Neuro Flashcards
what are the names of cells that produce myelin in the CNS?
oligodendrocytes
what are the names of cells that produce myelin in the PNS?
schwann cells
which nervous system does MS affect?
central
what is the most common presentation of MS?
optic neuritis
what are 4 risk factors for MS?
FHx and genetics
EBV infection
Smoking
Low vitamin D
what are 5 signs of upper motor neurone lesions?
Inspection - No significant wasting
Tone - Increased (spasticity/rigidity) + Ankle Clonus
Power - Pyramidal (extensors weaker in arms, flexors weaker in legs)
Reflexes - Hyperreflexia
Plantars - Upgoing (Babinski)
What are 5 signs of lower motor neurone lesions?
Inspection - Fasciculations + Wasting
Tone - Reduced (or normal)
Power - reduced in affected nerve distribution
Reflexes - reduced or absent
what are 2 eye signs of MS?
optic neuritis (pain on eye movement and temporary vision loss to one eye)
Eye movement abnormalities (Internuclear opthalmoplegia or conjugate lateral gaze disorder - 6th CN palsies)
what are 4 motor signs of MS?
Bells palsy
horners syndrome
limb paralysis
incontinence
what are 4 sensory symptoms of MS?
triegeminal neuralgia
Numbness
Paraesthesia
Lhermitte’s sign
what is Lhermitte’s sign? What condition does it signify?
MS
Electric shock sensation that travels down spine into limbs when flexing neck (due to disease in DCML of cervical spine)
what is Uhtoff’s phenomenon?
worsening symptoms of demyelinating disorders (MS) when the body becomes overheated hot weather or hot showers
what are 2 coordination symptoms of MS?
sensory ataxia (due to loss of proprioception +ve romberg)
cerebellar ataxia
what is the mcdonald criteria for MS diagnosis?
2 or more episodes of central nervous system damage disseminated in time and space
Demonstrated on MRI
What is one episode of an MS like attack called?
clinically isolated syndrome
what are 3 patterns of disease in MS?
relapsing-remitting
secondary progressive (starts relapsing-remitting then progresses)
Primary progressive
what 2 investigations can be done to diagnose MS?
MRI brain and spinal cord - gadolinium enhanced T2 weighted
Lumbar puncture
what is seen on LP in someone with MS?
oligoclonal bands in the CSF
what is seen on LP in someone with MS?
oligoclonal bands in the CSF
what are 4 key features of optic neuritis?
Central scotoma (enlarged blindspot)
pain on movement
impaired colour vision
relative afferent pupillary defect
what can be seen o/e in optic neuritis?
Pale optic disk
internuclear ophthalmoplegia
what are 7 causes of optic neuritis?
MS
Sarcoidosis
SLE
Syphilis
Measles and Mumps
Neuromyelitis optica
Lyme disease
what is the management of optic neuritis?
High dose corticosteroid - pred or methylpred
what is the treatment for acute MS relapse?
Methylprednisolone 500mg orally OD for 5 days
OR
1g IV Methylprednisolone daily for 3-5 days (in severe cases)
(plasma exchange can be used in V severe disability)
What is the ongoing treatment for MS?
Immunomodulators and Biologcal therapy
Natalizumab
Ocrelizumab
Fingolimod
Beta-interferon
Glatiramer acetate
What medications can be used to treat neuropathic pain in MS?
amitriptyline or gabapentin
what medication can be used to treat urge incontinence in MS?
anticholinergics - tolterodine or oxybutynin
what medications can be used to treat spacticity in MS?
baclofen
gabapentin
Physio
what are 5 complications of MS?
Neuro - spasticity, fatigue, cognitive impairment, dysphagia
Mental health
Osteoporosis
VTE
Bladder and bowel dysfunction
What is the pathophysiology of parkinson’s disease?
progressive reduction in the dopamine of the basal ganglia leading to disorders of movement
where is dopamine produced in the brain?
substantia nigra
what is are 6 features of benign essential tremor?
Symmetrical
5-8 Hz (5-8 times a second)
improves at rest
Worse with intentional movement
No other parkinsons features
Improves with alcohol
what is the movement disorder diagnostic criteria for essential tremor?
Isolated bilateral tremor
>3 years
May have other tremor locations
No other neurological signs
what is the treatment of essential tremor?
1 - propranolol - 40-160mg BD/TDS
OR
primidone - 50mg OD up to 750mg
gabapentin and benzos can also be used
what are 6 features of parkinson tremor?
pill rolling
asymmetrical (starts unilaterally)
4-6 Hz (times a second)
worse at rest and with distraction
improves with intentional movement
no change with alcohol
what are 3 motor symptoms of parkinsons? (core diagnostic)
Unilateral resting tremor
Cogwheel rigidity
Bradykinesia
what are 5 manifestations of bradykinesia in parkinsons?
handwritting gets smaller (micrographia)
Shuffling gait
Difficulty initiating movement
Difficulty in turning
reduced facial movement and expressions (hypomimia)
what are 5 non-motor symptoms of parkinsons?
Depression
sleep disturbance and insomnia
anosmia
cognitive impairment and memory problems
Postural hypotension
what is the difference in presentation in drug induced parkisons?
Bilateral and rapid onset of symptoms
rigidity and rest tremor are uncommon
what is multiple system atrophy?
degeneration in multiple systems in the brain - causes parkison’s like symptoms as well as autonomic and cerebellar dysfunction
what are 4 symptoms of autonomic dysfunction?
postural hypotension, constipation, abnormal sweating and sexual dysfunction
what is the 1st line investigation for parkinsons?
dopaminergic trial
What is the first line treatment of parkinsons?
Levodopa (co-beneldopa)
what are 4 treatment options for parkinsons?
Levodopa PLUS peripheral decarboxylase inhibitors (carbidopa, benserazide)
Dopamine agonist
Monoamine oxidase B (MAO-B) inhibitors
COMT inhibitors (extends life of levodopa)
what are 2 dopamine agonists?
Cabergoline
Pergolide
Bromocriptine
stimulate dopamine receptors by mimicking action of dopamine
what are 2 notable side effects of dopamine agonists?
pulmonary fibrosis
highest risk of impulsive behaviours
what are 2 monoamine oxidase B inhibitors?
Selegiline
Rasagiline
inhibits enzyme that breaks down dopamine
what are 8 side effect of levodopa?
dyskinesia
impulsive and compulsive behaviours
wearing off
dry mouth
anorexia
palpitations
postural hypotension
psychosis
what medication can be used to manage dyskinesia with levodopa?
Amantadine - glutamate antagonist
when medication shouldn’t be prescribed with levodopa?
iron - reduces absorption of levodopa
what are the 4 diagnostic criteria for lewy body dementia?
Fluctuating cognition with pronounced variation in attention and alertness
Recurrent visual hallucinations
REM sleep behaviour disorder
1+ key feature(s) of Parkinsonism
what protein are lewy bodies made up of?
alpha-synuclein
what is the first line pharmacological treatment for lewy body dementia?
acetylcholinesterase inhibitors
what are 3 examples of acetylcholinesterase inhibitors?
Donpezil
Rivastigmine
galantamine
what medication can be used to treat REM sleep disorder in lewy body dementia?
Clonazepam
or melatonin
what medications should not be used in lewy body dementia?
1st generation antipsychotics
what is the most common cause of dementia?
Alzheimer’s disease
what is the 2nd most common cause of dementia?
Vascular dementia
what are 3 classes of medications which can cause confusion?
Anticholinergics - oxybutuninin, solifenacin, tolterodine
Antihistamines
Tricyclic antidepressants
what are 5 neurological conditions that can cause confusion?
Brain tumours
parkinsons
huntingtons
progressive supranuclear palsy
Normal pressure hydrocephalus
what are 4 endocrine conditions that can cause confusion?
Hypothyroid
Adrenal insufficiency
Cushing’s
Hyperparathyroid (+ hypercalcaemia)
what are 2 nutritional deficiencies that can cause confusion?
B12
Thiamine
what scan should be done in all patients presenting with dementia?
MRI head
what specialist assessment can be used to assess memory?
