Neurology_Medicine & Surgery Flashcards

Question 1

Q

Neuroanatomy - lobes

Answer

A

Lobes

Frontal:
- Primary motor cortex (posterior frontal lobe) - initiates + regulates movement
  - NOTE: in motor cortex face/arms are lateral, legs are medial
- Broca zone - speech production (defect –> expressive dysphasia)
Parietal:
- Primary somatosensory cortex (anterior parietal lobe)
Occipital:
- Primary visual cortex (posterior occipital lobe)
  - Optic nerve pathways to lateral geniculate nucleus –> optic radiations through temporal and parietal lobes –> primary visual cortex
Temporal:
- Wernicke’s zone - understanding language (defect –> receptive dysphasia)

Question 2

Q

Visual field defects

Answer

A

Bitemporal hemianopia = compression at optic chiasm

Presentation: “I didn’t notice the bus coming”, “I didn’t see the car when I walked across the road” –> peripheral vision is gone
Causes:
- Craniopharyngioma - compress from above
- Pituitary adenoma - compress from below

Quadrantonopias:

Causes:
- Superior = temporal lobe lesion
- Inferior = parietal lobe lesion
- NOTE: use inverse rule - superior means that it is affecting the bottom of retina and temporal lobe is below the parietal lobe

Question 3

Q

Neuroanatomy - blood supply to the brain

Answer

A

Circle of Willis - perfuses brain

Cerebral arteries - anterior, middle, posterior (all bordering the circle)
- ACA - supplies anterior & medial brain (frontal lobe + medial motor & somatosensory cortices) –> behavioural changes, weakness legs > arms, mild sensory defect
- MCA - supplies the lateral brain (lateral motor & somatosensory cortices, language - Broca & Wernicke’s, optic tracts) –> weakness face > arms > legs, aphasia (expressive/receptive), hemisensory defect, homonymous hemianopia
- PCA - supplies inferior & posterior brain (affecting visual cortex and tracts) –> homonymous hemianopia, visual agnosia/prosopagnosia
Major arteries giving rise to the circle of Willis:
- Vertebral artery (from the spine)
- Internal carotid artery (gives rise to A & M cerebral arteries)

Question 4

Q

UMN vs LMN:

Motorpathway name + route
UMN & LMN lesion causes and signs

Answer

A

Corticospinal tract = voluntary motor pathway for body:

Cortex - primary motor cortex (posterior frontal cortex)
Medulla - tracts from PMC converge and cross over = pyramids
Spinal cord - UMN synapases with LMN
Peripheral nerves - LMN innervates muscle

UMN lesions

Causes (anything affecting brain itself): stroke, SOL, MND, MS
Signs: hypertonia, hyperreflexia, spasticity (velocity/direction-dependent), upgoing plantars, pronator drift
NOTE: increased tone/reflexes as -ve feedback loop from the brain is removed

LMN lesions

Causes (affect peripheral nerves): MND, trauma, polio, GBS
Signs: hypotonia, hyporeflexia, muscle atrophy, fasciculations, fibrillations
NOTE: reduced tone/reflexes because no nerve supply

Question 5

Q

Intracranial bleeds - layers of the outer brain? types? Appearance on CT-head? Presentations?

Answer

A

Outer layers of the brain:

Skull > dura mater (adherent to inside of skull) > arachnoid mater > pia mater > brain parenchyma

Types:

Extradural haemorrhage- bleed between dura mater & skull
- Most common = skull fracture of pterion (temple) –> trauma to middle meningeal artery
- Hx: clear trauma –> transient LoC –> lucid interval –> ongoing headache –> reduced consciousness
- Convex (EGG) appearance on CT-head as blood sealed in by dura ± soft-tissue oedema outside skull (trauma site)
- Mx: A-E approach, refer to neurosurgery
  - Monitor GCS = deterioration
Subdural haemorrhage - bleed between dura & arachnoid mater
- Tearing of bridging veins going from outer surface of brain to dura mater
- Common in elderly + alcoholics (both have cerebral atrophy)
- Types: both concave (SICKLE) appearance on CT-head
  - Acute - assoc w/ trauma + blood white (hyperdense) on CT-head
  - Chronic - little/no Hx of head trauma e.g. fall several weeks ago and then not been normal since (elderly) + dark (hypodense) blood on CT-head
  - Reduced consciousness, if severe = focal neurology (esp. if midline shift)
- Mx: A-E, neurosurgery referral
  - Monitor GCS, reverse Warfarin
Subarachnoid haemorrhage - between arachnoid & pia matter
- Caused by an aneurysm (berry) or trauma
- Presentation: sudden-onset worst headache ever, photophobia, neck stiffness
- LP 12hrs after Sx-onset (xanthochromia)
- Mx: A-E approach
  - Monitor GCS and neuro obs
  - Discuss with neurosurgery
  - Other – fluids, monitor Na, nimodipine
- Complications:
  - Vasospasm – presents like a stroke
  - Hyponatraemia – can be SIADH
  - Rebleed - coil ASAP if possible

Question 6

Q

Headache - types? Ix? Mx?

Answer

A

Types:

Tension - band-like, front of forehead, causes: dehydration, stress
Cluster - intense pain behind/around eye (worse @night + lacrimation & ptosis during attack), daily clusters for 4-6wks then nothing for months, M>F
- Mx: 100% O2 + nasal triptans
Migraine - intense/throbbing, focal, aura, photophobia
- Triggers: alcohol, chocolate, cheese, oestrogens (OCP)
- Mx:
  - Acute - simple analgesia, dark room + Triptans (take as soon as Sx start)
  - Prevent - trigger avoidance + propranolol/ topiramate (anticonvulsant, terratogenic) /amitriptytline (TCA)
Meningitis - generalised, moves down neck, neck stiffness + fever + rash (meningococcal)
- Ix: CT-head, LP (type of meningitis), BC (causative organism)
- Mx:
  - Suspected in primary care - IM/IV Benzypenicillin
  - Secondary care - IV Ceftriaxone ± Amoxicillin (infants/>50yrs)
  - If could be encephalitis (seizure/behavioural) - IV Aciclovir
SAH - sudden-onset, worst headache ever ± neck stiffness, can get preceding sentinel bleed (minor), assoc w/ PCKD (Berry aneurysm in Circle of Willis)/Marfan’s
- Ix: non-contrast CT –> LP for xanthochromia (after 12hrs)
- Mx: nimodipine (CCB, give every 4hrs, reduces vasospasm in cerebral arteries)
Giant cell (temporal) arteritis - vasculitis –> scalp tenderness, jaw claudication, vision loss, assoc w/ polymyalgia rheumatica (>50yrs)
- Ix: ESR, temporal artery biopsy
- Mx: high-dose Prednisolone (to prevent blindness, do not wait for biopsy)
Trigeminal neuralgia - electric shock-like pain on touching face (extremely tender)
- Divisions of trigeminal nerve (V2&3)
- Ix: MRI brain
- Mx: Carbamazepine (for pain), neurology referral
Raised ICP - worse at night/on waking/coughing/straining ± N&V, visual disturbances
- Mx: osmotic diuresis - mannitol/hypertonic saline
Cerebral Venous Sinus Thrombosis (VTE in the brain) - papilloedema, 6th nerve palsy, seizures, Cushing’s reflex (HTN, bradycardia, Cheyne-Stokes breathing)
- Ix: MRI w/ MR venography
- Mx: LMWH
Idiopathic intracranial HTN (IIH) - Obese, female, papilloedema, postural headache worse with straining, assoc w/ OCP
- Ix: visual field testing, fundoscopy, MRI brain, LP (≥25 opening pressure)
- Mx: acetazolamide (carbonic anhydrase inh), weight loss, LPs + VP/LP shunt, ophthalmology follow-up

Question 7

Q

Cardiovascular accident (CVA) - Def? Presentation? DDx? Classification? Ix? Mx?

Answer

A

Def: better to use cardiovascular accident (CVA)

Stroke ≥24hrs, TIA ≤24hrs

Presentation:

Pyramidal weakness (flexors stronger than extensors in upper limb, opposite in lower limb), hypertonia, hyperreflexia
Hemiplegic/scissor gait –> circumduction
TIA: Amaurosis Fugax (black curtain)
- If ≥2 in 1wk = high risk of stroke –> ADMIT
- Aspirin 300mg + secondary prevention (as for stroke)

DDx:

Cerebral lesion - 80% ischaemic, 20% haemorrhagic (reduced GCS & raised ICP)
Neoplastic - SOL
Rare: syphilis, Todd’s paralysis (post-ictal), migraine, MS, cerebral abscess, rheumatological (arteritis)

Classification: Bamford aka Oxford

Total anterior circulation stroke: hemiplegia, homonymous hemianopia, higher cortical dysfunction
Partial anterior circulation stroke: 2/3
Lacunar circulation stroke: hemi-motor OR hemi-sensory stroke only
Posterior stroke: cranial nerve problems, eye-movement disorder, cerebella presentation
- Weber’s & Wallenberg’s (lateral medullary syndrome = posterior cerebellar artery infarct)

Ix:

BEFORE Tx = CT-head - 80% strokes ischaemic, 20% haemorrhagic (wrong Tx will make it much worse) –> haemorrhage has an area of hyperdense (white) bleeding
After initial Mx –> identify cause:
- Structural heart defect - echocardiogram
- AF - ambulatory ECG (24hr tape if no obv sign of AF as outpatient)
- Carotid atherosclerosis - carotid doppler –> carotid artery stenosis (70-99%) = carotid endarterectomy

Mx: A-E approach + URGENTLY contact stroke team –> dedicated stroke unit, if ischaemic:

Immediately:
- Aspirin 300mg OD + STOP anticoagulants (high risk of haemorrhagic transformation in first 2wks)
- <4.5hrs since Sx onset: thrombolysis (Alteplase = tPA)
  - C/I: any sign of active bleeding, very high BP, recent surgery, raised ICP
- >4.5hrs since Sx onset: conservative Mx:
  - BM - keep controlled <11 (sliding scale insulin)
  - NG tube (nutrition)
  - MDT - dietician, SALT, PT/OT
- < 6hrs (sometimes up to 24hrs, depending on size, area, damage) –> thrombectomy (neurosurgery)
After 2wks:
- STOP aspirin –> start clopidogrel 75mg OD + consider anticoagulation (e.g. if AF) - DOAC/Warfarin
- Manage vascular RFs (DM, HTN, QRISK etc.)

