Station 3: Neurology

Question 1

General inspection findings of isolated ulnar nerve neuropathy?

Answer 1

• wasting of the dorsal interossei “guttering” of the hands, and hypothenar eminence
• ulnar claw: flexion of the interphalangeal joints of the 4th and 5th digits
• sparing of the thenar eminence

cause: look for scars around the wrist or near the elbow

Question 2

if suspecting ulnar neuropathy, what other important negatives?

Answer 2

rule out medial/ nerve palsies or C8/T1 nerve root problem

Question 3

Power findings in ulnar neuropathy?

Answer 3

weak
- finger abduction (dorsal interossei)
- Froment’s sign positive (weakness of adduction of the thumb, adductor policis)

test finger flexion of the 5th finger for flexor digitorum profundus involvement (more proximal lesions may result in weakness of IP joint flexion)

test for wrist flexion at ulnar side (flexor carpi ulnaris)

Question 4

ulnar paradox?

Answer 4

the higher the level of injury to ulnar nerve, the less obvious the “clawing”

if ulnar nerve injured more proximally, the ulnar half of the flexor digitorum profundus muscle may be affected. As a result, flexion of the IP joints of the 4th and 5th fingers is weakened, which reduces the claw-like appearance of the hand.

Question 5

sensory testing in ulnar neuropathy?

Answer 5

medial 1.5 fingers affected

Question 6

what is the anatomical course of the ulnar nerve?

Answer 6

begins from the medial cord of the brachial plexus (C8, T1)

enters the forearm via the cubital tunnel (medial epicondyle and olecranon proocess) and motor supply to the flexor carpi ulnaris and ulna half of the flexor digitorum profundus

gives off a sensory branch just above the wrist

and enters the Guyon’s canal and supplies the sensory medial 1.5 fingers and hypothenar as well as motor to all intrinsic muscles of the hands except LOAF (lateral two lumbricals, opponens pollicis, abductor pollicis brevis, flexor pollicis brevis)

Question 7

what does ulnar nerve supply in terms of motor function?

Answer 7

motor to all muscles of the hands except the LOAF:
- lateral two lumbricals.
- opponens pollicis.
- abductor pollicis brevis.
- flexor pollicis brevis

forearm: flexor carpi ulnaris (wrist flexion and adduction) and flexor digitorum profundus to 4th/5th fingers

Question 8

level of ulnar lesions and its clinical correlation?

Answer 8

wrist- hypothenar eminence wasting, froment’s positive, weakness of finger abduction, pronounced claw and loss of sensation

elbow- less pronounced claw, loss of terminal flexion of the DIPJ and loss of flexor carpi ulnaris tenon on the ulnar flexion of the wrist

Question 9

how do you differentiate ulnar nerve palsy vs T1 lesion?

Answer 9

motor- wasting of the thenar eminence will be seen for T1

sensory- loss of T1 dermatomal distribution

Question 10

what is the ulna claw hand?

Answer 10

• hyperextension of the 4th and 5th MCPJ associated with flexion of the IPJs of the 4th and 5th fingers

due to the unopposed long extensors of the 4th/5th fingers in contrast to the IF and MF

Question 11

what is froments sign?

Answer 11

asked to grasp piece of paper between thumbs and the lateral aspect of the index finger

affected thumb will flex as the adductor policis muscles are weak
-> pt trying to compensate by using the flexor pollicis longus supplied by the median nerve

Question 12

causes of ulnar nerve palsy?

Answer 12

• compression or entrapment (Cubital tunnel at elbow; Guyon’s canal at the wrist)
• trauma (fractures or dislocation- cubitus valgus leads to tardive ulnar nerve palsy)
• surgical
• mononeuritis multiplex
• infection: leprosy (thickened nerves, hypopigmented hypoaesthetic patches)
• ischaemia- vasculitis
• inflammatory -CIDP

Question 13

ix of ulnar nerve palsy?

Answer 13

• blood ix to rule out DM if no obvious cause
• Xrays of the elbow and wrist KIV C spine
• EMG (axonal degeneration for chronic)
• Nerve conduction studies: can help to locate level and monitor

Question 14

mx of ulnar nerve palsy?

Answer 14

education and avoidance of resting on elbow

PTOT

Medical: NSAIDs

Surgical decompression with anterior transposition of the nerve

Question 15

causes of sensory predominant peripheral neuropathy?

Answer 15

DM
Alcohol
Metabolic: B1, B6, B12 deficiency
CKD
Infective: Leprosy

Question 16

causes of motor predominant peripheral neuropathy?

Answer 16

inflammatory: GBS, amyloid, sarcoid, hiv
drugs: lead poisoning, dapsone, organophosphate
metabolic: DM, porphyria
Congenital: charcot-marie-tooth (HSMN type 1)
PAN

Question 17

causes of mononeuritis multiplex? (ie involvement of 2 or more peripheral or cranial nerves by the same disease)

Answer 17

Endocrine: DM, Acromegaly
Infiltrative: Amyloidosis, Sarcoidosis
Autoimmune: RA/SLE, PAN, Sjogren, GPA, eGPA
Neoplastic: Carinomatosis
Infection: Leprosy, Lyme, HIV

Question 18

causes of thickened nerves?

Answer 18

CIDP
Charcot-Marie Tooth Disease (HMSN)
Acromegaly
Amyloidosis

Others:
Sarcoidosis
Leprosy
Neurofibromatosis

Refsum Disease (Retinitis pigmentosa, optic atrophy, cerebellar and deafness, cardiomyopathy and ichthyosis)

Dejerene-Sottas disease (hypertrophic peripheral neuropathy)

Question 19

Causes of sensorimotor peripheral neuropathy?

Answer 19

• DM
• Alcohol
• Endocrine: Hypothyroidism
• Uraemia (CKD)
• Sarcoidosis
• Inflammatory: Vasculitis
• Paraneoplastic
• Immune mediated: CIDP
• Congenital: HSMN
• Drugs: Vincristine, Cisplatin, Gold, Amiodarone

Question 20

Drugs causing sensory peripheral neuropathy?

Answer 20

isoniazid, chloroquine, metronidazole

Question 21

causes of peripheral neuropathy with autonomic dysfunction?

Answer 21

GBS
infection: botulism, Chagas disease, HIV
porphyria
paraneoplastic
DM
Amyloidosis

Question 22

Demyelinating causes of peripheral neuropathy?

Answer 22

CIDP
Multiple myeloma
HSMN Type 1 and 3
HIV
POEMs disease
Multifocal motor neuropathy
Hereditary neuropathy with pressure palsy

Question 23

Screening for causes of peripheral neuropathy during neuro examination?

Answer 23

• features of DM: diabetic dermopathy
• thickened nerves or hypopigmentation patches (leprosy)
• parotidomegaly, dupuytrens (ETOH)
• sallow (uraemia)
• pallor (B12 deficiency)
• cachexia, toe clubbing (paraneoplastic)
• symmetrical deforming polyarthropathy (RA)
• clinical features of acromegaly, hypothyroidism

Question 24

gait in peripheral neuropathy?

Answer 24

sensory ataxic gait
- may be high stepping

Question 25

causes of peripheral neuropathy (mixed/ sensory/ motor)

Answer 25

DAMIT BICH Drugs: Isoniazid, chloroquine, nitrofurantoin, gold, penicillamine, cyclosporin A, phenytoin, vincristine Alcohol Metabolic: DM, uraemia (CKD), porphyria Infectious: Leprosy, HIV, botulism Inflammatory: GBS, CIDP Tumour: paraproteinaemia (MM), Paraneoplastic (Lung Ca) B12, B6, B1 Infiltrative: Amyloid, sarcoid Congenital: HMSN, porphyria Hormonal: Acromegaly, Hypothyroidism

Question 26

causes of a painful peripheral neuropathy?

Answer 26

DM, Alcohol, B12 deficiency Carcinoma, Porphyria, Arsenic

Question 27

types of neuropathy in DM?

Answer 27

- glove and stocking symmetrical sensory neuropathy - predominantly motor, asymmetrial (DM amyotrophy) - mixed motor and sensory peripheral neuropathy - mononeuropathy - mononeuritis multiplex - autonomic neuropathy

Question 28

neurological complications of alcohol?