ACE-III
Addenbrooke’s cognitive examination III
what is the presentation of vascular dementia?
Dementia symptoms with stepwise progression due to vascular events
what criteria can be used to diagnose vascular dementia?
NINDS-AIREN criteria
what is the first line treatment for vascular dementia?
Aspirin
(clopidogrel 2nd line)
Optimise CVD risk factors
what is the definition of dementia?
a chronic progressive neurodegenerative diseasecharacterised by global, non-reversible impairement in cerebral functioning
what are the 4As of dementia?
Aphasia
Amnesia
Apraxia - loss of learned tasks
Agnosia - inability to recognise objects/people
what counts as early onset dementia?
<65 years
how long do dementia symptoms have to be present for diagnosis?
6 months
presence of what gene increases risk of dementia?
apolipoprotein (APOE) E4
what is the pathophysiology of alzheimers disease?
excess of beta-amyloid deposited in plaques along neurones
aggregation of tau proteins in neurofibrillary tangles
what is the 1st line pharma management of alzheimers?
Acetylcholinesterase inhibitors - donepezil, galantamine and rivastigmine
what medication is indicated in severe alzheimers?
Memantine (N-methyl-D-aspartate antagonist)
what class is memantine?
N-methyl-D-aspartate (NMDA) antagonist
what is fronto-temporal dementia?
a progressive dementia usually commencing in early age with slow progressive changes of character and social deterioration followed by impairment of intellect, memory and language functions with apathy euphoria and occasionally extrapyramidal phenomena
what is Bell’s palsy?
sudden acute onset unilateral facial palsy of the facial nerve which fully resolves within 72 hours
may have hyperacusis (find noises loud) due to stapedius muscle paralysis, altered taste and dry mouth
which of bells palsy or stroke are forehead sparing?
Stroke is forehead sparing
Bells palsy is lower motor neurone
What is the first line treatment for bells palsy?
Prednisolone and eye care (within 72 hours)
what can be a complication of bells palsy?
keratoconjuctivitis sicca
what classes as a TIA?
a sudden onset focal neurological deficit which completely resolves within 24 hours of onset
OR
A transient episode of neurological dysfunction caused by a focal brain, spinal cord or retinal ischaemia without acute infarction
what scale can be used to assess for urgent need of thrombolysis?
National institute of health stroke scale
what is the immediate management of TIA? (2)
aspirin 300mg initially then 75mg OD
Refer to specialist to be seen within 24 hours
MRI - including diffusion weighted and blood sensitive sequences
what is the long term management of TIA?
DAPT
1 - Aspirin 300mg then 75mg for first 21 days
PLUS
Clopidogrel 300mg then 75mg indefinitely
2 - Aspirin + MR dipyridamole if clopi contraindicated
PPI cover
Statins - atorvastatin 80mg
what modality should be used for carotid imaging after a non-disabling stroke in the carotid teritories?
Carotid duplex US
OR
MR/CT angiography
what criteria need to be met for carotid enarterectomy?
> 50% stenosis (American guidelines)
70% stenosis (European guidelines)
How long from presentation can alteplase be given for stroke?
up to 4.5 hours
How long from presentation can thrombectomy be considered for ischaemic stroke?
<6 hours
24 hours if affecting large artery can be considered
What is the first line treatment for stroke within 4.5 hours?
IV alteplase (PLUS thrombectomy)
What is the first line treatment for stroke from hours 6-24?
thrombectomy
what scale is used to measure neurological disability?
Modified Rankin scale
what is the first line investigation in stroke?
CT head (non-contrast)
what is the most common cause of extradural haemorrhage?
skull trauma
what blood vessel is most commonly ruptured to cause extradural haemorrhage?
middle meningeal artery (MMA)
what kind of haematoma is visible on CT in extradural haemorrhage?
biconvex and (usually) does not cross suture lines
what is the most common cause of subdural haemorrhage?
sheering of bridging veins which empty the ventral sinuses
what two populations are at great risk of subdural haematomas?
alcoholics
elderly people
due to brain atrophy there is more room for expansion of the haematoma prior to symptoms
what kind of haematoma is visible on CT in subdural haemorrhage?
crescent (sickle) shaped haematoma which can cross suture lines
what is the textbook presentation of subarachnoid haemorrhage?
sudden onset ‘thunderclap’ headache which peaks in severity within 1-5 minutes and lasts more than an hour. Also may be with vomiting, photophobia and non-focal neurological signs
what are 3 surgical options for subdural haematoma?
Burr hole craniotomy and suction irrigation
Trauma craniotomy
Hemicraniotomy and duraplasty
what medication is given insubarachnoid haemorrhage to prevent vasospasm?
nimodipine (calcium channel blocker)
what is the most common cause of a subarachnoid haemorrhage?
rupture of berry (intracranial saccular) aneurysm
what are 5 genetic conditions that increase risk of subarachnoid haemorrhage?
Ehlers-danlos syndrome
marfans syndrome
autosomal dominant polycystic kidney disease
neurofibromatosis type 1
pseudoxanthoma elasticum
what is the surgical treatment of subarachnoid haemorrhage?
1 - endovascular coiling
surgical clipping
what is the CT presentation of subarachnoid haemorrhage?
pooling of blood usually around the circle of Willis or in the sylvian fissure
what are 2 early complication of giant cell arteritis?
vision loss
stroke
what is the most common ocular complication of GCA?
Anterior ischaemic optic neuropathy
what might be seen on fundoscopy in GCA?
swollen pale disc and blurred margins
what are 5 symptoms of GCA?
severe unilateral headache around temple and forehead
scalp tenderness (brushing hair)
jaw claudication
blurred or double vision
painless complete sight loss
May also have fever, aches, fatigue, wt loss, loss appetite
what are 5 diagnostic criteria for GCA?
> 50 years
New onset headache
Temporal artery abnormalities - tenderness on palpation, decreased pulsation
ESR >50 mm/h
Abnormal temporal artery biopsy
what condition is associated with GCA?
Polymyalgia rheumatic
what cells are found on temporal artery biopsy in GCA?
multinucleate giant cells
Granulomas
-ve does NOT rule out GCA
what are 2 diagnostic tests for GCA?
raised ECS (>50 mm/h usually)
temporal artery biopsy
what are 4 non-diagnostic investigations that can be used for GCA?
FBC - may have normocytic anaemia and throbocytosis
LFTs - can have raised alk phos
CRP raised
Duplex USS of temporal artery has hypoechoic halo sign
what can be seen on temporal artery US in GCA?
halo sign
what is the management of GCA?
1- Prednisolone 40-60mg OD until symptoms resolve
Aspirin 75mg OD
PPI - for gastric prevention
Bisphosphonates - bone protection on steroids
what is the management for GCA in patients with visual symptoms or jaw claudication?
500-1000mg methylprednisolone
what are 3 late complications of GCA?
relapse
steroid related complication
stroke
aortitis - aortic aneurysm and dissection
what are 4 different types of migraine?
Migraine without aura
Migraine with aura
silent migraine (aura, no headache)
Hemiplegic migraine
How long does a migraine usually last?
4-72 hours
what are 7 characteristic of a migraine?
Moderate to severe intensity
Pounding or throbbing
Usually unilateral (can be bilateral)
photophobia
phonophobia
osmophobia (smells)
with or without aura
nausea and vomiting
what is the international headache society criteria for migraine?
5 attacks with:
Headache 4-72 hours
At least 2 of - unilateral, pulsating, moderate or severe, aggravated by routine physical activity
At least one of - nausea and vomiting OR photophobia and phonophobia
Not attributed to another disorder
what are 4 most common different types of aura?
sparks in vision
blurring vision
lines across vision
loss of different visual fields
what is a hemiplegic migraine?
migraine with motor symptoms that can mimic stroke
symptoms include - hemiplegia, ataxia and changes in consciousness
Can be autosomal dominantly inherited - Familial hemiplegic migraine
what are some triggers for migraine?
CHOCOLATE
CHeese, chocolate and caffeine
Oral Contraceptive pill (and menstruation)
Alcohol (OH) + dehydration
Anxiety (+ stress)
Travel
Exercise
what is the acute management of migraine?