Question 8

Q

Neuroanatomy - motor function overview

Answer

A

Primary motor cortex –> pyramidal tracts = VOLUNTARY - include:

Corticobulbar tracts - head & neck voluntary movement
Corticospinal tracts - body voluntary movement

Extrapyramidal tracts = INVOLUNTARY - basal ganglia, cerebellum, vestibular nucleus

NOTE: pyramidal = GAS (starts movement), extrapyramidal = CLUTCH (smooths movement)

Question 9

Q

Parkinson’s disease definition? Cardinal Sx? DDx? Ix? Mx? Complications?

Answer

A

Def: a neurodegenerative disease of dopaminergic neurones of substantia nigra (part of basal ganglia in the midbrain)

Cardinal Sx (from extrapyramidal involuntary dysfunction): resting tremor, rigidity (cogwheeling due to superimposed tremor), bradykinesia, postural instability

+ insomnia, hypomimia (mask face), depression, autonomic dysfunction

DDx:

Benign essential tremor - bilateral, postural, FHx, alcohol improves it (in 50%)
Drug-induced (anti-psychotic, metoclopramide) - drug chart
MSA (multisystem atrophy) incl Shy-Drager (autonomic) - cerebellar signs
PSP (progressive supranuclear palsy) - loss of downward gaze
LBD (Lewy body dementia) - visual hallucinations
Normal-pressure hydrocephalus - gait disturbance, cognitive impairment, impaired bladder control
Other:
- Corticobasal degeneration - single-arm signs, alien limb syndrome (myoclonus)
- Wilson’s disease (copper) - motor

Ix: clinical Dx –> dopaminergic agent trial ± MRI scan/DAT scan

Mx: problem = lack of dopamine in substantia nigra –> aim of Tx is to increase dopamine here

Dopamine agonist (cross BBB):
- Good for younger patients to reduce time on levodopa (as get very sensitised)
- Types:
  - Non-ergot - ropinirole, pramipexole - avoid below SEs = 1st line but still get dopamine dysregulation syndrome (hypersexuality & gambling)
    - SC Apomorphine for advanced disease
  - Ergot - cabergoline, bromocriptine –> retroperitoneal/pulmonary fibrosis
‘L-DOPA (levodopa) AND peripheral DOPA-decarboxylase enzyme (Carbidopa)’ = Sinemet/Medapar
- Relevant physiology:
  - Substantia nigra contains dopaminergic neurones with DOPA decarboxylase (converts L-DOPA –> dopamine) - not happening as normal in Parkinson’s
  - Peripheral DOPA-decarboxylase in body
  - Chemoreceptor trigger zone (CTZ) –> triggers nausea & vomiting
- Drug explanation:
  - If give L-DOPA by itself it will be broken down by peripheral DOPA-decarboxylase into dopamine - this can’t cross BBB to reach substantia nigra but can reach CTZ –> nausea/vomiting
  - Instead, give L-DOPA with peripheral DOPA-decarboxylase inhibitor to allow more L-DOPA to cross BBB to reach dopaminergic neurones in substantial nigra to be converted to dopamine (some still reaches CTZ –> SE of nausea/vomiting)
- Other SEs: dyskinesia, on-off phenomena, postural hypotension
  - Amantadine - for dyskinesia secondary to levodopa
  - COMT inhibitors e.g. entacapone for tailing off betw/ doses of levodopa

Complications:

Falls, cognitive impairment, depression
Drool (SALT/glycopyrronium)
Meds SEs (e.g. vomiting)

Question 10

Q

Rigidity vs Spasticity

Answer

A

Rigidity (in Parkinson’s disease) = increased muscle tone, that is not velocity or direction-dependent

In Parkinson’s get Cogwheeling = rigidity overlayed by tremor

Spasticity (in Stroke) = increased muscle tone, that is velocity or direction-dependent

Flexors/extensors become imbalanced in strength - certain directions have increased muscle tone (not all) AND worse stiffness if move arm more quickly

Question 11

Q

Dementia - Types?

Answer

A

Types:

Alzheimer’s disease
- Progressive decline in cognitive function
- Tx: Acetylcholinesterase inhibitors (Donepezil)/NMDA receptor antagonists (Memantine)
Vascular dementia
- A stepwise decline in cognitive function
- Background of vascular disease (IHD, PVD)
Dementia with Lewy Bodies
- Triad - dementia, hallucinations, parkinsonism
- Balance - Parkinsonism = dopamine def vs hallucinations = dopamine excess
Frontotemporal - personality changes, early onset
Depressive pseudodementia - disinterested, low mood

Question 12

Q

CSF appearance for meningitis, TB, SAH, GBS

Question 13

Q

Neuro examination Qs:

Good acronym for general inspection?
What screens do you do for upper and lower limbs?
What are you feeling for on examining tone?
What to do before checking power against resistance?
How to find the tendon for each reflex?
How do you accentuate neuro reflexes?
What to remember when doing the finger-to-nose test?

Answer

A

SWIFT - scars, wasting, involuntary mov, fasciculations, tremors
Screens:
- Upper - hands out pronate (tremor?), supine hands and close eyes (pronator drift = UMN lesion)
- Lower:
  - Stand up from chair with hands crossed (prox power)
  - Gait assessment - normal, only on heels/toes (distal power), tandem walk (balance)
  - Stand with both feet together (balance) –> Romberg’s test if they could do prior (eyes closed - proprioception).
Tone:
- “Clasp-knife spasticity” = pyramidal (UMN) lesion - velocity/direction-dependent e.g. stroke/spinal cord compression/MND
  - CLONUS also present in UMN lesion
- Rigidity = extrapyramidal lesion - velocity/direction independent, types:
  - “Lead-pipe” = NMS (anti-psychotics)
  - “Cogwheeling” = Parkinson’s (tremor on rigidity)
Check power against gravity - raise arms/legs (if so MRC is 3+)
Eliciting reflexes:
- Biceps - thumb over cubital fossa (from lateral side) while they move hand towards shoulder (should be central) - place nail over tendon and hit nail. Get patient to rest hand on knee
- Brachioradialis - first 2 fingers 1/3 up arm from wrist, lateral arm. Get patient to rest hand on knee
- Triceps - hold out patient arm at 90 degrees takin the weight of the patient’s arm, stand behind patient, hit just above the olecranon
- Knee jerk - if patient supine, support behind knee
- Ankle jerk - if patient supine, cross one leg over the other, dorsiflex foot, strike ankle tendon OR keep straight, dorsiflex foot and strike ball of the foot (just below ball under toes)
Jendrassik maneuver - clench hands together/clench jaw at the same time as eliciting reflex
Put finger just beyond reach so they have to stretch

Question 14

Q

What are the nerve innovations for deep tendon reflexes in upper and lower limb neuro examination?

Answer

A

Nerve innovations:

S1,2 - buckle my shoe - ankle jerk (Achilles tendon)
L3,4 - kick the door - knee jerk (patellar tendon)
C5,6 - pick up sticks - biceps, brachioradialis reflex
C7,8 - lay them straight - triceps reflex

Question 15

Q

Cerebellar examination Qs:

Acronym for cerebellar signs?
What are the causes of cerebellar disease?
Key aspects of cerebellar assessment?

Answer

A

DANISH - Dysdiadokokinesia, Ataxia, Nystagmus, Intention tremor, Slurred speech (scanning dysarthria), Hypotonia
PASTRIES
- Paraneoplastic syndrome, Alcohol, Sclerosis (MS), Tumour, Rare (Friedrich’s ataxia), Iatrogenic (Phenytoin, carbamazepine), Endocrine (hypothyroid), Stroke (posterior circulation)
- Friedrich’s ataxia - inherited, young males, deep tendon reflexes absent, Babinski +ve, distal weakness, dorsal column disease (vibration, proprioception), cardiac (cardiomegaly, heart murmurs)
Cerebellar assessment:
- General inspection for wheelchair, posture, neglect (alcohol)
- Gait - sit to stand arms folded (truncal ataxia), tandem walk (ataxic gait), feet together stand –> Romberg’s test (sensory ataxia, -ve in cerebellar disease)
- Face - H-test (nystagmus, hypometric saccades - fast hand mov), move tongue side to side (SOL), say British Constitution/Baby Hippopotamus (slurring)
  - Cerebellar pathology - fast saccades towards the side of the lesion, worse looking towards the lesion
  - Vestibular nystagmus - fast away from lesion, slow back
  - Head tilt test – eyes should remain in same position but if vestibular lesion eyes move with tilt (if cerebellar – vestibulocochlear reflex intact)
- Focussed upper limb:
  - Tone (hypotonia) + pronator drift assessment (pronation = UMN lesion, upward drift = cerebellar lesion) + rebound test (push wrists down quickly)
  - Coordination - finger-to-nose test (intention tremor, past pointing), dysdiadokokinesia
- Focussed lower limb - tone (hypotonia), coordination (heel-shin test)
- To complete exam: fundoscopy (papilloedema - SOL), full neuro exam and cranial nerves exam (5,7,8 - trigeminal, fascial, vestibulochoclear –> cerebellopontine angle lesion)

Question 16

Q

Key aspects of Parkinson’s examination?