Answer 28

wernicke's (Confusion, ophthalmoplegia, ataxia) korsakoffs psychosis (anterograde amnesia, confabulation) cerebellar degeneration central pontine myelinolysis epilepsy myopathy and rhabdomyolysis peripheral neuropathy

Question 29

nerve conduction studies in diabetic peripheral neuropathy?

Answer 29

axonal loss

Question 30

Further examination if patient presents with ptosis?

Answer 30

1. visual acuity 2. pupils: miosis (horners), mydriasis (CN III) 3. EOM (down and out: CN III, complex: MG?) 4. fatiguability: MG

Question 31

DDx unilateral ptosis?

Answer 31

Horner's syndrome 3rd Nerve Palsy Myasthenia gravis Previous tarsorraphy/ congenital ptosis Muscle - local orbital infiltration/ pathology, dystrophia myotonica

Question 32

DDx Bilateral ptosis?

Answer 32

Myasthenia gravis Myotonia dystrophy GBS/ Miller Fisher Motor Neuron Disease Oculopharygneal dystrophy Thyroid eye disease Senile ptosis/ congenital ptosis Mitochondrial dystrophy e.g. CPEO, Kearn Sayre syndrome Tabes dorsalis Nuclear 3rd nerve palsy Syringomyelia with bilateral Horner's syndrome

Question 33

Esotropia

Answer 33

6th nerve palsy

Question 34

DDx exotropia

Answer 34

3rd nerve palsy 4th nerve palsy

Question 35

DDx complex ophthalmoplegias?

Answer 35

Central cause: Intranuclear ophthalmoplegia Wernicke's encephalopathy Nerves: Miller Fisher NMJ: Myasthenia gravis Myopathies: Thyroid eye disease CPEO/ Kearn-Sayre synrome or Ragged Red Fiber Myopathy Glass eye/ trauma

Question 36

cause of unilateral facial droop? + Long tract signs

Answer 36

UMN lesion - pontine and above

Question 37

unilateral facial droop + VIII nerve involvement? neurolocalisation

Answer 37

Internal auditary canal

Question 38

unilateral facial droop + 5th/6th/8th CN involvement neurolocalisation?

Answer 38

Cerebellopontine angle lesion

Question 39

unilateral facial droop + cerebellar involvement; neurolocalisation?

Answer 39

middle cerebellar peduncle

Question 40

Approach to CN examination, if no obvious signs on general inspection. what to think of?

Answer 40

visual fields RAPD (with fundoscopy) EOM restriction Myasthenia Gravis Bulbar/ pseudobulbar palsy

Question 41

Cranial nerve examination: general inspection. What can be suggestive of underlying defect?

Answer 41

- Ambulation aids: ?LL weakness - Supplemental O2 - NG tube ?CN 9/10/12 lesion - Urine catheters ?cauda equina, neurogenic bladder, myelopathy - IV fluids/drugs then look more closely: any ophthalmoplegia? ptosis? facial asymmetry? abnormal speech? limb asymmetry/ posturing tremors fasciculations

Question 42

CN exam: checking CN 1

Answer 42

olfactory nerve - ask "any problems with your sense of smell?"

Question 43

CN exam: checking CN 2?

Answer 43

optic nerve - check visual acuity grossly (how many fingers?) - pupillary reflexes (direct, consensual, accommodation) - RAPD?

Question 44

CN exam: checking CN 3/4/6?

Answer 44

Oculomotor, Trochlear, Abducens - check ROM of eyes (diplopia, ophthalmoplegia) - in presence of complex ophthalmoplegia or ptosis, check for fatiguability

Question 45

CN exam: CN 5 testing?

Answer 45

Trigeminal nerve - pin prick sensation over forehead, maxilla, chin - "bite your teeth" -> feel for bulk masseters - "open your mouth" -> feel for strength of pterygoids and see if jaw moves to one side (displaced to the side of weakness)

Question 46

CN exam: CN 7 testing?

Answer 46

- "Look up" -> loss of wrinkling of forehead (frontalis) = LMN lesion - "Close your eyes as tight as possible, don't let me open them" -> bell's phenomenon, unable to bury eyelashes - "show me your teeth" -> facial droop, loss of nasolabial fold - "puff out your cheeks" additional: lacrimation - lesion proximal to geniculate nucleus if affected taste over tip of tongue salivary gland hyperacusis - paralysis of stapedius muscle

Question 47

CN exam: CN 8 testing?

Answer 47

"I'm going to say something into your ear, please repeat the words after me" - Rinne's test: place 256Hz tuning fork on mastoid, then in line with ear - Weber's test: place 256Hz tuning fork on forehead Rinne's positive = AC>BC. negative: BC > AC (conductive HL) Weber's test: localizes to side with conductive HL, localizes away from side with SNHL

Question 48

CN exam: testing for CN 9/10 nerves

Answer 48

Glossopharyngeal, vagus nerves "open your mouth and say AH" -> look for deviation of uvula and palatal weakness CN IX: mainly sensory function CN X: mainly motor function in the mouth

Question 49

CN exam: testing CN XII nerve?

Answer 49

Hypoglossal nerve stick out tongue (deviation, wasting, fasciculations) move it left to right

Question 50

CN exam: testing CN XI accessory nerve?

Answer 50

"push your head against my hand" test SCM ->feel for SCM contraction/bulk shrug shoulder and feel for trapezius contraction/bulk

Question 51

CN exam: how to screen for cerebellar and UL signs?

Answer 51

Cerebellar: Nystagmus, finger-nose test (intention tremor), dysdiadochokinesia, upper limb rebound, heel shin test, toe finger test, gait (ataxia) UL and LL: Hemiplegia, pyramidal signs

Question 52

CN exam: how to say you will complete your examination?

Answer 52

- fundoscopy - visual field defect - proper assessment of visual acuity with snellen's chart - check the corneal reflex: afferent CN V1, efferent VII - check gag reflex: afferent CN IX, efferent X

Question 53

CN II lesion: how may different pathologies present?

Answer 53

- homonymous hemianopia: optic tract/radiation lesion - superior homonymous quadrantonopia: contralat temporal lobe lesion - inferior homonymous quadrantonopia: contralat parietal lobe lesion - bitemporal hemianopia: optic chiasm lesion - monocular blindness: pre-chiasmal lesion

Question 54

CN III: how may different pathologies present?

Answer 54

- pupils normal: ischaemic CN III palsy - pupils fixed and dilated: PCOM aneurysm - DDx OAS, SOFS, CSS, Benedikt's syndrome, MG (excluded if pupillary reflex abnormal)

Question 55

CN V lesion: how may different pathologies present?

Answer 55

ganglion/sensory root lesion: total loss of sensation in all 3 divisions post-ganglionic lesion: total loss of sensation in 1 division (usually ophthalmic division a/w CN III, IV, VI palsies due to lesion in cavernous sinus) Brainstem/upper cervical cord lesion: - dissociated sensory loss of face: ie. loss of temp/sensation but retention of touch and propioception sensations of face

Question 56

CN VI lesion: how may different pathologies present?

Answer 56

nerve tract: - cavernous sinus (may p/w isolate CN VI if early) - clivus lesino - NPC/meningeal lesino - GBS/MFS- check reflexes - ischaemic - false localising sign NMJ: MG Local/muscle: infiltrative orbital process, tumour thyroid eye disease DDx: OAS, SOFS, CP angle

Question 57

Causes of UMN CN VII lesion?

Answer 57

contralateral cortical or subcortical lesion: vascular: stroke Tumours -> if detected, examine UL and look for hemiparesis on same side of facial weakness -> check for xanthelasma, DM signs, BP

Question 58

Causes of LMN CN VII lesions?