Triptans - sumatriptan 50mg
+ Paracetamol/NSAIDs/Aspirin
Antiemetics (metoclopramide)
how do triptans work?
5HT receptor agonists (serotonin receptor)
act on smooth muscles in arteries to cause vasoconstriction
act on peripheral pain receptors to inhibit activation
reduce neuronal activity in CNS
what are 3 contraindications to triptans?
HTN
coronary artery disease
previous stroke, TIA or MI
what triptans are offered in predictable menstrual migraine?
Frovatriptan 2.5mg BD
Zolmitriptam 2.5mg BD/TDS
what type of triptans are offered to 12-17 year olds?
Nasal triptans
what is prophylactic management of migraine?
Propanalol - 80-160mg OD
Topiramate (teratogenic)
amitriptyline
acupuncture
what migraine prophylactic medication is teratogenic?
Topirimate - causes cleft lip/palate
what are the 3 branches of the trigeminal nerve?
Ophthalmic (V1)
Maxillary (V2)
Mandibular (V3)
How long does trigeminal neuralgia last?
seconds to hours
what are 3 features of trigeminal neuralgia?
intense unilateral facial pain
electricity like shooting pain
Triggered by cold weather, spicy food, caffeine and citrus fruits
Associated with MS
what are 7 red flags for trigeminal neuralgia?
Sensory changes
Deafness or ear problems
Hx of skin or oral lesion
Pain in ophthalmic division
Optic neuritis
FHx of MS
<40 years at onset
what is the 1st line treatment for trigeminal neuralgia?
carbamazepine 100mg PO OD/BD
what are 8 symptoms of cluster headache?
Unilateral
Clusters of attacks for 4-12 weeks followed by remission for at least 3 months
15 mins to 2 hours
red, swollen watery eye
pupil constriction (miosis)
Ptosis
runny nose
facial sweating
restlessness
what are 4 triggers for cluster headache?
alcohol
strong smells
sleep/circadian disruption
weather changes
what are 5 risk factors for cluster headache?
male
FHx
head injury
smoking
drinking
what is the acute management of cluster headaches?
Triptans sub cut (sumatriptan - 6mg SC)
High flow O2
what is the prophylaxis of cluster headache?
verapamil 80mg PO TDS
what monitoring is needed with verapamil
ECG, BP, LFTs
what is chronic tension type headache?
> 15 days a month
what are 3 management options for chronic tension type headaches?
10 sessions of acupuncture over 5-8 weeks
Low dose amitriptyline
what are headache redflags?
Meningisms
Sudden onset occipital headache
New/changed headache >50
New neurological symptoms
Recent head trauma
Worse on valsalva, or lying
Worse on standing (CSF leak)
Household similar (CO)
Pregnant
current or past malignancy
what are 2 medications that increase risk of medication overuse headache?
Triptans
Opioids
what is the management of medication overuse headache?
withdraw analgesia abruptly or slowly if opioids
what is the triad of horner’s syndrome?
Ptosis
miosis
anhydrosis
what is the pathophysiology of Horner’s syndrome?
damage to the sympathetic nervous system supplying the face
sympathetic nerves from spinal cord pass through sympathetic ganglion in base of neck to be post-ganglionic nerves and travel back into the head alongside the internal carotid
what are 2 methods of investigating horner’s syndrome?
cocaine eye drops - should usually dilate pupil - not in horners
adrenaline eye drops - won’t dilate normal pupil but will dilate in horners
what are 4 central causes of Horner’s?
4 Ss
Stroke
MS
Swelling (tumours)
Syringomyelia - cysts in spinal cord
what are 4 pre-ganglionic lesions that can cause horners?
4Ts
tumour (pancoast)
trauma
thyroidectomy
top rib (extra cervical rib)
what are 4 post-ganglionic lesions that can cause horners?
4Cs
carotid aneurysm
carotid artery dissection
cavernous sinus thrombosis
cluster headache
what feature can be seen in congenital horners syndrome?
Heterochromia - different coloured irises
how can you differentiate between different causes of horners?
Central - anhidrosis of face, arm , trunk
Pre-ganglionic - anhidrosis of face
Post-ganglionic - no anhidrosis
what is the most common cause of bacterial meningitis in adults?
1 - Neisseria meningitidis
2 - streptococcus pneumoniae
what does Neisseria meningitidis look like under the microscope?
gram negative (pink) diplococci
what causes the petechial rash in meningitis?
disseminated intravascular coagulopathy (DIC) and subcutaneous haemorrhages
what is the most common cause of bacterial meningitis in neonates?
Group B strep (strep aglactiae)
what is Kernig’s test?
for meningeal irritation in meningitis
patient lies on back, hip and knee flexed to 90 degrees then straighten knee with hip still flexed - +ve if pain or resistance to movement
what is brudzinski’s test?
for meningitis
patient flat on back, flex their neck to their chest - +ve if causes involuntary flexion of hips and knees
what medication can be given in community for suspected bacterial meningitis?
IM (or IV) benzylpenicilin
<1 year - 300mg
1-9 years - 600mg
>10 years - 1200mg
or cefotaxime in pen allergy
what blood test can be done for meningitis?
meningococcal PCR - quicker than blood cultures
what is the treatment for meningitis in <3 months?
Cefotaxime + amoxicillin (listeria cover)
what is the treatment for meningitis in >3 months?
ceftriaxone (or cefotaxime)
+ dexamethasone QDS 4 days
what non-antibiotic meds can be given in bacterial meningitis?
steroids - Dexamethasone QDS > 3 months
150 micrograms/Kg <16 years
10mg - >16 years
what medication can be given in viral meningitis caused buy HSV or VZV?
Aciclovir
what Abx can be used in highly resistant pneumococcal meningitis?
vancomycin
what Abx can be used in penicillin allergy in meningitis?
Chloramphenicol
what is the prophylactic for meningitis contacts?
ciprofloxacin single dose PO
1-11 mon - 30mg/kg
1-4 year - 125 mg
5-11 years - 250mg
>12 years - 500mg
what is the incubation period of meningitis usually?
7 days
what is the 3 most common causes of viral meningitis?
Coxsackie virus - most common
Mumps
HSV, CMV, Herpes zoster
where does the spinal cord end?
L1-2
what level is a lumbar puncture taken at?
L3-4
when should an LP not be done in suspected meningitis?
Signs of severe sepsis or rapidly evolving rash
Severe respiratory/cardiac compromise
significant bleeding risk
signs of raised ICP
what is the LP picture in bacterial meningitis?
cloudy
high protein
low glucose
high neutrophils
+ve culture
what is the LP picture in viral meningitis?
clear
normal (may be mildly raised) protein
Normal glucose
High lymphocytes
+ve PCR, -ve culture
what is the LP picture in fungal meningitis?
Clear
Mildly raised WBC
raised protein
normal glucose
-ve culture and PCR
what are 5 complications of meningitis?
hearing loss
seizures and epilepsy
congnitive impairement and learning disability
memory loss
focal neurological deficits
waterhouse-friderichsen syndrome - adrenal insufficiency secondary to adrenal haemorrhage
when are children given the meningitis B vaccine?
2 months
4 months
12-13 months
what is the most common cause of encephalitis in adults?
HSV-1
what is a non-infective cause of encephalitis?
autoimmune encephalitis
what are 4 risk factors for encephalitis?
extremes of age
immunodeficiency
viral infection
animal or insect bite - west Nile virus, Japanese encephalitis, contact with cats (toxoplasmosis)
what are 6 symptoms of encephalitis?
Altered consciousness
altered cognition
unusual behaviour
acute onset focal neurological symptoms
acute onset focal seizures
Fever
what lobes are typically affected in encephalitis?
temporal and inferior frontal lobes
what is seen on MRI in encephalitis?
medial temporal and inferior frontal lobe changes - e.g. petechial haemorrhages
what lobe does herpes simplex encephalitis typically affect?
temporal lobes
what medication can be used to treat HSV and varicella zoster encephalitis?
aciclovir
what medication can be used to treat CMV encephalitis?
ganciclovir
what are 3 infections particularly associated with GBS?
campylobacter jejuni
CMV
EBV
what is the pathophysiology of GBS?