Answer

A

Parkinson’s assessment:

General inspection for tremor (close eyes count backward from 20), walking aids
Gait - shuffling, hesitant (hard to start/turn), lack of arm swing, unsteadiness, stooped posture
Face - hypomimia, glabellar reflex (ask to keep eyes open while tapping forehead if blink lots = +ve), ask to say British constitution - hypotonia
Focussed upper limbs - tone (cogwheeling, rigidity), bradykinesia (snap hands like crab, piano fingers)
Focussed lower limbs - bradykinesia (heel tap)
Extra:
- Write name (micrographia), draw spiral (tremor)
- Eye movement - conjugate gaze disorder:
  - Up/down (progressive supranuclear palsy (PSP) - vertical limitation = DDx)
  - Left to right (nystagmus - multisystem atrophy (MSA) = DDx)
To finish exam: cerebellar signs (MSA), postural BP (MSA), MMSE (LB dementia), drug chart (antipsychotics)

Question 17

Q

End of the bed observations Dx?

Question 18

Q

MRC Power Scale

Answer

A

1 flicker
2 is moves with gravity removed
3 is movement against gravity
4 is reduced power against resistance
5 is normal

Question 19

Q

Gait abnormalities vs normal

Answer

A

SUMMARY:

Normal gait cycle: 1) heel strike 2) foot flat 3) mid stance 4) heel off 5) toe-off
Antalgic gait - limping due to pain e.g. osteoarthritis, inflammatory joint disease, fracture, sciatica
Hemi/Diplegic gait - uni/bilateral circumduction of leg - CNS lesion (bilateral is called a ‘scissoring gait’)
Parkinsonian gait - shuffling gait, no arm swing - Parkinson’s/DLB/Antipsychotics
Ataxic gait - wide/broad-based stance & imbalance - Cerebellar/Vestibular/Sensory neuropathy
- NOTE: can’t do tandem walk
Neuropathic gait - high-step gait (from foot drop) - motor neuropathy e.g. L5 radiculopathy (e.g. herniated disc), common peroneal neuropathy, MS, Charcot-Marie-Tooth disease
- Can’t walk on heels
Myopathic gait - waddling/Trendelenburg gait (hip sways to one/both sides - hip abductor muscles weak) - muscular dystrophy/myopathy
Choreiform gait - involuntary movements - Basal ganglia disease e.g. Parkinson’s meds, Huntington’s, Wilson’s, Cerebral Palsy
Stomping gait - sensory neuropathy (loss of proprioception & vibration sense = dorsal column disease)
- Vibration –> proprioception (Romberg’s +ve) –> light touch (in order lost)
- Causes: SACD (subacute combined degen cord - B12), Friedrich’s ataxia, Tabes/taboparesis & tabes dorsalis (syphilis),
Marche à petit pas - small steps = normal pressure hydrocephalus

IN-DEPTH:

Normal gait cycle: 1) heel strike 2) foot flat 3) mid stance 4) heel off 5) toe off

Antalgic gait - limping due to pain

Causes: osteoarthritis, inflammatory joint disease, lower limb fracture, nerve entrapment (sciatica)

Hemiplegic gait - unilateral circumduction of leg to prevent dragging foot

CNS lesion –> unilateral weakness & spasticity (spastic flexion of upper limb vs extension of lower limb)
Causes - UMN lesion:
- Unilateral cerebral lesion e.g. stroke, SOL, trauma, MS
- Hemisection of spinal cord (trauma)

Diplegic gait - bilateral circumduction of legs (scissoring gait_)_

CNS lesion as above but bilateral clinical findings - spasticity worse in lower limbs vs upper limbs ± hyperreflexia, clonus, Babinski +ve, reduced power, sensory level if spinal cord pathology
Causes:
- Spinal cord lesion e.g. prolapsed intervertebral disc, spinal spondylosis, spinal tumour/infarct, transverse myelitis
- Bilateral brain lesion - cerebral palsy, MS, bilateral brain infarcts, midline tumour (paraspinal meningioma)
- MND - if also LMN findings (wasting, fasciculations)

Parkinsonian gait - shuffling gait

Loss of dopaminergic neurones in substantia nigra of basal ganglia –> extrapyramidal dysregulation = rigidity, bradykinesia, resting tremor, postural instability
Features of parkinsonian gait:
- Initiation - slow to start walking
- Step length - shuffling gait (short steps) & festinant gait (progressively smaller steps)
- Arm swing - reduced (early feature)
- Posture - flexed trunk & neck = stooped
- Turning - postural instability
Causes: Parkinson’s, Dementia w/ LBs, Parkinson’s plus syndromes (Multisystem atrophy & progressive supranuclear palsy), drug-induced (antipsychotics/emetics)

Ataxic gait - wide/broad-based stance (to maintain balance)

Assoc w/:
- Midline cerebellar disease (alcoholism/B12 def, MS, cerebellar stroke) - DANISH (dysdiadokokinesia, ataxia, nystagmus, intention tremor, slurred speech, hypotonia)
- Vestibular disease (labyrinthitis, Meniere’s, acoustic neuroma) - vertigo, N&V
- Sensory ataxia (peripheral neuropathy e.g. DM) - +ve Romberg’s, impaired proprioception, impaired vibration sense, lack of other cerebellar signs
Features of ataxic gaint:
- Stance - broad-based ataxic gait
- Stability - staggering, slow, unsteady –> veer towards side of lesion
- Turning - very difficult

Neuropathic gait - high-step gait (from foot drop)

Weakness in muscles of distal limb (foot dorsiflexors) - damage to peripheral nerves providing motor innervation –> foot drop & dragging toes –> knee & hip flex excessively to compensate
Other features: peripheral vascular disease, peripheral sensory impairment, reduced/absent reflexes
Causes: motor neuropathy
- Isolated common peroneal nerve palsy (trauma/compression)
- L5 radiculopathy (disc prolapse)
- Generalised polyneuropathy involving multiple nerves (DM, MND, Charcot-Marie Tooth disease)

Myopathic gait - waddling/Trendelenburg gait (hip sways to one/both sides)

Weakness of hip abductors –> can’t stabilise pelvis –> tilts down towards unsupported side
Assoc features: hard to stand from seat/squat/lying without arms, +ve trendelenburg’s sign (stand on one leg - if the hip on the side of the raised leg drops = +ve)
Causes: muscular dystrophy, myopathy (e.g. in hyperthyroidism, Cushing’s, acromegaly, Polymyalgia rheumatica)

Choreiform gait - involuntary movements

Oro-facial dyskinesia (grimacing/lip-smacking) & choreic mov of upper/lower limbs = dance-like/writhing @rest/mov
Causes: Basal ganglia disease
- Huntington’s, Sydenham’s chorea, Cerebal palsy (Choreiform type), WIlson’s disease, Dopaminergic meds (e.g. Parkinson’s)

Question 20

Q

Post-stroke signs

Question 21

Q

Parkinsonism causes

Question 22

Q

Cerebellar syndrome causes?

Answer

A

KEY:

MS - eye signs (RAPD, INO), spastic paraparesis, catheterised
Alcoholism - peripheral neuropathy, liver signs
Hypothyroidism
Phenytoin & lithium
Coeliac disease

PASTRIES

Paraneoplastic syndrome, Alcohol, Sclerosis (MS), Tumour, Rare (Friedrich’s ataxia), Iatrogenic (Phenytoin, lithium), Endocrine (hypothyroid), Stroke (posterior circulation)
- Friedrich’s ataxia - inherited, young males, deep tendon reflexes absent, Babinski +ve, distal weakness, dorsal column disease (vibration, proprioception), cardiac (cardiomegaly, heart murmurs)

Question 23

Q

MS - def? key features? DDx? Clinical courses? Ix? Mx? Poor prognostic features?

Answer

A

Def: evidence of damage to CNS that is separated in time & space

Key features:

Profile: young, female (catheter & walking aid/wheelchair), eye & cerebellar signs
Initial:
- Ipsilateral visual disturbance - greying/blurring ± pain on eye mov/loss of red colours
- Sensory phenomena - wetness/burning, uncomfortable band around the chest
  - Lhermitte’s sign - electric shock down neck and along spine & may radiate along limbs
  - Uthoff’s - neuro Sx worsen with increased temperature (hoff = hot)
UMN Sx: hyperreflexia, hypertonia, pyramidal pattern (arm flexors stronger, leg extensors stronger)
Dorsal column disease - reduced vibration, proprioception, Romberg’s +ve
Eyes:
- Internuclear ophthalmoplegia
  - Adduction paralysis and abduction nystagmus
  - From lesion in medial longitudinal fasciculus
- Optic nerve damage:
  - Colour & visual acuity loss, RAPD
  - Central scotoma (central blindspot), optic atrophy
- CN palsy (most commonly 6th - as longest)
Cerebellar signs (imbalance, can’t tandem walk)

DDx:

Cervical spondylosis - wo/ UMN signs but no cerebellar signs
Subacute combined degeneration of the cord (SACD) - dorsal columns & cerebellar - if low B12
Neuromyelitis optica aka Devic’s disease (IgG against aquaporin-4 channel) - optic neuritis & transverse myelitis but no cerebellar Sx

Clinical courses/patterns:

Relapsing-remitting - steady decline + strong flair
Primary progressive - steady decline without attacks
Secondary progressive - initial relapsing-remitting becoming primary progressive
Marburg variant - very severe, rapidly progressive

Ix:

Bedside:
- Fundoscopy (optic neuritis), full functional assessment (physio, OT)
- Nerve conduction studies - visual evoked potentials (VEPs) - if single nerve lesion, delayed response
Bloods - B12 (SACD), TFTs
Imaging:
- MRI (T2) head (lesions disseminated in time & space) - McDonald’s criteria
  - Periventricular white matter lesions
- LP: IgG oligoclonal bands, high protein

Mx:

Acute relapse: high-dose methylprednisolone (shortens acute attack) e.g. optic neuritis (painful eye mov + reduced visual acuity)
Long-term - MDT approach (physio, OT, SALT)
- Mobility - mobility aids, physio, OT
- Disease-modifying meds e.g. INF-beta, Fingolimod
- Spasticity –> Baclofen/Gabapentin (Dantrolene if CKD)
- Neuropathic pain –> anti-convulsant e.g. amitryptiline/gabapentin
- Bowel (laxatives) & bladder (oxybutynin, LT catheter)
- Fatigue –> Amantadine

Poor prognostic features:

Brainstem/cerebellar disease at onset
>40yrs at onset
Primary progressive MS (no resolution

Question 24

Q

Bell’s palsy - def? presentation? Ix? Mx?