Answer 58

infection: HSV (bell's palsy), zoster, lyme disease Connective tissue/ infiltrative: sarcoid, amyloidosis, Sjogrens, behcets Neoplasm Brainstem (infarct/ haemorrhage) - a/w gaze palsy MS **can organize by site of lesion:** brainstem: infarct/bleed, MS, abscess/tumour, syringobulbia base of skull lesion: infective, tumour, infiltrative CPA lesion: Acoustic neuromas Meningiomas neurofibroma Petrous temporal bone/ facial nerve canal: Bells palsy Ramsay Hunt syndrome Fractures otitis media Parotid gland: tumour, surgery, sarcoidosis mononeuritis multiplex

Question 59

causes of isolated CN II palsy?

Answer 59

ischaemic PCOM aneurysm

Question 60

causes of isolated CN IV palsy?

Answer 60

trauma decompensated squint

Question 61

causes of single CN VI palsy?

Answer 61

- false localising - ischaemic - NPC - meningeal - cavernous sinus pathology

Question 62

causes of CN VII isolated palsy?

Answer 62

Bells palsy Parotid tumour Middle ear disease trauma: # to mastoid area

Question 63

Causes of isolated CN XII palsy?

Answer 63

hypoglossal canal base of skull pathology carotid artery dissection ALS (bilateral wasted tongue)

Question 64

CN III palsy?

Answer 64

- abnormal EOM (down and out pupil) - abnormal light reflex - abnormal dilated pupils (may be normal in ischaemic CN III palsy)

Question 65

causes of isolated CN III palsy?

Answer 65

1. Posterior communicating artery aneurysm 2. Medical CN III palsy

Question 66

cavernous sinus syndrome?

Answer 66

CN III, IV, V1, V2, VI

Question 67

superior orbital fissure syndrome?

Answer 67

CN III, IV, V1, VI

Question 68

Orbital apex syndrome?

Answer 68

CN II, III, IV, V1, VI

Question 69

CN III palsy + long tract signs?

Answer 69

midbrain pathology

Question 70

CN III palsy + signs of peripheral neuropathy?

Answer 70

GBS/ Miller Fisher syndrome

Question 71

Signs of Horner's syndrome

Answer 71

miosis partial ptosis enophthalmos slight elevation of lower eyelids +/- anhidrosis depending on level of lesion

Question 72

Causes of Horners syndrome + what to examine for?

Answer 72

1. Pancoast tumour: - tracheal deviation, supraclavicular dullness, wasting of small muscle of hands, clubbing, loss of pain sensation in UL 2. scars of cervical sympathectomy 3. enlarged LNs 4. carotid and aortic aneurysms 5. examine for signs of brainsteam vascular disease or demyelination - nystagmus, cerebellar signs, cranial nerves, pale optic discs

Question 73

Suspected myasthenia gravis: what to examine?

Answer 73

Test for fatiguability - prolonged upward gaze - superio-lateral gaze -> Normal pupils, normal light reflex, EOM may be abnormal Test for bulbar weakness Test for proximal limb weakness Examine for associated signs: - thyroid goitre, retrosternal extension - retrosternal thymoma - RA hands - vitiligo

Question 74

Horner's syndrome: degree of anhidrosis according to location of lesion

Answer 74

central lesion (1st order neuron): anhidrosis over head, upper trunk and arm proximal to superior cervical ganglion (2nd order neuron): anhidrosis over face only distal to super cervical ganglion (3rd order neuron): none

Question 75

approach to unilateral ptosis?

Answer 75

1. rule out pseudoptosis - droopy eyelids 2. muscle - myotonic dystrophy 3. neuromuscular - Myasthenia gravis 4. nerve - III nerve palsy - horner's syndrome

Question 76

approach to unilateral horner's syndrome?

Answer 76

1. examine the other cranial nerves - cavernous sinus syndrome - superior orbital syndrome - lateral medullary syndrome - syringobulbia - multiple sclerosis (INO, cerebellar, RAPD) 2. neck - scars: trauma/ surgery - neoplasia - carotid aneurysm - cervical rib 3. upper limbs (examine in this sequence) - pronator drift then cerebellar signs (lateral medullary syndrome) - wasting of ipsilateral small muscles of the hands (T1) - clubbing - sensory loss T1 - dissociated sensory loss (syringomyelia) - contralateral loss to pain and temp (lateral medullary syndrome) - axilla (trauma to brachial plexus) 4. chest - pancoast tumour: tracheal deviation, dullness, auscultation 5. ask for loss of sweating and level

Question 77

what medications can help to delineate the site of lesion clinically?

Answer 77

adrenaline 1:1000 to both eyes (denervation hypersensitivity) - abovev the superior cervical ganglion (peripheral) = dilates the affected eye - below/ proximal to superior cervical ganglion or a normal eye = no effect cocaine 4% - dilates normal eyes - no effect on the affected side if above/ distal to superior cervical ganglion

Question 78

how do you delineate the site of lesion clinically via loss of sweating?

Answer 78

central lesion (1st order): loss of sweating in head, upper trunk and arm neck, proximal to superior cervical ganglion (2nd order): loss of sweating in face neck, distal to superior cervical ganglion: sweating intact (3rd order)

Question 79

causes of horner's syndrome?

Answer 79

Hypothalamus/ brainstem: stroke, pontine glioma, coning of temporal lobe cervical cord (C8-T2: intermediolateral column): syringomyelia, multiple sclerosis, tumour superior mediastinum (2nd order nerves exixt the spinal cord and synapses at the superior cervical ganglion): pancoast lesion (SCC lung), trauma to brachial plexus neck (carotid sympathetic plexus and superior cervical ganglion): neoplasia, trauma, surgery (cervical sympathectomy), carotid aneurysm, carotid dissection (triad of pain, ipsilateral horner's, cerebral/retinal ischaemia) idiopathic congenital - heterochromia of iris (grey blue on affected side) migraine- causes intermittent horner's syndrome

Question 80

approach to bilateral ptosis?

Answer 80

muscular (usually no wrinkling of the forehead): myotonic dystrophy ocular myopathy oculopharyngeal dystrophy chronic progressive external ophthalmoplegia (mitochondrial/Kearns sayre) Neuromuscular: myasthenia gravis Nerve: bilateral 3rd CN palsy (rare) bilateral Horners' (syringomyelia) tabes dorsalis miller fisher syndrome

Question 81

examination approach in bilateral ptosis?

Answer 81

- general screen for myotonic dystrophy, fascioscapular dystrophy - screen for myasthenia gravis - check CNs: > III, Horner's > Argyll Robertson pupils (Tabes) > Ophthalmoplegia (kearns sayre) > bulbar palsy (syringomyelia) - Neck - Upper Limbs: > ataxia (kearns sayre, miller fisher) > Syringomyelia: flaccid and wasted ULs, dissociated sensory loss, spastic paraparesis > Areflexia (miller fisher) - complete examination by fundoscopy for retinitis pigmentosa (CPEO)

Question 82

Examination of myasthenia gravis, sequence?

Answer 82

1. eyes - ptosis with fatiguability - variable strabismus and diplopia that occurs after some time - check for hyperthyroid and thyroid eye disease - check for anaemia - check for malar rash of SLE 2. face - VII power - assessment of speech: count to 1 to 20, nasal voice (bulbar palsy) - masseter weakness but pterygoids normal - check neck for goitre and scars 3. ULs - normal deep tendon reflexes (Miller fisher/ lambort eaton are reduced) - normal sensation - fatiguability with weakness - RA and SLE features 4. Thymectomy scar and plasmapheresis line associations of Myasthenia gravis: endocrine: Thyroid, DM, pernicious anaemia CTD: RA, SLE, polymyositis Request for: negative inspiratory force drug history: antibiotics/ drugs that can worsen MG temperature chart for fever - can ppt weakness

Question 83

what is myasthenia gravis?

Answer 83

- autoimmune condition with antibodies targeting the post synaptic Ach receptors of the neuromuscular junction - resulting in progressive muscle weakness with use of the muscle and recovery of strength after a period of rest - weakness experienced once number of receptors is 30% or less

Question 84

how common is the thymus involved in myasthenia gravis ?

Answer 84

75% of cases: of which 15% are thymomas and 85% are thymic hyperplasia

Question 85

usual age for myasthenia gravis?