B cells create antibodies against antigens on pathogen of proceeding infection which match proteins on the neurones and cause them to be attacked leading to demyelingation. This is called molecular mimicry
what are 4 features of GBS?
symmetrical ascending weakness
reduced reflexes
loss of sensation or neuropathic pain
can progress to cranial nerves
what is the criteria for clinical diagnosis of GBS?
Brighton criteria
what is seen on LP in GBS?
Raised protein
normal cell count
normal glucose
what are 2 investigations for GBS?
Nerve conduction studies
lumbar puncture
what antibody is often present in GBS?
Anti-ganglioside antibodies
what is the 1st line management of GBS?
IV Ig
what is the leading cause of death in GBS?
PE - given VTE prophylaxis
what is Miller-fisher syndrome?
Variant of GBS
Opthamoplegia, areflexia, ataxia - typically affecting eye muscles first
Descending paralysis
what are 4 types of motor neurone disease?
Amyotrophic lateral sclerosis
Primary lateral sclerosis - UMN signs only
Progressive muscular atrophy - LMN signs only
progressive bulbar palsy
what is the most common motor neurone disease?
Amyotrophic lateral sclerosis - ALS
what is the second most common MND?
progressive bulbar palsy - primarily affects muscles of talking and swallowing - tongue wasting and fasiculations - worst prognosis
what is the pathophysiology of MND?
progressive degeneration of upper and lower motor neurones
what muscles are never affected in MND?
external ocular muscles
what are nerve conduction studies like in MND?
NORMAL
what medication can be used to prolong survival in ALS?
Riluzole
what tumour is linked to myasthenia gravis?
thymoma - prolongs like around 3 months
what is the best way to support nutrition in MND?
PEG feeding
what is the pathophysiology of myasthenia gravis?
autoimmune antibodies to Ach receptors at neuromuscular junction of motor nerves bind to receptors and block triggering which would lead to muscle contraction
what are 3 ways to elicit fatiguability in myasthenia gravis?
repeated blinking - leads to ptosis
prolonged upward gazing leads to diplopia
repeated abduction of arm on one side leads to unilateral weakness
what are 6 medications that can exacerbate myasthenia gravis?
penicillamine
quinidine, procainamide
beta blockers
lithium
phenytoin
antibiotics - gent, macrolides, quinolones, tetracyclines
what antibodies are tested for in myasthenia gravis?
acetylcholine receptor antibodies
muscle specific kinase antibodies
LRP4 antibodies
what is a diagnostic test for myasthenia gravis?
edrophonium test - increases Ach at NMJ causing relief of weakness
what are 4 tests for myasthenia gravis?
rapid nerve stimulation
single fibre electromyography
serological antibody testing
CT or MRI chest for thymoma
what are 5 treatment options for myasthenia gravis?
reversible acetylcholinesterase inhibitors - pyridostigmine or neostigimine
immunosupression - prednisolone or axithioprine
thymectomy
MAN - rituximab, eculizumab
what are 3 side effects of acetycholinesterase inhibitors?
abdo cramps
diarrhoea
excessive salivation
what is the treatment of myasthenic crisis?
Immunomodulatory therapies - IV Ig or plasmapheresis
may need NIV if respiratory muscle affected
what are 3 complications of myasthenia gravis?
myasthenia crisis
resp infections and aspiration pneumonia
thymoma
what is the most common type of neurofibromatosis?
type 1
what chromosome is the gene that causes neurofibromatosis type 1 on?
chromosome 17 - codes for tumour suppressor protein neurofibromin
what is the inheritance pattern of neurofibromatosis?
autosomal dominant
what is the diagnostic criteria for neurofibromatosis 1?
CRABBING
Cafe au lait spots (6+ over 5mm kids and 15mm adults)
Relative with NF1
Axillary or inguinal freckles
BB - Bony dysplasia - Bowing of long bone
Irish hamartomas - 2+ yellow/brown spots on iris
Neurofibromas 2+ or 1 plexiform neurofibroma
Glioma of optic nerve
what are 12 complications of neurofibromatosis type 1?
Migraines
epilepsy
renal artery stenosis - HTN
Learning disability
behavioural problems
scoliosis
vision loss - optic nerve gliomas
malignant peripheral nerve sheath tumours
GI stromal tumours
spinal cord tumours
Increased risk of cancers - pheochromocytomas
what chromosome is the neurofibromatosis type 2 gene found on?
chromosome 22
what kind of tumours does neurofibromatosis type 2 lead to?
shwannomas
what tumour is neurofibromatosis 2 most associated with?
acoustic neuromas (bilateral)
what nerves are effected in bulbar palsy?
CN9,10,11,12
what is the function of CN1?
olfactory nerve
smell
what is the function of CN2?
optic nerve
Vision
what is the function of CN3?
oculomotor
Eye movement (up, nasally, down, inferior oblique)
what is the function of cranial nerve 5?
trigeminal
V1 - ophthalmic - scalp, forehead and nose sensation
V2 - maxillary - cheek, lower eyelid, nasal mucosa, upper lip, upper teeth and palate sensation
V3 - jaw, lower teeth, anterior 2/3 tongue sensation, muscles of mastication
what is the function of CN6?
Abducens
lateral rectus movement
what is the function of CN7?
facial nerve
sensation to ear
taste in anterior 2/3 tongue, hard and soft palate
muscles of facial expression
lacrimal, submandibular and sublingual glands and mucous glands
what is the function of CN8?
vestibulocochlear
hearing and balance
what is the function of CN9?
glossopharyngeal
taste and sensation posterior 1/3rd tongue
parotid gland
elevation of larynx and pharynx (stylopharyngeus muscle)
what is the function of CN10?
vagus
sensation external ear, larynx, pharynx
taste from epiglottis
smooth muscle of pharynx and larynx
what is the function of CN11?
spinal accessory
trapezium and sterocleidomastoid innervation
what is the function of CN12?
hypoglossal
intrinsic and extrinsic tongue muscles
what are 4 causes of bulbar palsy?
brainstem stroke or tumour
degenerative disease (MND)
Autoimmune disease (GBS)
Genetic disease - kennedy disease
what causes pseudobulbar palsy?
disease of corticobulbar tracts due to cerebrovascular events, MS, MND, neurosyphilis etc that causes bulbar symptoms along with exaggerated jaw jerk and emotional lability
what are 7 causes of cerebral palsy?
antenatal - maternal infection (rubella, toxoplasmosis, CMV), trauma in pregnancy
perinatal - birth asphyxia, pre-term birth
postnatal - meningitis, severe neonatal jaundice, head injury
what are 4 types of cerebral palsy?
spastic - hypertonia (damage to UMNs)
dyskinetic - muscle tone control problems with abnormal movements (damage to basal ganglia)
Ataxic - coordination problems (damage to cerebellum)
Mixed
what is spastic cerebral palsy?
hypertonia and reduced function due to UMN damage
what is dyskinetic cerebral palsy?
problems controlling muscle tone with hyper and hypotonia casing athetoid (slow writing) movements and oro-motor problems
due to basal ganglia damage
what is ataxic cerebral palsy?
problems with coordinated movement due to cerebellar damage
what are 6 presentations of cerebral palsy?
failure to meet milestones
increased/decreased tone
hand preference before 18 months
problems with coordination, speech or walking
feeding or swallowing problems
learning difficulties
what can be seen on MRI head in cerebral palsy?
Periventricular leukomalacia, congenital malformation, stroke or haemorrhage
what medications may someone with cerebral palsy need?
muscle relaxants for spasticity and contractures - baclofen
antiepileptics
glycopyrronium bromide - excessive drooling
what are 6 complications of cerebral palsy?
learning difficulty
epilepsy
kyphoscoliosis
muscle contractures
hearing/visual impairment
GORD
what is the grading system for hypoxic-ischaemic encephalopathy grade called?