Answer

A

Def: acute, sudden-onset, unilateral facial palsy of probable viral aetiology

Presentation: URTI –> unilateral facial palsy (all branches, no-forehead sparing) with pain behind the ear & dry eye

Bell’s sign: failure of eye closure (dryness and conjunctivitis)
Precipitating factors (e.g., dental extraction, upper respiratory tract infection)
Single episode (recurrence = likely alternative Dx)
Unilateral (bilateral = likely alternative Dx e.g. Lyme disease, GBS, sarcoidosis, leukaemia)
Involves all nerve branches (originates proximal to the geniculate ganglion so should be equal distribution)
No constitutional Sx (would indicate Lyme disease, AI, granulomatous disease)
Keratoconjunctiva sicca (dry eye - from inadequate blinking)
Pain behind the ear

Ix: clinical Dx (Dx of exclusion)

Electroneurography (ENoG, 72hrs-14 days after palsy starts)
Needle electromyography (>90% degeneration in CMAP)
Serology for Borrelia burgdorferi (if recent visit to Lyme disease-endemic region)

Mx:

Pred (if <72hrs Sx onset)
Eye protection (to prevent keratoconjunctivitis sicca progressing to exposure keratopathy)
Severe palsy/complete paralysis –> concurrent antiviral therapy (aciclovir), surgical decompression
Prognosis = 75% make a full recovery

Question 25

Q

Neuro station - exam observations with assoc Dx

Answer

A

Circumducting gait, flexed arm –> hemiplegia, stroke
Shuffling gait –> Parkinson’s
Ataxic gait –> Cerebellar syndrome, MS
Facial palsy –> Bell’s palsy, Stroke
Mixed signs –> MND

Question 26

Q

Headache Red Flags? Ix?

Answer

A

New severe headache
Progressive/persistent headache
Morning vomiting
Worse on lying down
Focal neuro signs

CT-head

Question 27

Q

Clinical Dx of migraine? Mx? What is C/I in asthmatics?

Answer

A

Headache lasting 4-72hrs

with ≥2: Unilateral, throbbing, aggravated by daily activities
with ≥1: N/V, photophobia, phonophobia

Asthma –> no NSAID or propranolol

NOTE: topiramate requires reliable contraception in women of child-bearing age

Question 28

Q

Visual hallucination + macular degen - Dx?

Answer

A

Charles Bonnett syndrome

Question 29

Q

Campylobacter, ascending polyneuropathy, anti-GM1 - Dx?

Question 30

Q

Spinal cord lesion, normal MRI, anti-aquaporin-4 +ve —> Dx?

Answer

A

Neuromyelitis optica

Question 31

Q

Wernicke’s encephalopathy - Def? Sx triad? Mx - in hypoglycaemia and without? Complication?

How does delirium tremens present?

Answer

A

Def: Acute presentation of thiamine (B1) def

Triad: confusion, ataxia, nystagmus

Complication = Korsakoff syndrome (chronic & permanent memory problems)

Delirium tremens: confusion, visual hallucinations, tachycardia, pyrexia

Question 32

Q

Coarse tremor, confusion, hallucinations with Hx of alcohol excess - Dx?

Answer

A

Delirium tremens

Question 33

Q

Alcohol excess, MA, ketosis - Dx?

Answer

A

Alcoholic ketoacidosis

Question 34

Q

Disulfiram (promotes alcohol abstinence) - MoA?

Answer

A

Inhibits acetaldehyde dehydrogenase

Question 35

Q

Anterior spinal artery syndrome overview

Answer

A

Anterior spinal artery syndrome caused by occlusion - involves anterior 2/3 of spinal cord

RFs: atherosclerosis, hypotension, aortic aneurysm/dissection

Presentation:

Complete motor paralysis and loss of temperature and pain perception distal to the lesion.
Since posterior columns are spared, light touch, vibration, and proprioceptive input are preserved

Question 36

Q

Spinal cord injury - RFs? Ix? First Mx?

Answer

A

RFs: atherosclerosis, hypotension, aortic aneurysm, aortic dissection

Ix:

Traumatic - CT-spine (bony injury/fracture)
Non-traumatic - MRI spine (soft tissue injury)

Mx: spinal immobilisation, manage in tertiary neurosurgical centre

Question 37

Q

“Cape-like” distribution of pain + temp loss - Dx?

Answer

A

Syringomyelia

Question 38

Q

Sensory level L1-2, upgoing plantars, absent knee reflexes - Dx?

Answer

A

Conus medullaris lesion

Question 39

Q

VDRL +ve, loss of proprioception/vibration - Dx?

Answer

A

Tabes dorsalis (tertiary neurosyphilis)

Question 40

Q

Subdural - appearance on CT-head

Answer

A

Crescenteric haematoma

Question 41

Q

What is Cushing’s triad? Indicates? Mx?

Answer

A

HTN, bradycardia, Cheyne-Stokes breathing

Indicates raised ICP (likely form herniation through foramen magnum)

Urgent referral to tertiary neurosurgical centre

Question 42

Q

Fall in elderly patient on warfarin - Dx?

Answer

A

Acute Subdural haematoma - consider prothrombin complex if INR raised

Question 43

Q

Most common cause of SAH?

High SAH suspicion but normal CT - Ix?

How do you reduce risk of ischaemia afterwards?

Answer

A

Berry aneurysm (85%)

LP in 12hrs for xanthochromia (yellow from bilirubin in CSF - RBC breakdown)

Nimodipine

Question 44

Q

Fasting beating nystagmus to left + left-sided dysdiadochokinesia - where is the stroke?

Answer

A

Left cerebellum (ipsilateral in cerebellum lesions)

Question 45

Q

What is vertigo? Vertigo Hx & general Tx? Breakdown? Presentation? Ix? Mx?

Answer

A

Def: The sensation of movement between person & environment: spinning sensation, N&V

Vertigo Hx:

Vertigo (sense of motion) vs light-headed vs pre-syncope
Assoc hearing loss? If yes = not BPPV
- Meniere’s disease - vertigo, tinnitus, hearing loss –> no driving
- Viral labyrinthitis - following URTI
Tx:
- Vertigo - prochlorperazine (reg for 3 days)
- Tinnitus - betahistine

Detailed breakdown:

Peripheral cause:
- BPPV - crystals displaced in SCC
  - sudden, brief, episodic, only on certain head movement
  - Ix: Dix-hallpike test to confirm (up-beat torsional nystagmus)
  - Mx:
    - Education & reassurance (remission from 3wks - 6 months)
    - Epley manoeuvre
    - Betahistine (for vertigo)
- Meniere’s disease - excessive endolymph in SCC
  - Clustered attacks <12hrs: Fluctuating hearing loss, tinnitus, vertigo lasts minutes- hours (more than one episode)
  - Ix:
    - +ve Romberg’s test
    - Pure-tone & speech audiometry
    - Tympanometry
    - Otoacoustic emissions (OAE)
  - Mx: dietary changes + lifestyle modification (limit salt, caffeine, alcohol/smoking, stress) ± thiazide diuretic (triamterene/hydrochlorothiazide)/acetazolamide
    - Vertigo - betahistine
    - Tinnitus - devices
    - Hearing loss - CS
- Labyrinthitis - inflammation of otic capsule caused by viruses/bacteria affecting inner ear (cochlear & vestibular system)
  - Severe vertigo, nystagmus and vomiting following an URTI
    - Bacterial - severe hearing loss (irreversible)
    - Viral - less severe Sx (often reversible)
  - Ix: cranial nerve exam (Renne’s, Weber’s), audiometry (sensorineural hearing loss)
  - Mx:
    - If severe: IV prochlorperazine
    - If less severe: PO cyclizine and prochlorperazine
    - Tx cause e.g. bacterial
    - Ongoing - vestibular rebab (physio, OT)
  - NOTE: vestibular neuronitis is inflammation of the vestibular nerve = the same presentation without the hearing loss (normal audiogram) - comes on acutely but can take weeks to settle
    - Ix & Mx - as above
Central cause:
- Stroke/TIA - lateral medullary syndrome (Wallenberg syndrome), cerebellar infarct/haemorrhage
  - Cerebellar stroke - headache (occipital sudden & prominent), N&V, ataxia (imbalance)
- Acoustic neuroma (vestibular schwannoma) - benign cerebellopontine angle tumour
  - Unilateral sensorineural hearing loss, facial numbness, progressive intermittent unsteadiness
  - Can have co-existing neurofibromatosis type 2
  - Ix: audiometry, (CT head (can be missed if small)) –> gadolinium-enhanced MRI (absence of dural tail)
  - Mx:
- Vestibular migraine - headache, photophobia, phonophobia, aura
- Vertebrobasilar ischaemia - reduced flow to posterior circulation of brain
  - Head position provokes vertigo
  - Hemifacial numbness & contralateral peripheral weakness/paraesthesia ± arm claudication (from subclavian steal)
- MS

Question 46

Q

Neurology Ix depending on UMN/LMN signs?

Answer

A

ALL: involve MDT for full functional assessment (physio & OT)

UMN:

Imaging (brain ± spinal cord)
CSF (LP)
Brain biopsy

LMN:

Nerve conduction studies & electromyography (NCS & EMG)
Bloods (metabolic, abs)
Muscle/nerve biopsy

Question 47

Q

What do crossed signs (e.g. right facial weakness but left leg weakness) indicate?

Answer

A

Brainstem lesion (if peripheral weakness is left then lesion on right)

Question 48

Q

GBS Ix? Mx?

Answer

A

Ix:

Bloods (metabolic, abs), CT –> LP (high protein, normal WCC) ± MRI spine (exclude other causes)
Nerve conduction studies & EMG (detects if demyelinating/affecting axon, conduction velocity reduced)

Mx: ADMIT suspected cases - normally self-limiting course

MDT - psychologist, SALT (if speech muscles affected)
Monitor FVC <20ml/kg, NIF <30cm H2O –> intubate + mechanical ventilation
Monitor BP –> IV fluids for hypo, Labetalol for hyper
High-dose IVIG, plasmapheresis (plasma exchange)

Question 49

Q

MND - Presentation? DDx? Types of MND? Dx? Mx? Complications?