Answer 85

2 peaks 20 to 30 years old with female predominance >50 years old with male predominance

Question 86

features of myasthenia gravis on presentation

Answer 86

ptosis, diplopia dysarthria, difficulty swallowing (isolated bulbar muscle involvement in ~20%) generalised weaness or reduced exercise tolerance respiratory failure in 1% tends to affect extra ocular muscles first then facial to bulbar and then limbs and truncal

Question 87

what may exacerbate myasthenia gravis or precipitate a crisis?

Answer 87

- non compliance to meds - infection - emotions/ stress - drugs

Question 88

what drugs may exacerbate MG crisis?

Answer 88

antibiotics: aminoglycosides, tetracyclines, macrolides, fluoroquinolones cardiac: BBs, CCBs (verapamil) others: chloroquine, procainamide, Lithium, Mg, prednisolone, quinine, penicillamine

Question 89

what is a cholinergic crisis?

Answer 89

not the same as myasthenic crisis results from excess of cholinesterase inhibitors such as neostigmine and pyridostigmine causes flaccid paralysis and SLUDGE (miosis, salivation, lacrimation, urinary incontinence, diarrhoea, GI hypermotility and emesis)

Question 90

ix to do for suspected myasthenia gravis?

Answer 90

Bloods: - AChR Ab (+ve in 80% with generalised MG, only +ve in 50% with ocular involvement only, also present in 90% of patients with penicillamine induced MG) - anti striated muscle Ab - Anti MUSK Ab (muscle specific kinase) - FBC to rule out infection Imaging: - CXR: thymus (Anterior mediastinal mass), aspiration pneumonia - CT for thymus Tensilon test: for diagnosis Ice pack test Electrodiagnostic studies: - repetitive nerve stimulation test - single fibre nerve EMG

Question 91

what is the ice pack test in myasthenia gravis?

Answer 91

ice applied with glove to eyelids for 2 mins improvement in ptosis (positive in 80%)

Question 92

what is the tensilon test in myasthenia gravis?

Answer 92

can help to distinguish from cholinergic crisis - edrophonium (half life 10 min) - look for objective improvement in ptosis - cardiac monitoring for bradycardia and astystole (Rx with atropine if any) - 1mg test dose and up to 10mg - in cholinergic crisis, will get increased salivation etc

Question 93

repetitive nerve stimulation test in myasthenia gravis?

Answer 93

shows a decrease in the compound muscle action potential by 10% in the 4th to 5th response to a train of nerve stimuli

Question 94

single fibre nerve EMG in myasthenia gravis?

Answer 94

evidence of neuromuscle blockade with increased jitter

Question 95

how do you grade the severity of weakness in myasthenia gravis?

Answer 95

Myasthenia Gravis Foundation of America - Grade 1: affects ocular muscles only - Grade 2: mild weakness affecting muscles other than ocular muscles > 2A: limbs/ axial muscles > 2B: respiratory and bulbar muscles - Grade 3: moderate weakness - Grade 4: severe weakness - Grade 5: intubation required Osserman's grading I: ocular II A: mild generalised with slow progression II B: Moderate generalised III: acute fulminant MG IV: late severe MG (Takes 2 years to progress from I to II)

Question 97

Management of myasthenia gravis?

Answer 97

crisis: ABC approach tx exacerbating factor: stop exacerbating medications, treat infection if any, treat fever with antipyretics oral pyridostigmine steroids, azathioprine, cyclosporine (to maintain remission) plasmapheresis IVIG Thymectomy

Question 98

complications of myasthenia gravis?

Answer 98

myasthenic crisis - severe exacerbation of MG - 10% require intubation treatment complications - cholinergic crisis - cx of medications

Question 99

what is Lambert Eaton syndrome?

Answer 99

myasthenic disorder associated with malignancy such as small cell Lung Ca affects the proximal (thigh/pelvic girdle) and truncal musculature, bulbar muscles rarely involved improves with exercise presence of Abs to calcium channels

Question 100

approach to examination upon detection of facial asymmetry (CN VII palsy)

Answer 100

1. test CN VII functions: > look up (frontalis) > close eyes (orbuclaris oculi) attempt to open > look for exposure keratitis, tarsorraphy > nasolabial fold, show teeth, blow against closed lips > look for drooling of saliva 2. determine LMN or UMN, unilateral or bilateral 2A UMN unilateral: examine UL and look for hemiparesis on same side of the facial weakness check for xanthelasma, DM signs and BP 2B LMN unilateral - examine other CN - VI nerve and contralateral weakness in brainstem lesions - CPA lesion (V, VI, VII, VIII with cerebellar) - other CN involvement non comforming type: mononeuritis multiplex, MG 3. look at palate for vesicles 4. examine the parotids and for surgical scars 5. mastoid tenderness 6. examine neck for cervical LNs 7. Upper limbs: contralateral hemiparesis, ipsilateral cerebellar ask to examine: otoscopy for vesicles in ear canal and otitis media for hyperacusis for loss of taste in anterior 2/3 of tongue urine dip for glucose and BP (mononeuritis multiplex)

Question 101

if noted to have bilateral rather than unilateral facial weakness?

Answer 101

rule out MG rule out myotonic dystrophy or fascio scapular humeral dystrophy bilateral LMN VII: > look for V VI VIII > examine parotids (sarcoidosis, amyloidosis) > examine tongue (scrotal tongue for MR syndrome) > examine UL for GBS, MND, leprosy, lyme (radiculopathy) and bilateral cerebellar signs if suggestive of bilateral CPA tumours rare: melkerssson rosenthal syndrome, mobius syndrome

Question 102

what is the course of the facial nerve?

Answer 102

VII nerve nucleus lies in the pons in close proximity to VI nerve nuclei VII leaves the pons with VIII via cerebellopontine angle VII enters facial canal and enlarges to become the geniculate ganglion A branch is given off to the stapedius muscle and the greater superficial petrosal branch goes to the lacrimal glands the chorda tympani which supplies taste sensation to anterior 2/3 of tongue joins the VII nerve in the facial canal VII nerve exits the skull via stylomastoid foramen through the parotids and branches into 5: temporalis, zygomatic, buccal, mandibular, cervical

Question 103

what are the causes of bilateral LMN VII nerve palsies?

Answer 103

after ruling out MG and myopathies bilateral CPA tumour as in NF type 2 Bilateral Bell's palsy bilateral parotid enlargement (Sarcoidosis- uveoparotid fever or Heerfordt's fever) GBS, MND, Lyme disease Rare: Rosenthal Melkersson syndrome (triad of VII palsy with facial oedema and plication of the tongue), Mobius syndrome (congenital facial diplegia, oculoparalysis from III and VI and infantile nuclear hypoplasia)

Question 104

what is Bell's phenomenon?

Answer 104

refers to the upward movement of the right eyeball with incomplete closure of the right eyelid in an attempt to close the right eye

Question 105

why are the muscles of the upper face spared in an UMN lesion?

Answer 105

the upper facial muscles are preserved in an UMN lesion as there are bilateral cortical representations of these muscles

Question 106

what is bell's palsy?

Answer 106

an idiopathic facial paralysis, believed to be due to viral-mediated cranial neuritis from HSV typically presents with abrupt onset weakness with worsening the following day, associated with facial or retroauricular pain, hyperacusis and excessive tearing

Question 107

what is ramsay hunt syndrome?

Answer 107

- herpes zoster infection of the geniculate ganglion - presents with vertigo, hearing loss, facial weakness, pain in the ear with vesicles seen on the external auditory meatus and palate

Question 108

what is facial synkinesis?

Answer 108

attempt to move one group of facial muscles results in movement of another group occurs as a result of anomalous regeneration of the facial nerve e.g nerve fibres that innervate facial muscles innervating lacrimal glands -> tearing on mastication (crocodile tears)

Question 109

ix of facial nerve palsy?

Answer 109

target ix according to history and physical examination bloods e.g. Lyme disease Imaging

Question 110

Management of Bell's palsy?

Answer 110

- educate - lubricating eye drops, eye patch, taping eyes closed - physiotherapy Meds: PO pred 1mg/kg/d for 7-10 days and PO aciclovir 400mg 5x/d for 7 days (within first 72h) regular follow up to look for resolution and exclude new developing signs suggestive of other conditions surgical (tarsorraphy) for chronic non resolving cases

Question 111

how would you educate or counsel patient with Bell's palsy?