Sarnat staging
what are 3 features of mild hypoxic ischaemic encephalopathy?
poor feeding, irritability, hyperalertness
resolves within 24 hours
normal prognosis
what are 3 features of moderate hypoxic ischaemic encephalopathy?
poor feeding, lethargy, hypotonic and seizures
can resolve in weeks
up to 40% develop cerebral palsy
what are 3 features of severe hypoxic ischaemic encephalopathy?
reduced consiousness, apnoea, flaccidity and reduced or absent reflexes
up to 50% mortality
90% cerebral palsy
what is a possible treatment of hypoxic ischaemic encephalopathy?
therapeutic hypothermia
cool down core temp of baby on ICU to 33-34 degrees for 72 hours which reduces inflammation and neurone loss after acute hypoxic injury
what is the inheritance pattern for huntingtons?
Autosomal dominant
what is the genetic error in huntingtons?
trinucleotide repeat disorder of the HTT gene on chromosome 4
leads to degeneration of cholinergic and GABAergic neurons in striatum of basal ganglia
what chromosome is affected in huntingtons?
chromosome 4
what genetic phenomenon is seen in huntingtons?
anticipation
what genetic mutations lead to anticipation?
trinucleotide repeats
what are 7 conditions with trinucleotide repeats ?
Huntington’s
Fragile X
Myotonic dystrophy
Freidrich’s ataxia
Spinocerebellar ataxia
spinobulbar muscular atrophy
Dentatorubral pallidoluysian atrophy
what is genetic anticipation?
sucessive generations have more trinucleotide repeats resulting in earlier onset and increased severity of disease
what are 6 symptoms of huntingtons disease?
Cognitive
Behavioural and mood changes
Eye movement disorders
Speech and swallow difficulties
Chorea
Rigidity
what is the life expectancy of huntingtons post diagnosis?
10-20 years from onset of symptoms
what are 3 medications that can be used for chorea in huntingtons?
Benzos - tetrabenazine
Antipsychotics
Amantadine
what causes Creutzfeldt-Jakob disease?
Prions
what is the general life expectancy after presentation of someone with CJD?
<1 year
what are 5 symptoms of prion disease?
sudden onset cognitive impairment and decline
Ataxia
Myoclonus
Psychiatric symptoms
parkinsonism
what are 4 ways prion disease can be passed on?
Genetically
Contaminated blood products
Contaminated surgical instruments
Consumption of contaminated beef
what is a specific test that can be done for CJD?
real time quaking induced conversion of the CSF
what do the nerves of the cauda equina supply?
Sensation to lower limbs, perineum, bladder and rectum
Motor supply to lower limbs, anal and urethral sphincters
Parasympathetic control of bladder and rectum
what nerve roots supply control of ureteral and anal sphincters?
S2,3,4 keeps shit of the floor
what are 8 causes of cauda equina?
Herniated disc
tumours
Degenerative lumbar canal stenosis
Abscess
Haematoma
Trauma
spondylolisthesis (displacement of vertebra)
what are 9 red flags of cauda equina?
Lower back pain
Saddle anesthesia
loss of sensation to bladder and rectum
Urinary retention or incontinence
Faecal incontinence
Bilateral sciatica
Bilateral/severe motor weakness of legs
Reduced anal tone
sexual dysfunction
what type of signs are present in cauda equina?
Lower motor neurone
what 2 categories can cauda equina be seperated into?
CE with retention
Incomplete cauda equina
what is the management of cauda equina?
Urgent MRI
Lumbar decompression surgery - decompressive laminectomy
Dexamethasone if due to tumour
what is the treatment for metastatic spinal cord compression?
high dose dexamethasone (8-16mg)
Analgesia
Surgery
radio/Chemo
what is a key feature of the pain in metastatic spinal cord compression?
Back pain worse on coughing or straining
what is the first line investigation for a metastatic spinal cord compression?
Gandolinium enhanced MRI Spine
what are 3 different types of spinal stenosis?
central - narrowing of central spinal canal
lateral - narrowing of nerve root canals
foramina - narrowing of intervertebral foramina
what are 5 causes of spinal stenosis?
Congenital
degenerative changes
Herniated discs
thickening of ligamentum falvum/posterior longitudinal ligament
Spinal fractures
spondylolisthesis
tumours
what are 3 symptoms of spinal stenosis?
intermittent neurogenic claudication - lower back pain, buttock and leg pain, leg weakness
mimics intermitted arterial claudication
what exacerbates central spinal stenosis?
Standing up straight and walking
what relieves spinal stenosis?
bending forwards and resting
what is the management of spinal stenosis?
exercise and wt loss
analgesia
physio
Decompressive laminectomy - if conservative tx fails
what is anterior cord syndrome?
incomplete spinal cord injury due to infarct of the anterior 2/3rds of the spine often because of flexion injuries to C spine
what is the presentation of anterior cord syndrome?
impaired pain and temperature sensation with intact vibration, proprioception and light touch
Motor deficits are also present
what are 3 causes of anterior cord syndrome?
Iatrogenic (throacic/abdo surgery)
Trauma
Hypo perfussion/ischaemia of the anterior spinal artery
what is the brown-sequard syndrome?
hemisection of spinal cord
what is the presentation of brown sequard syndrome?
paralysis, loss of proprioception and vibration on the side of the lesion and loss of pain and temperature on the contralateral side
what is a radiculopathy?
damage to the nerve root - pinched/trapped/inflamed nerve which causes pain, numbness or weakness radiating along the nerve root’s pathway
what are 4 causes of radiculopathy?
Disc herniation
spinal stenosis
spondylolisthesis
trauma or injury
what is the presentation of an L5 radiculopathy vs common peroneal nerve injury?
Both - Weakness of dorsiflexion, weakness of toe extension
L5 - weakness inversion, lower limb tendon reflex changes, L5 dermatomal distribution (lateral leg, middle of foot)
CPN - weakness of eversion, no changes to reflexes, sensory loss over anterior aspects of foot and leg
what examination can be done for eliciting sciatica pain?
Straight leg raise
sciatic stretch
what is the first line management of sciatica?
Amitriptyline
Duloxetine
which nerves form the sciatic nerve?
L4-S3
what cancers most commonly metastasise to bone?
Prostate
Renal
Thyroid
Breast
Lung
what medication can be given for back spasms?
benzodiazepams - diazepam => less than 5 days course
what are 5 symptoms of diabetic neuropathy?
peripheral sensation loss - glove and stocking
Peripheral pain - burning/prickling
reduced ankle reflexes
painless injuries
Erectile dysfunction
what can be used to manage pain in diabetic neuropathy?
1 - amitroptyline, duloxitine, gabapetin, pregabalin
tramadol rescue therapy
pain management clinics
diabetic control
what is narcolepsy?
chronic condition of disrupted sleep wake cycle and REM sleep intrusion into wakeful state
what is the classic tetrad of narcolepsy?
excessive daytime sleepiness
cataplexy
sleep paralysis
hypnagogic/hypnopompic hallucinations
what is the management of narcolepsy?
Sleep hygiene and lifestyle changes, trigger avoidance
Sodium oxybate - for cataplexy
Modafinil - for excessive daytime sleepiness
what are 4 causes of acute transverse myelitis?
Infection
Vaccination
NMO - Neuromyelitis optica
MOG - Myelin Oligodendrocyte Glycoprotein
what nutrients affect spinal cord functioning?
B12
copper
How does NOS affect B12?
effects of B12 blocked by NO though there is enough B12 in the body
what nutrients affect spinal cord functioning?
B12
copper
what are 5 causes of acute transverse myelitis?
Infection
Vaccination
MS
NMO - Neuromyelitis optica
MOG - Myelin Oligodendrocyte Glycoprotein
How does NOS affect B12?
causes functional deficiency of B12 => cannot be converted to active form
what are the 3 categories of GCS?
Eye response
verbal response
motor reponse
what are the GCS levels for eye response?
1 - no eye opening
2 – to pain
3 - to verbal command
4 - spontaneously
what are the GCS levels for verbal response?
1 - no response
2 - incomprehensible sound
3 - inappropriate words
4 - confused
5 - orientated
what are the GCS levels for motor response?