Answer

A

Presentation: mixed UMN/LMN (no sensory deficits, no eye involvement)

UMN in lower limbs (spasticity, hyperreflexia, upgoing plantars)
LMN in upper limbs (hypotonia, hyporeflexia, fasciculations, wasting)
Bulbar/pseudo-bulbar tongue/speech (tongue fasciculations, palatal paralysis, nasal speech)
NORMAL SENSORY EXAM

DDx mixed signs:

Dual pathology (stroke + peripheral neuropathy)
Conus medullaris lesion - painful, assoc with traumatic spinal injury/higher up mets)
B12 def (subacute combined degen of spinal cord) = cofactor in myelination (as is folate)

Types:

Amyotrophic lateral sclerosis (60%) - classic (UMN & LMN)
Progressive Bulbar Palsy (30%) - CN 9-12 –> dysarthria (speech), dysphagia (swallowing)
Primary Lateral Sclerosis - pure UMN onset
Progressive Muscular Atrophy - pure LMN onset, ‘flail limb’ appearance

Dx: clinical, exclude other causes

EMG/NCS (nerve conduction study) - chronic nerve root denervation
Imaging - MRI (cervical, thoracic, lumbar spine) - exclude other causes

Mx: no cure - MDT approach

Conservative - SALT (swallowing), physio & OT
Sx-control:
- Quinine - muscle cramp
- Anticholinergics e.g. hyoscine patches - drooling
- Exercise & nutrition
Prognostic:
- Riluzole - glutamate antagonist (extend life expectancy by 3-5 months)
- NIV - if respiratory muscles no longer functioning

Complications: resp compromise, frontal lobe dementia (2%)

Question 50

Q

Most common meningitis organisms by age

Answer

A

Baby - E. coli

5yrs - H. influenzae

15-25yrs - N. meningococcus

>60yrs - S. pneumo

Question 51

Q

Carpal tunnel syndrome - key sign?

Answer

A

Tinel’s sign - tap over flexor retinaculum = tingling

Question 52

Q

How to remember nerve innervation of the diaphragm? Nerve associated?

Answer

A

C3,4,5 keep the diaphragm alive

Phrenic nerve

Question 53

Q

Raised ICP - what is the Monro-Kellie Doctrine? Causes? Signs? Mx?

Answer

A

Monro-Kellie Doctrine: skull is a closed box, the sum of volumes of brain, CSF & IC blood is constant - increase in one should cause a decrease in one/both of others

In brain there is little wiggle-room, the only weak point is the foramen magnum –> herniation = coning –> death

Causes: IC haemorrhage (IC blood), tumour, (brain vol), cerebral oedema (CSF)

Signs:

Acute (pressure on brainstem): CUSHING’S TRIAD (HTN, bradycardia, irregular breathing) + reduced GCS
Chronic: long-term headache

Mx:

Conservative: sit up, hyperventilated (if intubated –> reduce pCO2 –> reduce vasodilation in brain)
Medical: mannitol (osmotic diuretic), hypertonic saline –> both draw fluid out of brain –> reduce ICP
URGENT neurosurgical input –> Burr hole surgery

Question 54

Q

Cauda equina - def? Sx? Causes? Key Ix? Mx?

Answer

A

Def: compression of Cauda Equina (nerve fibres below L1-2)

Sx: can you feel it while you urinate/when you tug on catheter?

Severe back pain
Saddle anaesthesia (numb around the anus)
Bladder/bowel dysfunction (urinary retention, faecal incontinence)
Lower limb weakness
Reduced anal tone (on PR exam)

Causes: large disc herniation, cancer, trauma, abscess, haematoma

NOTE: if likely mets e.g. background of prostate cancer –> 16mg Dexamethasone (reduces swelling)

Ix: urgent MRI scan (+ PR exam)

Mx:

In normal disc herniation (above cauda equina):
- Get the patient to keep moving (or muscles will seize up)
- Analgesia - PR Diclofenac (neuropathic pain) + Diazepam
In cauda equina –> urgent referral to neurosurgery for decompression/laminectomy

Question 55

Q

What should I not forget in upper limb neuro exam?

Answer

A

Upper limb:

Pronator drift (palms up)
Power - stabilise joint, chicken, boxer, arms out straight, , separate fingers separate + hold paper, thumb straight up in air (abduction)
Reflexes - nerve innovations:
- Biceps/brachioradialis - C5/6 - pick up sticks
  - Brachioradialis - feel for radial tubercle then go several cm lower
- Triceps - C7/8 - lay them straight
Coordination - finger-nose test (put elbow out like chicken) & dysdiadokokinesia
Sensation - dorsal column: “does this feel normal?”
- Fine-touch - glove & stocking or dermatomal:
  - C5 - deltoid area
  - C6 - thumb
  - C7 - middle finger
  - C8 - little finger
  - T1 - medial forearm
  - T2 - medial upper arm
- Vibration - 128Hz tuning fork (IPJ thumb, hit on elbow to trigger, ask patient to say yes when it stops)
- Proprioception (hold either side of thumb)
Sensation - spinothalamic:
- Pain - neurotip
- Temperature - prongs of tuning fork (sternum then dorsum of hand)
Sensation pathology:
- Radiculopathy (dermatome - nerve root)
- Glove & stocking - peripheral neuropathy e.g. DM

Question 56

Q

What bits not to forget in lower limb neuro exam?

Answer

A

Lower limb:

Gait & Romberg’s test
Tone - clonus
Power - stabilise joint (contralateral-side if hip)
Nerve innovations of reflexes:
- Knee - L3/4 - kick the door
- Ankle - S1/2 - in the shoe
Babinski reflex
Coordination = heel-shin test
Sensation - dorsal column:
- Fine-touch - glove & stocking or dermatomal: “does this feel normal?”
  - (L1 - higher upper inner thigh)
  - L2 - upper inner thigh
  - L3 - medial just above knee
  - L4 - medial upper calf
  - L5 - lateral calf
  - S1 - plantar aspect of the foot (sole)
- Vibration - 128Hz tuning fork (IPJ big toe, hit on elbow to trigger, ask patient to say yes when it stops)
- Proprioception (hold either side of big toe)
Sensation - spinothalamic:
- Pain - neurotip
- Temperature - prongs of tuning fork (sternum then dorsum of foot)
Sensation pathology:
- Radiculopathy (dermatome - nerve root)
- Glove & stocking - peripheral neuropathy e.g. DM

Question 57

Q

Patient with severe chest, neck and back pain + tingling in the hand and bruising around the right eye - what Ix need to be done?

Answer

A

CT head (for possible IC bleed)

CXR (for chest pain - rib fracture)

CT scan of the neck (history suggests cervical spine fracture)

Question 58

Q

Spinal cord compression Ix & Mx for malignancy?

Answer

A

Ix: MRI whole spine (often multifocal lesions)

Mx:

1st line = surgical decompression (if localised, fit enough)
2nd line = radiotherapy (external beam radiotherapy)

Question 59

Q

Autosomal dominant polycystic kidney disease is associated with what type of headache?

Answer

A

Thunderclap headache - SAH

Question 60

Q

Meningitis & encephalitis - presentation? causes? Ix? Mx?

Answer

A

Meningitis:

Presentation: headache, fever, photophobia, neck stiffness
Causes:
- Bacterial – N. meningitides, S. pneumo (TB; neonates/elderly – GBS/Listeria/E.coli) – Listeria also more common in alcoholics
- Viral – enterovirus (Coxsackie, echovirus), mumps, HSV2
- Fungal – cryptococcus neoformans (chr)
Ix: A-E, BC, CT head, LP (CT first, if raised ICP –> LP would cause coning)
- Kernig sign - flex knee & hip to 90 degrees –> extend knee (painful & limited extension)
- Brudzinski sign - raise head while flat –> hip & knee flexion
Mx: bacterial – IV ceftriaxone ± amoxicillin (Listeria - neonate/elderly)
- IM BenPen for possible meningococcal infection (rash) in GP setting before sending to the hospital
- Viral – supportive (self-limiting)
- Altered consciousness (encephalitis?) –> add IV acyclovir

Encephalitis:

Presentation: headache, fever, seizures, drowsiness, confusion (viral HSV1 affects temporal lobes –> affecting consciousness)
Ix: BC, CT head, LP
Mx: IV acyclovir ± anti-convulsants

Question 61

Q

Brief twitching in left hand spreading up arm over 2 minutes, weak for 1hr afterwards. PMHx: ischaemic stroke on left-side 6 months ago with good functional recovery

Dx?

Answer

A

Dx: partial seizure affecting right hemisphere caused by previous stroke

Question 62

Q

Vertigo, tinnitus, aural fullness, fascial weakness ± Neurofibromatosis type 2 - Dx?

Answer

A

Acoustic neuroma (CPA)

Question 63

Q

Sensory ataxia vs Cerebellar ataxia based on Romberg’s test?