Answer 111

common condition course: improvement onset 10d - 2mo plateau 6w-9mo residual signs - synkinesis in 50% - face weakness 30% - contracture 20% - blepharospasm: may occur years after paralysis

Question 112

synkinesis in facial nerve - can be treated with?

Answer 112

synkinesis may be due to anomalous regeneration of nerve may be treated with botulinum

Question 113

what are associated signs with better prognosis in bells palsy?

Answer 113

incomplete paralysis early improvement slow progression younger age normal salivary flow normal taste normal NCS, electrogustometry

Question 114

examination approach to visual field defects

Answer 114

general inspection: acromegaly hemiparesis dysphasia visual fields - introduce - sit about arms length - "can you see my whole face" - test gross VA- counting fingers (wear specs!) - test gross visual fields using finger movements as well as for visual inattention - test using white hat pin from all quadrants

Question 115

if single eye defect for visual field, examination approach?

Answer 115

proceed with fundoscopy: BRAO, haemorrhages, chorioretinitis optic atrophy, glaucoma, RP

Question 116

single eye visual defect with constricted field (tunnel vision)

Answer 116

> chronic papilloedema > chronic glaucoma > retinitis pigmentosa > chorioretinitis > hysteria (visual field does not widen as object is brought further away from the patient compared to organic cause)

Question 117

single eye visual field defect: scotoma? causes

Answer 117

retinal haemorrhage or infarct (paracentral or peripheral scotomas) - does not cross the horizontal midline optic nerve (pale in atrophy, normal in retrobulbar neuritis, pink/swollen in papillitis) resulting in central scotoma - compression: tumour, aneurysm, paget's - glaucoma - neuritis: MS, ischaemia (temporal arteritis, idiopathic, C/BRAO), toxic (methanol), B12 deficiency, Hereditary (Friedrich's ataxia, LHON), 2' retinitis pigmentosa

Question 118

totally blind in one eye causes?

Answer 118

retina optic nerve lesion

Question 119

single eye altitudinal visual field defect (ie. superior or inferior)?

Answer 119

retinal infarcts ischaemic optic neuropathy

Question 120

bitemporal visual field defect causes

Answer 120

upper > lower= inferior chiasmal - pituitary tumour - suprasellar meningioma lower > upper - craniopharyngioma others: aneurysm, metastasis, glioma

Question 121

homonymous hemianopia - causes?

Answer 121

infarcts/ haemorrhages/ tumour left homonymous hemianopia = right cerebral lesion and vice versa incongruous: optic tract congruous: upper quadrantonopia: temporal lobe lower quadrant: parietal lobe macula sparing (test with red hat pin): occipital cortex no macula sparing: optic radiation note any DM dermopathy, xanthelasma. AF. hemiparesis (causes / RF for stroke)

Question 122

ix of bitemporal hemianopia?

Answer 122

- lateral skull XR: enlarged sella turcica, calcification for craniopharyngioma - CT or MRI head - formal field perimetry - serum prolactin - screen for hypopituitarism - IGF1: screen for acromegaly if suspected

Question 123

what signs to mention when patient has a unilateral homonymous hemianopia?

Answer 123

incongruous/ congruous macula sparing or not if any: hemiparesis, dysphasia, visual inattention look for CVRF- DM dermopathy, xanthelasma, AF tumour - cachexia, clubbing for metastatic disease request for full neurological examination to evaluate for stroke/ SOL

Question 124

ix of homonymous hemianopia?

Answer 124

CT head formal field testing, perimetry

Question 125

examination findings for right INO?

Answer 125

left eye can abduct with nystagmus, right eye fails to adduct on leftward gaze right eye is able to independently adduct saccadic eye movement - horizontal saccade is abnormal with the right eye lagging behind the left eye

Question 126

Internuclear ophthalmoplegia: what examination finding can help to neurolocalise?

Answer 126

convergence intact: pons convergence not intact: midbrain

Question 127

what else to examine for if internuclear ophthalmoplegia is picked up?

Answer 127

eye movements convergence proceed with other CN examination: - multiple sclerosis (RAPD) - myasthenia gravis limbs: - multiple sclerosis: may have cerebellar signs - CVA: DM dermopathy, xanthelasma, AF request for fundoscopy: optic atrophy

Question 128

what causes a right INO?

Answer 128

lesion is in the right medial longitudinal fasciculus that connects the ipsilateral third nerve innervation to the right medial rectus to the left gaze center

Question 129

causes of INO?

Answer 129

multiple sclerosis brainstem infarction pontine glioma infections: Lyme disease, syphilis, viral drug intoxication: TCAs, phenytoin, CMZ trauma myasthenia gravis (pseudo INO due to EOM weakness)

Question 130

ix of internuclear ophthalmoplegia?

Answer 130

as per possible aetiologies: MG MRI brain Lipid panel, fasting glucose Lyme disease titre VDRL drug levels

Question 131

what is wall eyed bilateral INO? (webino syndrome)

Answer 131

- due to bilateral MLF damage - bilateral INO with exotropia and failure of convergence - lesions in the pons and midbrain - due to MS/ brainstem infarct, gliomas, wernicke's

Question 132

what is fisher's one and a half syndrome?

Answer 132

ipsilateral horizontal gaze palsy (ie. both eyes can't look right) and ipsilateral INO (ie. left eye can look left, right eye cannot adduct) - due to lesion in MLF and adjacent gaze center

Question 133

causes of conjugate upward vertical gaze palsy?

Answer 133

midbrain lesion: MS (demyelination), vascular (infarct, haemorrhage, aneurysm), tumour

Question 134

causes of conjugate downward vertical gaze palsy?

Answer 134

lesion in midbrain or foramen magnum - arnold chiari malformation, dandy walker - acquired lesion (tumour, vascular, demyelination, abscess)

Question 135

features of parinaud's syndrome?

Answer 135

loss of vertigal gaze (usually upward gaze affected) + nystagmus on convergence (particularly with upward gaze and saccades upward) + light near dissocation (poor bilateral pupillary constriction in response to light, but preserved constriction with convergence) + bilateral lid retraction

Question 136

parinaud syndrome -causes?

Answer 136

lesions affecting structures in the dorsal midbrain most often: pineal region tumors, brainstem hemorrhage, and ischemic infarction MS

Question 137

features of progressive supranuclear gaze palsy in parkinsons plus disease?

Answer 137

loss of downward gaze, then upward gaze then horizontal gaze can be overcome by doll's reflex loss of saccadic (frontal lobe) and pursuit movements (occipital lobe)

Question 138

isolated CN III palsy - what to examine for upon completion of eye/ CN examination?

Answer 138

look for intortion of the affected orbit by asking patient to look down and medially of affected eye (if affected, implies 4th nerve palsy) rule out: thyroid eye disease, MG Superior orbital syndrome and cavernous sinus syndrome proceed with - neck for LNs - examine upper limbs for cerebellar, hemiplegia, EPSE, areflexia - look for DM dermopathy

Question 139

isolated CNIII palsy: what to request for on completion of examination?

Answer 139

- corneal reflex (Reduced or absent) - visual fields (bitemporal hemianopia) - fundoscopy for optic atrophy (MS), DM or hypertensive changes - visual acuity - blood pressure - urine dipstick - temperature chart - headache or pain

Question 140

what is the course and anatomy of the 3rd CN?

Answer 140

- nuclear portion at the midbrain - fasicucular intraparenchymal portion: close to the red nucleus, emerges from the cerebral peduncle - fascicular subarachnoid portion: meninges, PCA aneurysm - fasiculular cavernous sinus portion: sella turcica between the petroclinoid ligament below and interclinoid above - fascicular orbital portion: superior orbital fissure

Question 141

what are the causes of an isolated 3rd nerve palsy?

Answer 141

Brainstem: - infarct, haemorrhage, tumour, abscess, MS peripheral: - subarachnoid portion: PCA aneurysm, meningitis, infiltrative - cavernous sinus lesions - orbital - mononeuritis multiplex, MG, Miller fischer

Question 142

features of a third nerve nucleus lesion?