1 - no response
2 - extension to pain
3 - flexion to pain
4 - withdrawal from pain
5 - localised pain
6 - obeys commands
what is charcot marie tooth disease?
genetic condition that causes peripheral motor and sensory deficits which usually presents <10 years but can present later in life depending on the type
what are 7 classical features of charcot marie tooth?
high foot arches - pes cavus
disal muscle wasting and inverted champagne bottle legs
weakness in lower legs - particularly ankle dorsiflexion
weakness in hands
reduced tendon reflexes
reduced muscle tone
peripheral sensory loss
what are 5 possible causes of peripheral neuropathy?
ABCDE
alcohol
B12 deficiency
Cancer and CKD
Diabetes and Drugs
Every vasculitis
what are 3 medications that can cause peripheral neuropathy?
isoniazid
amiodarone
cisplatin
what are 4 features of lambert-eaton myasthenic syndrome?
Progressive muscle strengthening
Limb girdle weakness
Hyporeflexia
autonomic symptoms - dry mouth, impotence, difficulty urinating
what condition most typically occurs with lambert-eaton syndrome?
small cell lung cancer
causes antibodies against voltage-gated calcium channels in presynaptic terminals of the NMJ
what is central cord syndrome?
incomplete cord injury caused by trauma to the cervical spine leading to weakness or neurological symptoms worse in the upper > lower limbs
what nerve is compressed in carpal tunnel?
median nerve
what branch of the median nerve travels through the carpal tunnel?
palmar digital cutaneous branch - supplies sensation to palmar aspect of thumb, index and middle finger, lateral half of ring finger
what hand muscles does the medial nerve supply motor innervation to?
Abductor pollicis brevis - thumb abduction
opponens pollicis - thumb opposition
Flexor pollicis brevis - thumb flexion
what are 7 risk factors for carpal tunnel?
repetitive strain
obesity
perimenopause
rheumatoid arthritis
diabetes
acromegaly
hypothyroidism
what are 2 special tests to test for carpal tunnel?
phalen’s - full flexion of wrists and hold - +ve if produces symtpoms
tinel’s test - tapping over carpal tunnel causes symptoms
what is the management of carpal tunnel?
6 weeks of
night wrist splint
corticosteroid injection into carpal tunel
hand exercises and mobalisation techniques
then - surgical decompression with flexor retinaculum division
what are 5 presentations of labert-eaton syndrome?
proximal muscle weakness
intraoccular muscle weakness - double vision
levator muscle weakness - ptosis
oropharyngeal muscle weakness - dysphagia
autonomic dysfunction - dizziness, impotence, dry mouth, blurred vision
what is the treatment for lambert-eaton syndrome?
1 - amifampridine
imunosuppresion
IVIG
plasmapheresis
what are hamartomas?
benign neoplastic growths
what condition are hamartomas seen in?
tubular sclerosis
what genetic mutations cause tubular sclerosis?
TSC1 gene on chromosome 9 - codes for hamartin
TSC2 gene on chromosome 16 - codes for tuberin
what are 6 skin signs seen in tubular sclerosis?
ash leaf spots
shagreen patches - thickened, dimpled, pigmented patch of skin
angiofibromas - small skin coloured or pigmented papules over nose and cheeks
subungual fibromata - circular painless lumps growing from nail bed
cafe au lait spots
poliosis - isolated patch of white hair
what are 2 neurological features of tubular sclerosis?
epilepsy
learning disability and developmental delay
what are 5 non-neuro signs of tubular sclerosis?
rhabdomyomas of heart
gliomas
polycystic kidneys
lymphagioleimyomatosis
retinal hamartomas
damage to white nerve causes winging of the scapula?
spinal accessory - CN11
what is the most common cause of foot drop?
common peroneal nerve injury
what is the CSF picture in viral encephalitis?
raised WBCs
HSV +ve PCR
what is the treatment for herpes encephalitis?
IV acyclovir 10mg/kg every 8 hours for 14 days (21 days if immunocompromised)
what is the most common viral cause of encephalitis?
HSV
what is the most common cause of autoimmune encephalitis?
N-methyl D-aspartate receptor antibody encephalitis
(anti-NMDA receptor encephalitis)
what condition is anti-NMDA receptor encephalitis be associated with?
ovarian teratomas
what scale is used for stroke?
national institute of health stroke scale (NIHSS)
what is a mild NIHSS stroke?
1-5
what is a moderate NIHSS stroke?
5-14
what is a severe NIHSS stroke?
15-24
what is a very severe NIHSS stroke?
> 25
what is the management for TIA or minor stroke if thrombolysis/thrombectomy are not appropriate?
dual anti-platelet - aspirin 300mg followed by 75mg daily + clopidogrel 300mg followed by 75mg daily
what 5 investigations should be done for stroke?
CT
MRI
ECG
carotid doppler
what is the management of moderate/severe stroke if thrombectomy/thrombolysis are not indicated?
aspirin 300mg then 75mg daily for 2 weeks until starting antiplatelet therapy - clopidogrel
what is CSF absorbed by?
arachnoid granules
what is the most common cause of hydrocephalus?
aqueductal stenosis
what are 4 congenital causes of hydrocephalus?
aqueductal stenosis
arachnoid cysts - blood csf outflow
arnold-chiari malformations - cerebellum herniates through foramen magnum
Chromosomal abnormalities and congenital malformations
what are 5 signs of hydrocephalus in babies?
rapid increasing head circumference
bulging anterior fontanelle
poor feeding and vomiting
poor tone
sleepiness
what is the management for hydrocephalus?
vetriculoperitoneal shunt
what are 5 ventriculoperitoneal shunt complications?
infection
blockage
excessive drainage
intraventricular haemorrhage during surgery
outgrowing them
what are 3 features of normal pressure hydrocephalus?
Wet, Whacky, Wobbly
gait apraxia (disturbance)
cognitive impairment
urinary frequency/incontinence/urgency - also sometimes foecal
what are 5 secondary causes of normal pressure hydrocephalus?
Subarachnoid haemorrhage
Meningitis
Traumatic brain injury
Intracranial tumours
Ventricular shunting or LP
what can be seen on imaging in normal pressure hydrocephalus?
Enlarged 4th ventricle
Absence of sulcal atrophy
what is the management for normal pressure hydrocephalus?
ventriculoperitoneal shunt if suitable for surgery
what is the presentation of ME? (8)
persistent disabling fatigue
post exertional malaise
unrefreshing sleep
congnitive and autonomic dysfunction
myalgia
arthralgia
headaches
sore throat and lymph nodes (no lymphadenopathy)
what are 5 risk factors for brain abscess?
sinusitis/otitis media
dental procedure
meningitis
recent head/neck/neuro surgery/trauma
HIV/immunocompromised
Cyanotic congenital heart disease - bypass pulmonary capillary filter
what are the 3 routes of spread of brain abscesses?
Haematogenous spread
Direct spread from contiguous foci
Direct inoculation
what are 3 pathogens responsible for brain abscesses in neonates?
GBS
E. Coli
Listeria monocytogenes
what are 3 pathogens responsible for brain abscesses in immunocompromised people?
aspergillus
candida
toxoplasmosis gondii
what are 2 bacteria species which cause brain abscess associated with dental work, otitis media and sinusitis?
Streptococci species
bacteroides species
what is the presentation of brain abscess?
headache - severe and localised, refractory to analgesia
Altered mental status
Slurred speech
Seizure
Nausea and vomiting
fever
focal neurological deficits
meningism
what on MRI with contrast in brain abscess?
ring enhancing lesion
what is the management of brain abscess?
