: elderly, alcoholism, anticoagulation
Clinical presentation
- fluctuating consciousness
- altered mental state
- focal neurology
- falls
- can be triggered by alcohol excess
management
- acute subdural haematoma: neurosurgical referral for evacuation of haematoma e.g. decompressive craniectomy
- chronic subdural haematoma:
> asymptomatic: conservative
> symptomatic: burr hole drainage
describe Wilson’s disease
AR disorder characterised by excessive copper deposition in tissues
Features
- Liver: hepatitis, cirrhosis
- Neurological: speech, behavioural and psychiatric problems
- Kayser-Fleischer rings: green-brown rings in the periphery of the iris
- Renal tubular acidosis
- Haemolysis
- Blue nails
Lab
- Reduced serum caeruloplasmin
- Reduced total serum copper
- Increased 24h urinary copper excretion
Management
- penicillamine (chelates copper)
describe an extradural haematoma
pathophysiology
> mostly trauma, low-impact head injury
> especially over temporal region due to the pterion overlying the middle meningeal artery
patients may experience a lucid interval
> briefly regain consciousness after injury before progressing into coma
imaging
- biconvex or lentiform hyperdense collection limited by suture lines of skull
management
- no neurological deficit: cautious clinical and radiological observation
- definitive treatment: craniotomy and evacuation of haematoma
describe Bell’s palsy and its management
acute, unilateral, idiopathic, facial nerve paralysis.
risk factors
- age 20-40 years
- pregnant women
Features
> lower motor neuron facial nerve palsy → forehead affected
> post-auricular pain (may precede paralysis)
> altered taste
> dry eyes
> hyperacusis
Management
- oral prednisolone within 72 hours of onset of Bell’s palsy
- eye care to prevent exposure keratopathy: prescribe artificial tears and eye lubricants, taping at night
describe syringomyelia
due to development of syrinx (CSF-filled cyst) in the anterior white commisure of the spinal cord
Presentation
- cape-like loss of pain and temperature sensation due to compression of spinothalamic tract fibres decussating in centre of spinal cord
- preserved light touch, proprioception and vibration
- spastic weakness
- neuropathic pain
- upgoing plantars
- autonomic features
> Horner’s syndrome due to compression of sympathetic chain
> scoliosis can develop if untreated
causes
- congenital e.g. Arnold-Chiari malformation
- acquired: trauma e.g haemorrhage, tumour
- idiopathic
investigations
- full spine MRI with contrast - exclude tumour / tethered cord
- brain MRI - exclude Chiari malformation
Treatment
- manage underlying cause
- shunt into syrinx
describe a brain abscess and its management
causes
- extension of sepsis from middle ear or sinuses
- trauma or surgery to the scalp
- penetrating head injuries
- embolic events from endocarditis
clinical features
- depends on the site of the abscess
- headache (dull, persistent)
- fever
- focal neurology e.g. oculomotor nerve palsy
- other features of raised ICP: nausea, papilloedema, seizures
investigations
- CT head
management
- surgery
> craniotomy and debridement of abscess cavity
- IV Abx: IV 3rd generation cephalosporin + metronidazole
- Intracranial pressure management: e.g. dexamethasone
describe Cheyne Stokes breathing
abnormal breathing pattern characterised by periods of hyperventilation alternating with apnoea
occurs due to compression of brainstem
describe juvenile myoclonic epilepsy
typically affects teenage girls
- infrequent generalised seizures often in the morning / following sleep deprivation
- daytime absences
- sudden shock-like myoclonic seizure
describe the management of haemorrhagic transformation in ischaemic stroke
- stop antiplatelets / anticoagulants
- control BP (target 140 mmHg)
describe cavernous sinus syndrome
usually caused by cavernous sinus tumours
features
- pain
- ophthalmoplegia
- proptosis
- trigeminal nerve lesion (ophthalmic branch)
- Horner’s syndrome
describe degenerative cervical myelopathy
presentation
- pain (neck, upper or lower limbs)
- loss of motor function (loss of digital dexterity, arm or leg weakness/stiffness leading to impaired gait and balance)
- loss of sensory function causing numbness
- loss of autonomic function (urinary or faecal incontinence / impotence)
- Hoffman’s sign: exaggerated flexion of terminal phalanx of thumb after flicking DIP joint of middle finger
> UMN sign
MRI - gold standard
management
- urgent referral for assessment by specialist spinal services
- decompressive surgery
describe driving guidelines after TIA
can start driving if symptom free after 1 month - no need to inform DVLA
describe pituitary apoplexy
clinical features
- headache
- vomiting
- neck stiffness
- visual field defects: bitemporal superior quadrantic defect
- extraocular nerve palsies
- features of pituitary insufficiency
> hypotension, hyponatraemia secondary to hypoadrenalism
investigation: MRI
management
- urgent steroids due to loss of ACTH
- careful fluid balance
- surgery