Answer 142

will also have contralateral ptosis and elevation palsy as axons from the third nerve nucleus also innervate the contralateral superior rectus and the contralateral levator palpebrae

Question 143

emergency causes of third nerve palsy?

Answer 143

coning (raised ICP) giant cell arteritis aneurysm

Question 144

how would a patient present with thrid nerve palsy? (symptoms)

Answer 144

diplopia ptosis symptomatic glare from failure of constriction of pupil blurring of vision on attempt to focus of near objects due to loss of accommodation pain in certain aetiologies: DM, PCA aneurysm, migraine

Question 145

causes of a dilated pupil?

Answer 145

- III nerve palsy - optic atrophy (direct light and accommodation absent with intact consensual reflex) - Holmes Adie Pupil (myotonic pupil) - Mydriatic eye drops - Sympathetic overactivity

Question 146

why does a PCA aneurysm result in pupillary involvement whereas conditions such as DM or hypertension spares the pupil?

Answer 146

the pupillary fibres are situated superficially and prone to compression whereas ischaemic lesions tends to affect the core of the nerve thus sparing the pupillary fibres

Question 147

ix of isolated CN III palsy?

Answer 147

imaging: CT, MRI Angiogram Bloods: Fasting blood glucose ESR TFT screen for myasthenia if suspected LP

Question 148

mx of 3rd nerve palsy?

Answer 148

medical third nerve palsy - education: watchful waiting, avoid driving/ heavy machinery/ climbing high places - treat underlying conditions such as DM/ hypertension - most spontaneously recover within 8 weeks - symptomatic treatment: NSAIDs for pain, eye patch for severe diplopia and prism glasses for mild diplopia surgical 3rd nerve palsy - surgery

Question 149

Bilateral foot drop in examination, with UMN pattern of weakness. Causes?

Answer 149

Cord lesion Parasagittal lesion ACA territory stroke Bilateral cortical / sub cortical lesion

Question 150

Bilateral foot drop in examination, with LMN pattern of weakness. Causes?

Answer 150

Peripheral neuropathy Multifocal motor neuropathy HSMN/ Charcot Marie tooth

Question 151

Unilateral foot drop on examination, UMM pattern of weakness. Causes?

Answer 151

Cortical or hemicord lesion

Question 152

Unilateral foot drop, LMN pattern of weakness. Causes?

Answer 152

Need to differentiate between Common peroneal nerve palsy L5 plexopathy Sciatic nerve palsy

Question 153

Unilateral foot drop with LMN pattern of weakness -> what to assess for next?

Answer 153

Knee flexion -> if weak knee flexion: sciatic neuropathy * beware complex polyradiculopathy, lumbosacral plexopathy, polyneuropathy, myopathy, peripheral neuropathy -> if knee power normal, then move on to differentiate between common peroneal nerve palsy and L5 radiculopathy

Question 154

Features of sciatic neuropathy?

Answer 154

Weak knee flexion (hamstrings) - knee extension and hip adduction normal Weak ankle dorsi and plantar flexion-> foot drop Weak foot inversion and eversion Wasting of peroneus and anterior tibialis muscle Loss of ankle reflex and plantars absent, knee jerk intact Everything below knee except medial aspect of leg and foot may be numb

Question 155

Unilateral foot drop with LMN pattern of weakness, knee flexion normal. What to examine for next?

Answer 155

Hip abduction Hip internal rotation Foot inversion -> if weak: L4-5 radiculopathy -> if normal: peroneal nerve

Question 156

general features of Parkinsonism in UL and general inspection?

Answer 156

mask like facies, monotonous speech, dyskinesias resting tremors which disappears with use of hand bradykinesia leadpipe rigidity and cogwheeling acute dystonia or alien limb syndrome pronator drift and cerebellar signs

Question 157

features of parkinsonism in the face?

Answer 157

- eye movements, vertical dolls if vertical gaze impaired - close eyes for blepharospasm - feel for seborrhoea - KF rings - count 1-20

Question 158

features of parkinsonism in gait?

Answer 158

shuffling gait with festination stooped posture

Question 159

parkinsonism: how to test for function on examination?

Answer 159

unbutton shirt, cap a pen, comb hair

Question 160

parkinsonism: how to request to complete examination?

Answer 160

speech if not done swallowing handwriting postural BP AMT

Question 161

Parkinonism: what important negatives to present?

Answer 161

Parkinsons Plus features - no evidence of Supranuclear gaze palsy ie. vertical gaze palsy - no evidence of cerebellar signs to suggest multisystem atrophy - no evidence of corticobasal ganglia degeneration such as dystonic arm or alien limb syndrome - no evidence of dyskinesias which can result from L-dopa therapy

Question 162

what is parkinson's disease?

Answer 162

it is a progressive neurodegenerative disorder associated with degeneration of the dopaminergic nigostriatal neurons - clinical diagnosis with 2 out of 3 signs comprising of resting tremors, bradykinesia and rigidity. 4th sign of postural instability occurs later in the course of disease

Question 163

causes of Parkinsonism?

Answer 163

- Parkinson's disease - Parkinson Plus syndrome - Drugs: Dopamine antagonists, antiemetics - Anoxic brain damage (post cardiac arrest, CO, Manganese) - Post encephalitis - Tumour such as giant frontal meningioma

Question 164

pathological findings of Parkinsons disease

Answer 164

loss of pigmented dopaminergic neurons in the substantia nigra presence of lewy bodies (eosinophilic cytoplasmic inclusions)

Question 165

What are the Parkinsons plus syndromes

Answer 165

1. Progressive supranuclear palsy 2. Multiple system atrophy 3. Corticobasalganglionic degeneration (fronto parietal lobe) 4. Diffuse Lewy Body disease 5. Parkinonism -dementia-ALS complex

Question 166

features of progressive supranuclear palsy?

Answer 166

vertical gaze palsy - downgaze affected first, then upgaze, then horizontal - can be overcome by vertical Doll's - other features such as blepharospasm, slow pursuit, saccadic eye movements postural instability and axial rigidity with falls early in the course of the disease frontal lobe signs

Question 167

features of multiple system atrophy?

Answer 167

MSA-P (Parkinsonism features) MSA-C (Cerebellar features) - Cerebellar signs - Autonomic features: orthostatic hypotension, urinary dysfunction, erectile dysfunction - Corticospinal signs: hyperreflexia and extensor plantar response

Question 168

features of corticobasal degeneration

Answer 168

limb apraxia or alien limb syndrome dystonia

Question 169

what is the significance of diagnosing parkinson plus syndrome?

Answer 169

poorer prognosis poor response to L dopa therapy

Question 170

what features suggest that patient may have parkinson plus syndromes?

Answer 170

- early onset dementia - presence of hallucinations or psychosis - early onset of postural instability - truncal symptoms more prominent than appendicular symptoms - marked symmetry of signs early in the stage of the disease - lack of response to levo dopa therapy in the early stage of the disease - presnce of signs and symptoms suggestive of parkinson plus syndrome

Question 171

what are the stages of parkinson's disease?

Answer 171

staged via the Hohen and Yahr staging system 1: symptoms and signs unilateral and mild 2: bilateral, mild disability 3: generalised dysfunction with significant bradykinesia and gait impairment 4: rigid and bradykinesia, severe symptoms with limited walking 5: completely invalid and requires nursing care

Question 172

ix of parkinsonism

Answer 172

MRI brain: to rule out NPH, multi infarct syndromes, frontal meningiomas, parkinson plus syndrome if patient is young ie <50, rule out wilson's disease: - slit lamp examination - serum caeruloplasmin and 24h urinary copper

Question 173

features of multiple system atrophy on MRI brain

Answer 173

atrophy of the cerebellum, brainstem "hot cross bun" sign: cruciform hyperintensity within the pons

Question 174

features of progressive supranuclear palsy on MRI brain?

Answer 174

atrophy and hyperintensity of the midbrain and red nucleus "hummingbird" sign: atrophy of the midbrain results in a profile of the brainstem (in the sagittal plane) in which the preserved pons forms the body of the bird, and the atrophic midbrain the head, with beak extending anteriorly towards the optic chiasm.