Answer

A

Postural imbalance/swaying:

When eyes open & closed = Cerebellar ataxia
When eyes are closed = sensory ataxia (somatosensory nerve affected)

Question 64

Q

Spinal cord anatomy

Answer

A

Dark matter middle (cell bodies = synapses) –> white matter on the outside (neurone tracts)

Sensory pathways (ascending - goes up):

Dorsal columns (posterior) - fine touch, vibration, proprioception
- Sensory receptors - 1st order neurone up spinal column along ipsilateral side
- At the medulla oblongata - synapses with 2nd order neurone that crosses the midline (decussation)
- At the thalamus synapses with 3rd order neurone to the Primary Somatosensory cortex
Spinothalamic tracts (anterior) - pain & temperature
- Nociceptors (pain) - 1st order neurone to Substantia Gelatinosa within grey matter of spinal cord
- Synapses with 2nd order neurone & immediately crosses midline (decussation) to ascend spinothalamic tract
  - NOTE: spinothalamic tracts decussate at the level the neurone enters the spinal cord
- At the thalamus synapses with 3rd order neurone to the Primary Somatosensory cortex
(Spinocerebellar tracts (lateral) - proprioceptive info to cerebellum)

Motor pathways (descending - goes down):

Corticospinal tracts (anterior = trunk, neck, shoulders; lateral - limbs) - voluntary motor control (from primary motor cortex)
- From the Primary Motor Cortex - UMN goes to the Medulla and crosses over (decussation)
- At the anterior horn for each spinal level synapses with LMN–> muscle
- NOTE: at any spinal level will be LMNs exiting the spine and UMNs that travel down further before synapsing with their respective LMN
  - SO cord pathology above the level of Cauda Equina –> some UMN & LMN signs

Answer 60

A

Def: tendency to have recurrent, unprovoked seizures

Seizure = episodes of abnormal uncoordinated excessive brain activity
- Provoked seizures = consequence of inf/drugs
- Unprovoked = epilepsy

Types:

Generalised (whole brain):
- Tonic-clonic, Tonic, Atonic, Myoclonic –> Tx: Na Valproate (AVOID in girls/women of childbearing age)
- Absence –> Tx: Ethosuximide/Na Valproate
Focal - aware (conscious) OR impaired awareness (impaired consciousness)
- Tx: Lamotrigine

Status epilepticus = >5mins/repeated seizures without full recovery in between

1st - IV lorazepam 4mg –> repeat
2nd - phenytoin infusion
3rd - general anaesthesia
If no IV access –> rectal diazepam/buccal midazolam

Answer 61

A

Arcuate Fasciculus

Answer 62

A

Cause: Reactivation of the varicella-zoster virus in the genticulate ganglion of CNVII

Presentation:

Otalgia
Facial nerve palsy
Vesicular rash around ear
Vertigo + tinnitus

Mx:

Valaciclovir PO
Steroids PO
NOTE: If treated within 72 hours, 75% recover, otherwise only 1/3 fully recover

Answer 63

A

Ix:

Otoscopy
Cranial nerve assessment including Rinne’s & Weber’s
Pure-tone audiometry
MRI of internal acoustic meatus (to exclude acoustic neuroma)

Mx:

Discuss with local ENT on-call to arrange urgent pure-tone audiometry + plan review after initial primary care Tx
Short-course high-dose oral steroids (caution if DM/IS/elderly) –> 2nd: x3 intratympanic steroid injections (over 3wks) considered in secondary care
If acoustic neuroma - surgery & focused-radiotherapy

NOTE: most cases idiopathic

Answer 64

A

B12 def (much less common in folate def)

Answer 65

A

Presentation

Disc herniation - acute unilateral radiculopathy (pain & numb in specific dermatome/weakness in muscle group)
- Straight leg raise +ve
- Worse on flexion (bending over/sitting)
Spinal stenosis - insidious neurogenic claudication (intermittent back pain worse on walking/standing) ± pain radiating down leg/leg paraesthesia
- RFs: rev back injury/surgery, manual labour
- Worse on extension (standing/walking)

Ix:

Disc herniation - erect lumbar x-ray & MRI spine
Spinal stenosis - plane x-ray, MRI (T2-weighted)

Mx: surgical decompression

Answer 66

A

How to describe:

Eyes disconjugate in the primary position
Comment on the direction of globe e.g. down & out
Where is the diplopia worst? When has the outside image disappeared? Cover each eye in turn & the outer image will disappear when eye with palsy is covered
What is the reaction to light?

NOTE: LR6 SO4

3rd nerve palsy (oculomotor) –> down & out gaze

All the extraocular muscles not working except 4 & 6 so LR turns eye out, SO turns eye down & out + ptosis & diplopia (to the left)
Causes:
- Medial (internal infarct) = pupil-sparing (PS fibres intact)
  - Mononeuritis multiplex (microvascular causes) - HTN, DM
- Surgical (external compression) = pupil dilation (PS fibres damaged as on periphery of 3rd nerve trunk)
  - PCA Aneurysm (posterior communicating artery at junction with internal carotid artery), tumour, dissection
    - PCAA = painful
- SOL/haemorrhage in 3rd nerve nucleus –> bilateral ptosis
Mx: check BP & BM, examine for 4th nerve palsy (check for cavernous sinus problem)
- Dilated pupil - CT scan & angiography (esp if painful)
- Medical palsy = self-resolve within 3 months - prism glasses (minimise double vision)
- Ask to look down - if 4th nerve intact –> mov of scleral blood vessels (as eye tries to turn inwards) = subtle
  - NOTE: 3rd & 4th nerve palsy (± V1 (ophthalmic), 6th nerve) = cavernous sinus involvement (tumour/thrombosis/aneurysm)

4th nerve palsy (trochlear) - subtle up & in gaze + head tilt away from affected eye

SO not functioning so upward deviation of the eye (opposite of 3rd nerve palsy) –> unable to depress eye in adduction (have problems when walking down stairs/reading book)
Causes: head trauma e.g. whiplash/concussion; mononeuritis multiplex (microvascular e.g. DM, HTN), congenital
NOTE: see above about 3rd & 4th nerve palsy

6th nerve palsy (abducens) - medial gaze (can’t look laterally) = VERY IMPORTANT TO PICK UP

LR not functioning so medial deviation of the eye
Causes:
- Most common: microvascular (HTN, DM)
- Raised ICP (tumour/aneurysm), demyelination/mass/vascular
‘false localising sign’ - can’t identify what side of the brain is affected as 6th nerve has the longest IC nerve root in the brain (+ one of first nerves to be affected)
- NOTE: vagus nerve is the longest overall CN
Mx:
- Examine other CNs (7th, 8th) & cerebellar system (cerebellar pontine angle lesion)
- Fundoscopy - papilloedema (raised ICP)

7th nerve palsy (facial):

Presentation:
- Bell’s phenomenon (when asked to close eyes they can’t shut eyelids and eyes will roll back)
- Loss of taste anterior 2/3 tongue, mouth droop, smooth nasolabial folds, hyperacusis (one ear louder than other as stapedius muscle supplied by facial nerve –> dampens sound normally –> louder sound)
- UMN vs LMN (UMN spares forehead)
Causes:
- Viral/idiopathic
- Damaged facial nerve - trauma (e.g. forceps delivery)
- Inf - HZV (Ramsay-Hunt = vesicles in-ear canal), otitis media
- Compression/mass - parotid tumour/cerebellar-pontine angle (CPA) lesion
  - MUST get ENT to rule out cholesteatoma & acoustic neuroma = MRI brain scan
Mx: 50% LMN (Bell’s palsy) recover spontaneously in 6 months
- Eye drops + eye patch
- <72hrs –> steroids
Long-term consequences:
- Synkinesis of facial muscles (when nerve regenerates –> innervate wrong muscles e.g. when scrunch eyes lip twitches upwards)
- Crocodile tears phenomena - where wrongly innervates lacrimal gland = cry when smiling

Answer 67

A

Loss of skeletal muscle tone with strong (usually positive) emotions

Answer 68

A

Common peroneal nerve injury

Answer 69

A

Dx: pellagra = B3 (niacin) deficiency

Cause: dietary & metabolic

Tryptophan converted to niacin (requires B6 - pyridoxine as co-factor)
Isoniazid (TB Tx) binds B6 –> niacin deficiency

Presentation = 3Ds: dermatitis, dementia, diarrhoea

Pellagra rash = ‘Casal necklace’

Answer 70

A

Cerebellar signs ipsilateral

Motor nerves travel laterally in brainstem & cross @medulla

Dorsal columns are posterior & cross @medulla

Spinothalamic tract is anterior in spinal cord & cross in spinal cord

Localisation:

Cortical - UMN pyramidal signs (hypertonia, hyperreflexia, babinski)
Basal ganglia - rigidity, tremor, bradykinesia
Cerebellar - DANISH
Nerve root (dermatome/myotome)/single nerve - LMN signs (hypotonia, hyporeflexia, fasciculations, wasting, sensory loss)

Answer 71

A

LMN presentation = muscle pathology

Face:
- Myopathic facies (sunken cheeks, bilateral ptosis, expressionless)
  - Wasting of facial & muscles of mastication
- Frontotemporal balding
Distal wasting, weakness w/ loss of ankle jerks
Associations: cataracts, dysphagia, cardiac (cardiomyopathy, heart block), DM, hypogonadism (gynaecomastia/testicular atrophy)
Exam - failure of immediate relaxation after voluntary contraction
- Percussion myotonia - tap thenar eminence and thumb contracts
- Slow-releasing grip - SHAKE HANDS (or make a fist and then spread fingers quickly or squeeze eyes shut and open quickly)

Ix:

Conservative: full functional assessment (incl. SALT), lung function tests (NM resp insufficiency), ECG/echo (CMO/HB), slit lamp (cataracts)
Bloods - BM (DM assoc), CK, genetic testing
Imaging & invasive:
- Electromyography (EMG) - ‘dive-bomber’ potentials
- Muscle/nerve biopsy

Mx: MDT approach

Phenytoin for myotonia, weakness has no Tx
Genetic counselling - AD w/ genetic anticipation (trinucleotide repeat - worse severity/earlier presentation with each generation)
Avoid statins (can cause myopathy)

Answer 72

A

MG

Myotonic dystrophy

COMBO: Bilateral Horner’s & 3rd nerve palsy

Congenital (normal variant)