IV vancomycin
IV Metronidazole
IV ceftriaxone
SURGERY - abscess drainage - usually >2.5cm
what are 5 complications of brain abscess?
raised ICP
Epilepsy
permanent neurological deficits
meningitis
recurrence of abscess
what is usually seen on CT in cerebral toxoplasmosis gondii?
multiple ring enhancing lesions
possible mass effect
what is mononeuritis multiplex?
progressive motor and sensory deficits in the distribution of specific peripheral nerves commonly caused by vasculitis, hypersensitivity reactions or infection.
what is an investigations for mononeuritis multiplex?
electromyogram
what is the management for mononeuritis multiplex?
corticosteroids - pred 1mg/kg/day BO
+/- immunosupression - oral cyclophosphamide
what is wernicke’s aphasia?
receptive aphasia causing sentences to not make sense and comprehension to be impaired
where is wernicke’s area?
superior temporal gyrus of dominant hemisphere
what blood vessel supplies wernicke’s area?
inferior devision of (usually) left middle cerebral artery
what is broccas aphasia?
expressive aphasia causing halting speech and impaired repetition with normal comprehension
where is broccas area?
inferior frontal gyrus of dominant hemisphere
what blood vessel usually supplies broccas area?
superior division of the (usually) left MCA
what is conduction aphasia?
due to stroke affecting arcuate fasiculus (connection between wernickes and broccas). Fluent speech with impaired repetition
which tumours most commonly metastasise to brain?
lung (most common)
breast
bowel
skin (namely melanoma)
kidney
what is the most common primary brain tumour in adults?
glioblastoma multiforme
what does glioblastoma multiforme look like on imaging?
solid tumours with central necrosis and a rim that enhances with contrast. Disruption of the blood-brain barrier and therefore are associated with vasogenic oedema.
what cells do meningiomas arise from?
arachnoid cap
what genetic condition is associated with bilateral acoustic neuroma?
neurofibromatosis type II
what is the most common primary brain tumour in children?
Pilocytic astrocytoma
what phenomena are seen in occipital lobe focal seizures?
visual flashers or floaters
what phenomena are seen in parietal lobe focal seizures?
sensory - parasthesia
what phenomena are seen in frontal lobe focal seizures?
head/leg movements, posturing, jacksonian march, post-ictal weakness (todds paresis)
what phenomena are seen in temporal lobe focal seizures?
pre-ictal aura
automatisms
what is the GCS scoring?
MoVE - 6,5,4
what is the MOA of memantine?
NMDA receptor antagonist
what are 2 side effects of donepezil?
contraindicated in bradycardia
can cause insomnia
what dermatome is the thumb and first finger?
C6 - make a 6 with left hand thumb and first finger
what dermatome is the nipple?
T4 - teat pore
what dermatome is the umbilicus?
BellybuT-TEN = T10
what dermatome is the inguinal ligament?
L1 - Li
what dermatome is the knee caps?
ALL Fours - L4
what dermatome is the big toe and foot dorsum?
L5 - largest of 5 toes
what dermatome is little toe?
S1 - smallest one
what dermatome is the genitalia?
S2/3
what are 3 side effects of dopamine receptor agonists?
pulmonary, retroperitoneal and cardiac fibrosis
impulse control
excessive daytime sleepiness
what is autonomic dysreflexia?
In patients with spinal injury at or above T6 - response to painful stimuli that causes unbalanced physiological response - extreme hypertension, flushing, sweating (above level of lesion), agitation and extreme hypertension
what level does an injury have to be above to cause autonomic dysreflexia?
T6
what is the most common cause of autonomic dysreflexia?
faecal impaction or urinary retention
what is the management of autonomic dysreflexia?
removal/control of the stimulus and treatment of any life-threatening hypertension and/or bradycardia
what are 8 side effects of carbamazepine?
P450 enzyme inducer
dizziness and ataxia
drowsiness
headache
visual disturbances (especially diplopia)
Steven-Johnson syndrome
leucopenia and agranulocytosis
hyponatraemia secondary to syndrome of inappropriate ADH secretion
what is the MOA of carbamezapine?
binds to sodium channel increasing their refractory period
what are 5 causes of foot drop?
common peroneal nerve lesion
L5 radiculopathy
sciatic nerve lesion
superficial or deep peroneal nerve lesion
CNS lesion (stroke etc)
what examination findings indicate peroneal nerve injury?
weakness of foot dorsiflexion and eversion. Reflexes normal
what examination findings indicate L5 radiculopathy?
weakness of hip abduction
what are 3 features of Intracranial venous thrombosis?
Worsening headache
Nausea and vomiting
Blurred vision
what are 3 features of sagital sinus thrombosis?
Seizures and hemiplegia
parasagital biparietal or bifrontal haemorrhagic infarctions
empty delta sign on MRI venogram
what are 5 signs of cavernous sinus thrombosis?
periorbital erythema and oedema
ophthalmoplegia - 6th nerve 1st then 3/4
Trigeminal nerve involvement
central retinal vein thrombosis
what is the gold standard investigation for venous sinus thrombosis?
MRI venogram
what is the management of Intracranial venous thrombosis?
LMWH acutely
then warfarin
what is lateral medullary syndrome and it’s 6 features?
Occurs due to occlusion of posterior inferior cerebellar artery (PICA)
Ataxia
Nystagmus
ipsilateral dysphagia, facial numbness, CN palsy
Contralateral - limb sensory loss
what is weber’s syndrome?
stoke of branches of posterior cerebral artery suppling mid brain
leads to ipsilateral II nerve palsy and contralateral weakness
what is normal intracranial pressure?
7-15 mmHg while supine
what is the equation for cerebral perfusion pressure?
CPP = mean arterial pressure - ICP
What is cushing’s triad of raised ICP?
widening pulse pressure
bradycardia
irregular breathing
sign of impending herniation
what is the management of raised ICP?
head elevation by 30 degrees
IV manitol
controlled hyperventilation
removal of CSF
dexamethasone - brain abscess or tumour
surgical decompression
what are 5 complications of raised ICP?
cerebral herniation
brain stem herniation
permanent neurological deficits
coma
death
what are 4 complications of mannitol?
hypotension
rebound increase in ICP
volume overload
hyponatraemia
what does occlusion of the retinal artery cause?
Amaurosis fugax
what does occlusion of the basillar artery cause?
locked in syndrome
what is the most common cause of Wernicke’s encephalopathy?
Alcoholism
what are 3 rarer causes of wernicke’s encephalopathy?
persistent vomiting
stomach cancer
dietary deficiency
what is the presentation of wernickes encephalopathy?
nystagmus or ophthalmoplegia
Ataxia
encephalopathy - confusion, disorientation
peripheral sensory neuropathy
what causes wernicke’s encephalopathy?
Thiamine B1 deficiency
alcoholism
persistant vomiting
stomach cancer
dietary deficiency
what is the management of Wernicke’s encephalopathy?
Thiamine (B1) replacement - IV pabrinex for 5 days followed by oral thiamine
what are examples of 5-HT3 antagonists?
Ondansetron
Palonesetron
Anti-emetics used in chemo
what are 2 side effects of ondansetron?
prolonged QT + ventricular tachycardia
constipation
what are 2 symptoms of a posterior cerebellar stroke?
Contralateral homonymous hemianopia with macular sparing
Visual agnosia
what are 2 signs of webbers syndrome (infarct of branches of the posterior cerebral artery that supply midbrain)?
Ipsilateral CN III palsy
Contralateral weakness of upper and lower extremity
what artery is affected in lateral pontine syndrome?
Anterior inferior cerebellar artery
what are of lateral pontine syndrome?
Ipsilateral: facial paralysis and deafness
Contralateral: limb/torso pain and temperature loss
Ataxia, nystagmus
what scale measures disability?
Barthel index
what quick stroke assessment scale is used by medical professionals?
Rossier
when is statin therapy started after stroke?
cholesterol >3.5
wait 48 hours to avoid haemorrhagic transformation
what is the 1st line secondary prevention of stroke?
Aspirin - 75mg
clopidogrel - 75mg - start 2 weeks post stroke to avoid haemorrhagic transformation
what is the 2nd line secondary prevention of stroke?
Aspirin + modified release dipyridamole
what is the management of idiopathic intracranial hypertension?
1 - loose weight
2 - Acetazolamide
3+ - Lumboperitoneal shunt, Optic nerve sheath fenestration, Ventriculoperitoneal shunt
what is status epilepticus?
a single seizure >5 mins
>2 seizures in a 5 minute period without regaining consciousness between
what is the management of status epilepticus?
A-E
1 - Benzodiazepines
2 - Antiepileptics
what is the 1st line management of status epilepticus?
1 - PR diazepam - 10mg (ADULTS) /buccal midazolam 10mg (ADULTS)
1 - IV Lorazepam 4mg (ADULTS)
2 - repeat dose IV lorazepam after 10 mins
What is the 2nd line management of status epilepticus?