Question 175

features of corticobasal degeneration in MRI brain?

Answer 175

frontoparietal cortical atrophy

Question 176

Mx of parkinsonism?

Answer 176

MDT approach Education and counselling, PTOTST Medications: e.g. L dopa combined with peripheral decarboxylase inhibitor, Dopamine agonist Surgical

Question 177

Surgical options in Parkinon's?

Answer 177

Deep brain stimulation: for all features especially tremors Lesion surgery: thalomotomy for tremors or pallidotomy (may help reduce tremors, stiffness)

Question 178

medical management of parkinons?

Answer 178

dopamine agonist e.g. pramipexole or ropinirole - use in younger patients or early in course of disease - delay onset of motor fluctuations and dyskinesias L dopa therapy + peripheral decarboxylase inhibitor (Madopar) - should be used if there is disability Tremors: can consider anticholinergics e.g. benztropine Rigidity: can use MAO-B inhibitors COMT inhibitors

Question 179

side effect of dopamine agonists e.g. ropinirole, pramipexole

Answer 179

nausea, orthostatic hypotension, hallucinations, somnolence, oedema

Question 180

side effect of L dopa therapy?

Answer 180

peak dose dyskinesia and end of dose rigidity nausea, orthostatic hypotension, hallucinations

Question 181

how to manage peak dose dyskinesia?

Answer 181

- reduce the dose and increase frequency of L dopa - add on COMT inhibitors e.g. entacapone which increases half life of L dopa therapy - initiating with dopamine agonist and adding on L dopa therapy resulting in less motor fluctuations

Question 182

how to manage end of dose L dopa dyskinesia?

Answer 182

- increase dose, frequency - switch to CR formulations - add dopamine agonist, MAO-b Inhibitor or COMT inhibitor

Question 183

what is the prognosis of Parkinsons disease?

Answer 183

chronic progressive disease - some will have mild, while others will have severe symptoms - some will be troubled by tremors, others by rigidity and bradykinesia

Question 184

what is the abbreviated mental test?

Answer 184

used as a screening for possible dementia in the elderly a score of less than 6 warrants further assessment includes: age DOB remembering an address- 40 west street time year recognition of 2 people place prime minister first year of WW1 count backwards from 20 to 1

Question 186

features of ataxia telangiectasia?

Answer 186

auto recessive childhood onset with death by 20s or 30s ataxia, slurred speech, choreathetosis, telangiectasia on face, ears, conjunctiva and skin crease low IgA with recurrent sinopulmonary infections and lymphoreticular malignancy

Question 187

what strokes can cause cerebellar symptoms?

Answer 187

can be bleed or infarct ataxic hemiparesis: lacunar stroke cerebellar, posterior circulation stroke lateral medullary syndrome: ipsilateral horners, CN V, IX, X, cerebellar signs/ nystagmus, contralateral spinothalamic tract deficits Benedikt's syndrome (midbrain stroke): ipsilateral CN III + contralateral hemiparesis, cerebellar ataxia and choreiform movements

Question 188

what SOL may cause cerebellar signs

Answer 188

primary or secondary malignancy, abscess at the CPA (+/- neurofibromatosis) or cerebellum

Question 189

what congenital lesions may cause cerebellar signs?

Answer 189

arnold chiari malformation: downward displacement of cerebellar tonsils through the foramen magnum, which may cause CSF outflow obstruction and hydrocephalus dandy walker syndrome: congenital brain malformation with partial/complete agenesis of cerebellar vermis, and cystic dilatation of 4th ventricle

Question 190

parkinsonism + cerebellar signs?

Answer 190

multiple system atrophy

Question 191

findings of peripheral neuropathy, how to complete your examination?

Answer 191

gait if not already done: high steppage gait (sensory ataxia) UL for distal sensory impairment urine dipstick for glycosuria (DM) ask history: drug history, chronic ETOH ingestion

Question 192

approach to examination of patient with hemiparesis?

Answer 192

examine UL and LL aim is to locate the lesion of stroke: brainstem, subcortical, cortical - EOM - cortical signs: gaze preference, sensory or visual neglect, hemianopia and dysphasia if dominant lobe involved causes or risk factors: - pulse, carotid bruit, heart murmur - signs of hyperlipidaemia (xanthelasma, xanthomas) - DM dermopathy - tar stains - bruising, telangiectasia (?brain bleed) function and complications: - gait - pressure sores, NG tube, urinary catheter

Question 193

presentation of patient with hemiparesis?

Answer 193

this patient has a L/R hemiparesis as evidence by - state UL and LL findings - state level of lesion (brain stem, subcortical, cortical) and justify - causes/ risk factors - functional status and complications

Question 194

types of lacunar strokes (subcortical)?

Answer 194

pure motor (50%) pure sensory (5%) mixed motor and sensory (35%) ataxic hemiparesis (10%) dysarthria clumsy hand syndrome (rare)

Question 196

What is webers syndrome?

Answer 196

midbrain stroke ipsilateral CN III palsy and contralateral hemiplegia

Question 197

what is millard gubler syndrome

Answer 197

ventral pons lesion ipsilateral CN VI, VII palsy + contralateral hemiplegia +/- contralateral loss of propioception and light touch with medial lemniscal damage

Question 198

what to request for at end of examination of patient with hemiparesis?

Answer 198

- CBG reading - BP - ECG - fundoscopy for papilloedema (to rule out SOL which is a potential differential)

Question 199

DDx of patient with unilateral hemiparesis?

Answer 199

Vascular: ischaemic (80%): intracranial thrombosis, embolic, lacunar strokes, dissection; haemorrhagic Space occupying lesion: primary/ secondary malignancy Infective: abscess, meningoencephalitis seizures with Todd's paresis Toxic/metabolic: hypoglycaemia, hyponatraemia

Question 200

causes of ischaemic stroke?

Answer 200

intracranial atherosclerotic disease (ICAD) extracranial atherosclerotic disease (ECAD): stenosis of internal/common carotid artery small vessel disease (SVD): common for lacunar strokes embolic: - cardioembolic (LV thrombus, AF) - septic (IE) - paradoxical embolus Embolic stroke of undetermined source (ESUS) dissection e.g. vertebral artery dissection

Question 201

how does an anterior cerebral artery stroke present?

Answer 201

affects frontal lobe function: AMS, impaired judgment contralateral lower extremity weakness gait apraxia

Question 202

how does MCA stroke present

Answer 202

contralateral hemiparesis involving face/UL > LL with reduced sensation ipsilateral hemianopsia (blindness in one half of visual field) gaze preference towards lesion receptive or expressive aphasia if lesion in dominant hemisphere

Question 203

presentation of PCA stroke?

Answer 203

homonymous hemianopia cortical blindness (loss of vision due to problem with visual cortex rather than eyes) visual hallucinations visual agnosia altered mental status

Question 204

how does vertebrobasilar artery stroke present?

Answer 204

varied depending on CN, cerebellar, brainstem area affected vertigo, nystagmus, diplopia, VF defects, dysphagia, dysarthrits, ataxia contralateral hemiparesis ipsilateral pain and temp loss in face

Question 205

ix of stroke?

Answer 205

confirm the diagnosis: CT brain to rule out bleed +/- Angiogram MRI brain stroke protocol: to look for restricted diffusion on DWI (diffusion weighted imaging) sequence -> and to look for complications e.g oedema Bloods: FBC Coagulation, renal panel, liver function, CK CVRF Lipid panel, Hba1c, fasting glucose CYP2C19 if planning for long term clopidogrel use ECG: AF, MI 24h holter CXR: enlarged mediastinum suspicious of aortic dissection Echo: cardioembolic cause US carotids to look for ipsilateral stenosis >70%

Question 206

ix for young stroke workup?

Answer 206

transcranial doppler bubble study: to look for right to left shunts TTE with bubble Autoimmune/inflammatory screen: ANA, dsDNA, ANCA, ESR Antiphospholipid syndrome: anticardiolipin antibodies, LAC, anti-B2 glycoprotein Homocystine Infective screen: Syphilis HIV screen Thrombophilia screen: Thrombophilia: Protein C, S deficiency Anti Thrombin III Factor V Leiden D Dimer CT TAP / malignancy workup in selected cases

Question 207

What are the limitations of the CT brain for evaluation of acute stroke?