Answer 73

A

Myasthenia Gravis

Def:
- Chr AI disorder of post-synaptic membrane of NMJ in skeletal muscle
- Abs to AChR on post-synaptic membrane
Presentation:
- Muscle fatiguability incl dysphagia, rarely SoB
- Eyes - bilateral ptosis (and compensatory increased frontalis muscle activity = raised eyebrows), diplopia
- Face - dysarthria (slurred speech), facial paresis
- Curtain sign - when raising one upper eyelid the other one drops down
- Assoc: thymomas (in the chest)
Ix:
- Abs: AChR (on post-synaptic membrane), MUSK abs
- Serial pul funct tests (FVC & negative inspiratory force)
  - NOTE: do not wait for ABG findings - late in course
- CT-chest (for thymoma)
Mx:
- Acetylcholinesterase inhibitors (Pyridostigmine) + IS (azathioprine)
- IVIG/Plasmapheresis
- Surgery - thymectomy
Myasthenic crisis = acute respiratory failure - FVC <1L, negative inspiratory force (NIF) ≤20cm H2O, need mechanical ventilatory support
- Accessory muscle use indicates sign inspiratory muscle weakness
- Weak cough indicates sign expiratory muscle weakness
- ABG often shows hypercapnia before hypoxia
- Low threshold for endotracheal intubation (rapid deterioration of bulbar & resp muscles)
- Mx: intubation + mechanical ventilation (& Mx above)

Lambert-Eaton Myasthenic Syndrome (LEMS)

Def: rare AI disorder of NMJ
- Pre-synaptic membrane Ab to voltage-gated Ca-channel receptors
- 40% occur as paraneoplastic disorder assoc w/ SCLC
Presentation:
- Limb weakness - proximal legs –> proximal arms e.g. waddling gait
  - STRONGER WITH USE (vs MG get’s weaker with use)
- Hyporeflexia, NO eye involvement (compared to MG)
- Autonomic disturbance - dry mouth (& orthostatic hypotension, sweating, GI & urinary problems, visual blurring, sexual dysfunction)
- Associated with: small cell lung cancer (SCLC)
Ix:
- Nerve conduction studies, EMG, serial PFTs
- Ab testing (Voltage-gated Ca-channels & AChR-abs)
- CT chest (SCLC)
Mx: MDT support
- Tx underlying cause, Amifampridine
- IVIg/plasma exchange
- Supportive care

Answer 74

A

Test: ask to rise from chair without using hands

DDx:

Diabetic amyotrophy (painful quads wasting)
Cushing’s
Thyrotoxic
Hypocalcaemia/osteomalacia
Polymyositis (infl of muscles - look for malig in lungs/GI tract)
Polymyalgia rheumatica (assoc w/ temporal arteritis)

Answer 75

A

Internuclear opthalmoplegia

RIGHT INO: right eye fails to adduct + nystagmus on lateral gaze in left eye
- When the abducting eye is covered, other eye abducts normally
- Convergence normal
Cause: Multiple sclerosis (MS) - from lesion in medial longitudinal fasciculus

Horner’s syndrome - partial ptosis, miosis (small) & enophthalmos & anhydrosis

Causes: damage to sympathetic fibres
- Pancoast’s, thyroid mass, mediastinal mass, cervical rib
- Carotid/aortic aneurysm/dissection, brainstem disease

Relative afferent pupillary defect (RAPD) aka Marcus Gunn pupil - swing light from eye to eye (eye with pathology dilates when you shine on it instead of constricting)

NOTE: move slower than you think from eye to eye (have to allow the pupil to constrict)
Causes:
- Retinal disease - retinal detachment/ischaemia
- Optic nerve disease - optic neuritis, infl from MS
- Severe glaucoma, trauma
Other pupils:
- Argyll Robertson pupil: bilateral small & irregular pupils
  - No constriction to light, constriction on accommodation reflex
  - Cause: neurosyphilis
- Adie’s tonic pupil: unilateral dilated pupil
  - No constriction to light, constriction on accommodation reflex
  - PS denervation of the affected pupil (damage to PS ciliary ganglion)
- Hutchinson pupil:
  - Stages:
    - 1. Pupil on side of injury constricts - irritation of 3rd CN
    - 1. Pupil on side of injury dilates - paralysis of 3rd CN
    - 1. Pupils on both sides dilated + no reaction to light
  - Cause: cerebral compression (e.g. head injury)

Answer 76

A

MS

HIV

GBS - aka Acute Inflammatory Demyelinating Polyneuropathy (AIDP) –> can progress to CIDP

Multiple myeloma, MGUS

Hereditary motor sensory neuropathy (HMSN) aka Charcot-Marie-Tooth Disease

Answer 77

A

Def: single nerve-damaged

Causes:

Commonest: DM & HTN
Infections - lyme disease (N. America), syphilis
AI disorders - SLE, RA, small vessel vasculitides (polyarteritis nodosa)
Malignancy - haem, paraneoplastic syndrome

Answer 78

A

Cover each eye in turn & the outer image will disappear when eye with palsy is covered

Answer 79

A

Phenytoin = anti-epileptic

Associated SEs:

Gum hypertrophy
Cerebellar (toxicity)
Enzyme-inducer (reduces the effects of other drugs)

Answer 80

A

Trinucleotide repeats - GAA on Chr 9 –> spinocerebellar degeneration

Presentation:

Cerebellar signs, pes cavus (high arch foot), <25yrs
- Pes Cavus - high foot arch that does not flatten on weight-bearing
Loss of ankle jerk BUT upgoing plantars - dorsal column loss –> “stamping gait” (bad loss of proprioception)
Corticospinal tract degeneration
Nerve conduction studies - axonal damage

Assoc: DM, CMO, kyphoscoliosis, SN deafness

Answer 81

A

Def: high foot arch that does not flatten on weight-bearing

Causes:

Bilateral - Friedrich’s ataxia, spinal cord tumours, syringomyelia
Unilateral - polio, compartment syndrome, spinal trauma/tumour

Answer 82

A

After exercising in normal adults

Low K/Mg, Lithium/salbutamol

Thyrotoxicosis

LMN: HMSN aka Charcot-Marie-Tooth disease, Acute & old polio

Answer 83

A

Hammertoes - bilateral, symmetrical, distal wasting of small muscles of feet

Advanced = dorsal guttering from distal wasting, wasting of extensor digitorum brevis at inferior border of lateral malleolus
Similar can be seen in the hands = wasting of first dorsal interosseus

Sign of Charcot-Marie-Tooth disease aka hereditary motor & sensory neuropathy (HMSN)/peroneal muscular atrophy (PMA)

Hx ankle sprains & scoliosis
Motor & sensory losses (sensory milder) = classic peripheral neuropathy
- LMN pattern of weakness:
  - Motor loss in anterolateral compartments of legs (ankle dorsiflexion & toe extension)
  - Absent reflexes (plantar reflexes show no response)
- Sensory loss in glove & stocking distribution bilaterally
Pes cavus ± palpable common peroneal nerve, thickened nerves @medial malleolus
High stepping (foot drop) & ataxic gait
- Test for foot drop = heel walking

What do I need to check for? NO scars over fibula (would indicate peroneal nerve damage from trauma e.g. car accident)

DDx:

Common peroneal nerve palsy (inversion of foot normal)
Sensory peripheral neuropathy: B12-def (SACD), Alcohol, DM, Hypothyroid (vitiligo?), HIV, drugs
Motor peripheral neuropathy: lead poisoning

Types:

Type 1 - demyelination, AD
Type 2 - axonal, AD/AR
Type 3 - demyelination, AR & presents as infant

Ix: full functional assessment, FHx, nerve conduction studies, genetic testing

Mx:

Physio, walking aids with ankle & foot supports (e.g. foot splint)

Answer 84

A

Causes:

Sensory/motor/autonomic
Mainly sensory - alcohol, DM, hypothyroidism (vitiligo?), B12-def, HIV, drugs (amiodarone, isoniazid, metronidazole)
Mainly motor - GBS (AIDP)/CIDP, HMSN (Charcot-Marie-Tooth disease), lead poisoning

Ix:

Alcohol Hx
Bloods - BM, B12, TFTs

Answer 85

A

Hand muscle wasting - dorsal guttering = first to be affected in ulnar lesions

First dorsal interosseous (next to thumb) = ulnar nerve
Abductor policies brevis (thenar eminence) = median nerve
Weakness of both - suspect T1 radiculopathy
NOTE: ulnar nerve innervates most of the intrinsic muscles of hand except median nerve serves LOAF: 2 radial Lumbricals, Opponenes pollicis, Abductor pollicis brevis & Flexor pollicis brevis

Ulnar nerve palsy = claw hand @rest (4th & 5th fingers in extension at MCP, flexion at PIP/DIP)

Commonest site of lesion = elbow (arthritis @wrist & elbow)
Froment’s sign (weak adductor pollicis brevis –> thumb flexion) = +ve if thumb arches to hold paper = ulnar nerve palsy
Ulnar paradox - higher lesion causes lesser deformity as lower lesions spares flexor digitorum profundus (causes flexion at DIP)

Median nerve palsy = sign of benediction on asking to close hand (thumb, index finger can’t close, middle finger can close partially)

Look for sensation over thenar eminence –> if lost can’t be carpal tunnel syndrome (still median nerve palsy but arises proximally to carpal tunnel)
Bilateral carpal tunnel syndrome causes: Acromegaly, Amyloid (periorbital purpura after sneezing), DM, Hypothyroid, pregnancy

Answer 86

A

Normal CSF opening pressure ≤25

Dx: Cerebral Venous Sinus Thrombosis (VTE in the brain)

DDx = Idiopathic intracranial HTN - but would not make you this unstable neurologically
- Obese, female, papilloedema, postural headache worse with straining, assoc w/ OCP
- Ix: visual field testing, fundoscopy, MRI brain, LP (≥25 opening pressure)
- Mx: acetazolamide, weight loss, LPs + VP/LP shunt, ophthalmology follow-up

Presentation: headache, papilloedema, signs of raised ICP (6th nerve palsy), seizures, Cushing’s reflex (HTN, bradycardia, irregular breathing = Cheyne-Stokes)

Ix: MRI w/ MR venography

Mx: LMWH

Answer 87

A

Causes: SOL, inf, thrombosis (CVST), Dysregulated CSF (hydrocephalus)

Ix:

BP (malig HTN) & temp (meningitis)
Neuro & CN exam (6th nerve palsy - raised ICP)
Bloods incl. CRP
Urgent CT-head (brain herniation) –> if normal check LP opening pressure (≥25 = raised ICP)

Answer 88

A

Mononeuropathy - single nerve affected e.g. carpal tunnel syndrome

Polyneuropathy - many nerves affected with bilateral presentation e.g. Charcot-Marie-Tooth, GBS/CIDP, vasculitis, HIV, DM

Mononeuritis multiplex - several nerves affected in different areas of the body e.g. DM (more commonly causes polyneuropathy), vasculitis, SLE, Sjogren’s, RA, Sarcoidosis, Amyloidosis, inf (lyme disease, HIV)

Answer 89

A

Dorsal column loss:

Tabes dorsalis (syphilis)
Subacute Combined Degeneration of Spinal Cord (B12 def)
MS

Sensory peripheral neuropathy

Answer 90

A

It identifies subtle UMN weakness e.g. stroke.