IV Levetiracetam
IV phenytoin
IV sodium valproate
Alert on call anaesthetist
what is the management of refractory status epilepticus?
> 45 minutes
induction of GA or IV phenobarbital
what are 6 risk factors for status epilepticus>
epilepsy
poor compliance with anti-epileptics
alcoholism
recreational drug use
previous neuronal injury
Electrolyte imbalance
what are 4 complications of status epilepticus?
Arrythmias and cardiac arrest
respiratory failure and aspiration pneumonia
hyperkalaemia
hypoglycaemia
memory impairment
How long does fatigue have to occur for to diagnose chronic fatigue syndrome?
3 months of fatigue affecting mental and physical functioning more than 50% of the time
what are 11 tests for people presenting with fatigue?
FBC
U+E
LFT
Glucose
TFT
ESR
CRP
Calcium
CK
Ferritin
coeliac screen
urinalysis
what are 5 types of seizures seen mainly in adults?
Generalised tonic clonic
Partial/focal seizure
Myoclonic seizure
tonic seizure
atonic seizure
what are 3 types of seizures more common in children?
Absence seizures
infantile spasms
febrile convulsions
what is the first line management for tonic-clonic seizures? M+F
Males - sodium valporate
Females - lamotrigine or Levetiracetam
what are atonic seizures?
drop attacks with breif lapses of muscle tone usually lasting <3 mins
may be indicative of lennox-gastaut syndrome
what is the 1st line management of atonic seizures?
M - sodium valporate
F - lamotrigine
what is the management of myoclonic seizues?
M - sodium valporate
F - lamotrigine
2nd - levetiracetam or topiramate
what condition can myoclonic occur as part of in children?
Juvenile myoclonic epilepsy
what are 8 side effects of phenytoin?
P450 enzyme inducer
Dizziness and ataxia
Drowsiness
Gingival hyperplasia
Hirtuism
Folate deficiency - megaloblastic anaemia, peripheral neuropathy
Osteomalacia - due to enhaced vit D metabolism
Lymphadenopathy
what is the MOA of phenytoin?
binds to sodium channel increasing their refractory period
what are 2 side effects of lamotrigine?
stevens-johnson syndrome
leukopenia
what is the first line management for tonic clonic seizures in children (under child bearing age)?
sodium valporate
what is the second line management for tonic clonic seizures in kids?
lamotrigine or levetiracitam
where in the brain do focal seizures usually start?
temporal lobes
affect hearing, speech, memory and emotion may cause people to do things on autopilot
what is the management for focal seizures?
1 - lamotrigine or levitiracitam
2 - carbamazepine, oxcarbazepine, zonisamide.
3 - lacosamide
what is the 1st line management for absence seizures?
1 - ethosuximide
2 - Male - sodium valproate
Female - lamotrigine/levetiracetam
what antiepileptic may exacerbate absence seizures?
Carbamazepine
what syndrome is atonic seizures linked to?
Lennox-Gastaut syndrome
what is the mangement for atonic seizures?
1 - sodium valporate
2 - lamotrigine
what is the 1st line management for myoclonic epilspsy?
M - Sodium valporate
F - Levetiracetam
what is the 2nd line management for myoclonic seizures?
lamotrigine, levetiracetam or topiramat
what are 8 side effects of sodium valproate?
teratogenic - neural Hepatitis
Hair loss
Tremor
Ataxia
Pancreatitis
Thrombocytopaenia
P450 enzyme inhibitor
what is the MOA of sodium valproate?
increases activitiy of GABA
what are 2 side effects of ethosuximide?
Night terrors
Rashes
what is the medical management of seizure in the community?
Buccal midazolam
Rectal diazepam
what are the rules surrounding epilepsy and driving?
No driving for 6 months following one seizure
with epilepsy much be fit free for 12 months
when are antiepileptics usually started?
after 2nd seizure
what are 4 indications for starting antiepileptics after 1st seizure?
neurological deficit
structural abnormality on imaging
EEG unequivocal
Patient/carers consider risk of further seizure unacceptable
how much folic acid should women hoping to become pregnant on antiepileptics take?
5mg OD
what birth defect is phenytoin associated with?
cleft palate
what is a seizure?
paroxysmal alteration of neurological function as a result of excessive hypersychronous discharge of neurons within the brain
what are 5 genetic conditions that increase risk of epilepsy?
cerebral palsy
tuberous sclerosis
neurofibromatosis
metabolic disorders
mitochondrial disease
what is one illegal drug that causes seizures in overdose?
Cocaine
what are lacunar infarcts?
small infarcts around basal ganglia, internal capsule, thalamus and pons
presents with unilateral weakness/sensory deficit of face/arm/leg
OR
Pure sensory stroke
OR
Ataxia hemiparesis
what is a total anterior circulation stroke?
Middle and anterior cerebral arteries
ALL THREE
Unilateral hemiparesis/hemisensory loss
Homonymous hemianopia
Higher cognitive dysfunction
what is a partial anterior circulation stroke?
smaller arteries of anterior circulation
TWO OF:
Unilateral hemiparesis/hemisensory loss
Homonymous hemianopia
Higher cognitive dysfunction
what is a posterior circulation infarct?
vertebrobasilar arteries
ONE of:
cerebellar or brainstem syndromes
Loss of consciousness
Isolated homonymous hemianopia
what is the maximum BP before thrombolysis/
185/110 mmHg
what are 10 contraindications to thrombolysis?
prev intracranial haemorrhage
seizure with stroke
Intracranial neoplasm
suspected haemorrhage
Stroke or traumatic brain injury in last 3 months
LP in last 7 days
GI haemorrhage in last 3 weeks
active bleeding
oesophageal varices
uncontrolled HTN
what are 6 relative contraindications to thrombolysis?
pregnancy
concurrent anticoagulation - INR >1.7
Haemorrhagic diathesis
Active diabetic haemorrhagic retinopathy
suspected intracardiac thrombus
major surgery/trauma in past 2 weeks
can dextrose be given as fluids in post stroke patients?
NO for 1st 48 hours
what is the equation for cerebral perfusion pressure?
CPP = Mean arterial pressure - intracranial pressure
what nerve is compressed in cubital tunnel syndrome?
Ulnar nerve
what is the presentation of cubital tunnel syndrome?
tingling and numbness of 4th and 5th finger - starts intermittently and becomes constant
weakness and muscle wasting
pain worse leaning on affected elbow
Hx of OA or prev trauma
what is the management of cubital tunnel syndrome?
avoid aggravating activities
pysio
steroid injections
surgery
what are the symptoms of cerebellar disease?
DANISH
Dysdiadochokinesia, dysmetria, Drunk appearance
Ataxia
Nystagmus
Intention tremor
Slurred staccato speech
Hypotonia
what are 8 causes of cerebellar disease?
friedreich’s ataxia
neoplastic
stroke
alcohol
MS
Hypothyroid
Drugs - phenytoid, lead poisoning
Paraneoplastic
what are 7 causes of peripheral nerve injuries/palsies?
Trauma
Compression - carpal/cubital tunnel
Inflammatory causes - vasculitis, sarcoidosis
Metabolic causes - DM
Infectious causes - lyme disease, HIV
Neoplastic causes
Iatrogenic
what is neuropraxia?
mild nerve injury characterised by temporary blockage of nerve conduction without anatomical disruption
transient motor/sensory loss, No wallerian degeneration, rapid recovery
what is axonotmesis?
damage to axon with preservation of endoneurium, perineurium and epineurium - caused by stretch or crush injuries
motor and sensory loss below level of injury, wallerian degeneration, potential for regeneration with variable recovery
what is neurotmesis?
complete transection or disruption of nerve fibre and all its structures
total motor and sensory lss below level, complete wallerian degeneration, no spontaneous recovery
what classification system can be used for nerve injuries?
Sunderland’s classification
what is Klumpke’s palsy?
damage to C8/T1 (lower trunk of brachial plexus) usually due to hyper-abduction traction during birth
causes ‘claw hand’ - supinated forearm with flexed wrist and fingers
damage to which nerve of the forearm can cause wrist drop?
radial nerve