If the forearm pronates, with or without downward movement, the patient is considered to have pronator drift on that side. The presence of pronator drift indicates a contralateral pyramidal tract lesion. Pronation occurs because, in the context of an UMN lesion, the supinator muscles of the forearm are typically weaker than the pronator muscles.

Answer 91

A

When you’re testing their elbow flexion/extension, ask them to tap their knee with their other hand while you continue to flex extend. This is called Synkinesis and it reinforces hypertonia.

Answer 92

A

Brain: stroke, SOL, MS

Spinal cord: MS, trauma, disc herniation/spinal stenosis, spinal cord infarct, syringomyelia

MS - weakness & paraesthesia based on UMN demyelination, disseminated in time & space
- Optic neuritis, periorbital pain, RAPD, INO (damage to medial longitudinal fasciculus)
- Ataxia, vertigo, chr constipation, blaadder dysfunct
- Lhermitte’s sign - electric shock down back on neck flexion
- Unhthoff’s phenomenon - worsening of Sx with increased body temp e.g. exercise, hot shower
- Ix: MRI (brain & spinal cord) + LP (oligoclonal bands), nerve conduction study (evoked potential)
- Mx: methypred for flare, INF-b long-term
Trauma - extremity weakness wo/ cortical signs/facial weakness e.g. hemisection of spinal cord (Brown-Sequard syndrome) - below level of lesion:
- Ipsilateral hemiparesis & loss of vibration/proprioception
- Contralateral loss of pain & temperature
- At level of lesion - loss of sensation + flaccid paralysis of muscles supplied by this spinal cord segment
Herniated disc/spinal stenosis - weakness at level of lesion & lower extremities + bladder/bowel dysfunction + sciatica
Spinal cord infarct - occlusion of anterior spinal artery (complication of aortic surgery)
- Affects anterior 2/3 spinal cord in affected segment
  - Sudden-onset bilateral flaccid paralysis –> developing into spastic paralysis after several days
  - Loss of pain & temp
  - Light-touch, vibration & proprioception-sense spared (dorsal columns not involved)
Syringomyelia - developmental disorder
- Syrinx (cavity) grows from centre of spinal cord & spreads outwards
- Normally affects cervical cord but can extend into brainstem
- Early - bilateral loss of pain & temp in cape-like distribution affecting neck, shoulders, upper arms
- Late - as syrinx expands anterior horn cells affected –> bilateral flaccid paralysis

Anterior horn (motor neurone lesions): MND, poliomyelitis

Poliomyelitis - enterovirus infection attacking anterior horn cells
- 1-2wks prodrome (fever, headache, N&V)
- Asymmetrical weakness + can develop years after inf (post-polio syndrome) + can take place after taking live-attenuated polio vaccine

Neuropathy: diabetic neuropathy

Polyneuropathy (multiple nerves): DANG My THeRAPIST
- Diabetic neuropathy (most common, T1/2)
  - Sensory - glove & stocking loss of sensation/paraesthesia
  - Motor - same distribution as above + CN palsies (3rd - pupil-sparing)
  - Autonomic - orthostatic hypotension, constipation, erectile dysfunction
- Alcoholic neuropathy (2nd most common)
- Nutritional (B1/6/12 def)
- GBS (AIDP - acute inflam demyelin polyneuropathy)
  - AI demyelination of peripheral nerves
  - 2-4wks post-inf e.g. diarrhoea with C. jejuni
  - Ascending weakness in distal lower extremities over hrs/days (worst after 4wks)
  - Flaccid paralysis with reduced/absent deep tendon reflexes
    - Can involve diaphragm –> resp failure
    - Bilateral facial nerve palsy
    - Difficulty swallowing –> aspiration
  - Autonomic dysfunction - sweating, orthostatic hypotension, urinary retention
  - NOTE: sensory Sx rare
  - Ix:
    - LP (high protein, normal WCC)
    - NCS & EMG (reduced conduction velocity)
    - Serum abs (anti-glycolipid abs)
  - Mx:
    - Monitor FVC <1L, NIF <20cm H2O –> intubate + mechanical ventilation
    - Monitor BP –> IV fluids for hypo, Labetalol for hyper
    - IVIG, plasma exchange
- M(y)-edications (colchicine, cisplatin, isoniazid –> niacin def)
- Toxins (lead)
- Hereditary - HMSN (CMT)
  - Progressive hereditary (AD) motor & sensory neuropathy (HMSN)
  - Distal back & lower extremity weakness:
    - Foot drop (damage to common peroneal nerve)
    - High-arch foot (pes cavus) - does not flatten with weight-bearing
    - Scoliosis
  - ± pain & sensory loss (can lead to foot ulcers)
  - Ix: nerve conduction study + EMG, genetic testing (electrophoresis/FISH)
  - Mx: physio, walking aids, foot & heel-support
- Renal failure (uraemic nephropathy)
- Amyloidosis
- Porphyrias
- Inf (HIV, syphilis)
- Systemic (hypothyroidism)
- Tumours (multiple myeloma)
Mononeuropathy (one nerve):
- Facial nerve palsy - not forehead-sparing, crying/sensitive to noise/abnormal taste (ant 2/3 tongue)
  - Idiopathic = Bell’s palsy
  - Secondary:
    - Lyme disease
    - Ramsay-Hunt syndrome - HZV reactivation in the geniculate ganglion, causes painful eruption in auditory canal
    - Tumours - acoustic neuroma, parotid
    - Bilateral palsy - Sarcoidosis, GBS
  - Tx: treat cause or if Bell’s palsy –> pred within 72hrs Sx onset + eye protection
- Carpal tunnel syndrome - entrapment of median nerve in flexor retinaculum
  - Repetitive use of wrist –> numbness & tingling in lateral 3 fingers (& 1/2 4th finger) –> weakness of thenar muscles
  - Tinel’s sign - tap nerve at wrist
  - Phalen’s sign - reverse prayer sign for 60s
  - Ix: EMG, wrist USS, MRI wrist (imaging to detect SOL e.g. ganglion cyst)
  - Tx: immobiliser wrist (splint), CS injection, carpal tunnel release

Neuromuscular junction: MG/LEMS

Myasthenia gravis (MG) - AI condition where Abs attach to NMJ post-synaptic nicotinic AChR in skeletal muscle
- FATIGUABILITY with use
- Initial - oculobulbar weakness (diplopia, ptosis, dysphagia, nasal voice)
- Chr:
  - Asymmetrical proximal limb muscle weakness
  - Resp muscle failure
- Assoc w/ thymic hyperplasia/thymoma
- NOTE: normal sensation, normal deep tendon & pupillary reflexes
- Ix:
  - Serological testing: AChR, MUSK abs
  - Repetitive nerve stimulation (CMAPs decrease)
  - CT chest - thymoma
  - Monitoring with serial pul funct tests (FVC & negative inspiratory force) if suspect myasthenic crisis
- Mx:
  - Acetylcholinesterase inhibitors (Pyridostigmine) + IS (azathioprine)
  - Surgery - thymectomy
- Myasthenic crisis = acute respiratory failure - FVC <1L, negative inspiratory force (NIF) ≤20cm H2O
  - Often trigger - meds/inf
  - Accessory muscle use/weak cough = sign. exp muscle weakness
  - Mx: intubation + mechanical ventilation (& Mx above)
    - IVIG/Plasmapheresis
    - Stop pyridostigmine (increases secretions –> aspiration risk), stop trigger (meds)
- Cholinergic crisis - same presentation as above (resp muscle weakness) BUT cause = excess pyridostigmine –> overstimulation of AChR –> eventually stop working
  - Pyridostigmine also binds to nicotinic receptors –> cholinergic Sx - SLUDGE (Salivation, Lacrimation, Urination, Diarrhoea, GI cramps, Emesis)
  - Mx: intubation + mechanical ventilation
    - Atropine (anti-muscarinic) for Sx but does not address resp weakness
    - Reduce dose of pyridostigmine
Lambert-Eaton myasthenic syndrome (LEMS) - AI condition where Abs target pre-synaptic voltage-gated Ca channels –> reduces release of ACh from presynaptic vesicles
- Proximal muscle weakness - IMPROVES with use
- Reflexes reduced/absent, ± autonomic Sx
- Assoc w/ SCLC, Hodgkin’s lymphoma
- Ix:
  - NCS, repetitive nerve stimulation (CMAPs increase)
  - Serological testing: VGCC abs
  - CT chest (SCLC)
- Mx: Tx underlying cause, Amifampridine
  - IVIg/plasma exchange
  - Supportive care incl. intubation + mech ventilation (if resp compromise)

Myopathy: dermatomyositis

Inflammatory: proximal muscle weakness & pain
- Dermatomyositis - skin rash (heliotropic rash & Gottron’s papules)
- Polymyositis
Inherited:
- X-linked disorders:
  - Duchenne’s & Becker’s muscular dystrophy
- Mitochondrial
Endocrine:
- Hypothyroidism - proximal muscle weakness & pain, deep tendon reflexes decreased, myoedema (swelling on percussion)
- Also possible in Addison’s Cushing’s, Vit D def
Meds - statins (proximal muscle weakness & pain), glucocorticoids (muscle weakness)
Ix: serum CK, TSH lvls, EMG

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