Answer 207

may be normal up to 6 hours of onset unable to visualise the posterior fossa structures such as brainstem and cerebellum well

Question 208

early signs on CT brain scan of stroke

Answer 208

loss of grey white differentiation insular ribbon sign sulcal asymmetry hyperdense MCA sign

Question 209

Management of stroke?

Answer 209

MDT approach: neuro, PTOTST Education and counselling Driving restrictions! Medications: antiplatelets, anticoagulation if cardioembolic Treat CVRF: HTN, DM, HLD Smoking cessation Surgical: intracranial bleeds, hydrocephalus carotid endarterectomy

Question 210

management of patient in acute stroke?

Answer 210

ABC approach If bleed, control BP. otherwise allow permissive hypertension up to 220/110 rule out bleed maintain euglycaemia, normothermia if ischaemic stroke, assess for possibility of thrombolysis or mechanical thrombectomy with use of NIHSS treat complications e.g. seizures, raised intracranial pressure, haemorrahagic transformation IV hydration

Question 211

neurological mimics of stroke?

Answer 211

seizures: todds paralysis migraine with aura: hemiplegic migraine structural lesion: subdural haematoma, tumour, AVM trauma/ head injury infection: abscess, encephalitis multiple sclerosis

Question 212

non neurological mimics of stroke?

Answer 212

metabolic: hypoglycaemia, uraemia, CO2 narcosis, electrolyte imbalances sepsis drugs: intoxication/withdrawal syncope labyrinthine disorder: vestibular neuritis traumatic/ musculoskeletal: hip/ knee contusion or fracture

Question 213

Rare causes of young strokes?

Answer 213

MELAS/ CADASIL/ CNS vasculitis: history of migraines SLE: malar rash, alopecia, joint pains Sjogrens Behcets: mouth ulcers Fabrys disease: Angiokeratomas, acroparesthesia, kidney disease malignancy: constitutional symptoms Recent neck trauma or manipulation: cervical artery dissection Young onset TIAs, strokes: moyamoya

Question 214

utility of NIHSS scoring

Answer 214

estimates stroke severity guides treatment decision enables uniform monitoring for progression

Question 215

features of higher cortical dysfunction

Answer 215

aphasia, visual/sensory neglect, apraxia, agnosia

Question 216

why is US carotid arteries not required for most posterior circulation strokes?

Answer 216

posterior circulation is supplied by vertebrobasilar system - exception would be presence of foetal origin of the PCA

Question 217

management of hyperacute stroke

Answer 217

stroke activation if within 6 hours of symptom discovery - thrombolysis if symptoms <4.5h ago - EVT (endovascular therapy) if <6h clinical and radiological criteria fulfilled

Question 218

BP management after thrombolysis/ EVT?

Answer 218

usually IV labetalol or nicardipine aim <180/105 post rTPA AIm SBP <160 post EVT

Question 219

antiplatelet therapy in stroke? when to consider DAPT

Answer 219

CHANCE trial: for mild strokes NIHSS <4, or high risk TIAs ABCD2 score 4 or more - DAPT 3/52 then lifelong SAPT SAMMPRIS trial: for ICAD, DAPT 3 months then lifelong SAPT

Question 220

when to delay starting antiplatelet therapy or anticoagulation therapy in stroke?

Answer 220

ischaemic stroke with parenchymal haemorrhage Malignant MCA infarct large stroke with surgical decompression

Question 221

when is carotid artery intervention indicated?

Answer 221

symptomatic carotid artery stenosis of >70% -> carotid artery endarterectomy, carotid artery stenting

Question 222

bilateral LL weakness, UMN pattern of weakness, normal sensation. causes?

Answer 222

MND subcortical (multiple strokes) parasagittal lesions (meningioma) hereditary spastic paraparesis

Question 223

Bilateral LL weakness, UMN pattern of weakness (Spastic paraparesis) with abnormal sensation (glove and stocking) causes

Answer 223

cervical myelopathy medical myelopathy (SACD, Tabes dorsalis)

Question 224

spastic paraparesis with cerebellar signs? causes

Answer 224

demyelinating disease: Multiple sclerosis Friedrich's ataxia Spinocerebellar degeneration Cervicomedually junction lesion

Question 225

spastic paraparesis with abnormal sensation but not glove and stocking?

Answer 225

test for sensory level: myelopathy, myelitis

Question 226

spastic paraparesis, UL normal, where is the lesion?

Answer 226

lesion between T1-L2 - look at back for scars - test for sensory level - test abdominal reflexes - Beevor's sign

Question 227

how to complete examination for spastic paraparesis?

Answer 227

Gait and assess function DRE for anal tone and saddle anaesthesia Scars and deformities of cervical spine UL, CN examination and examine for cerebellar signs

Question 228

causes of lesion between T1-L2?

Answer 228

compressive cord lesion (malignancy, infective, trauma) transverse myelitis (demyelinating, infective, inflammatory) anterior spinal artery occlusion: spinal infarct Subacute combined degeneration of the cord Tabes dorsalis

Question 229

spastic paraparesis + spastic UL, where is the lesion?

Answer 229

above C5 -> can do jaw jerk next if jaw jerk normal: lesion is between pons and C5 if exaggerated, lesion is above mid pons then ideally check CN, cerebellar signs

Question 230

causes of spinal cord lesion above mid pons? with exaggerated jaw jerk

Answer 230

pseudobulbar palsy MND bilateral CVA SOL demyelinating disease syringobulbia

Question 231

causes of spinal cord lesion mid pons-C5 causing spastic paraparesis in all four limbs, with normal jaw jerk?

Answer 231

compressive cord lesions (malignancy, infection, trauma) cervical myelopathy transverse myelitis syringomyelia Subacute combined degeneration of the cord tabes dorsalis spinal infarct (anterior spinal artery occlusion) Friedreich's ataxia

Question 232

spastic paraparesis + UL flaccid, where is the lesion?

Answer 232

LMN lesion at cervical cord -> check CN for palsies

Question 233

causes of spastic paraparesis with flaccid UL (ie. LMN lesion at cervical cord)?

Answer 233

MND Cervical myeloradiculopathy Syringomyelia Cervicomedullary lesion

Question 234

neuro examination: how to comment on one's functional status?

Answer 234

use of mobility aids: wheelchair, stick orthotic shoes gait

Question 235

what is Friedreich's ataxia?

Answer 235

autosomal recessive disease hereditary ataxia causing spinocerebellar degeneration - usually present as teens, inability to walk by 20s - pes cavus, distal wasting, spastic LL (pyramidal weakness pattern) - gait and limb ataxia, dysarthria (Cerebellar signs) - loss of ankle +/- knee reflexes (degeneration of peripheral nerves) - dorsal column loss (marked loss of cells in the dorsal root ganglion) - scoliosis

Question 236

spastic paraparesis with absent ankle +/- knee jerks causes

Answer 236

fasciculations: MND Cerebellar: Spinocerebellar degeneration (Friedreich's ataxia) Dorsal column loss: SACD, taboparesis conus medullaris lesion combined pathologies: cord compression + pre-existing peripheral neuropathy e.g. DM and cervical myelopathy

Question 237

spastic paraparesis + dorsal column loss (loss of propioception and vibration sense), causes?

Answer 237

spinocerebellar degeneration multiple sclerosis SACD, taboparesis

Question 238

features of MS to examine for if you suspect Multiple sclerosis

Answer 238

RAPD, INO, Optic neuropathy

Question 239

Spastic paraparesis and inverted supinator jerk positive in UL?

Answer 239

C5-6 lesion

Question 240

what is spinocerebellar ataxia?

Answer 240

inherited disorder with multiple subtypes >20 cx by cerebellar and spinal degeneration, slowly progressive with atrophy of cerebellum

Question 242

Friedreichs ataxia- complications?

Answer 242

spine - scoliosis Cardio: HOCM, AF Fundoscopy: optic atrophy Urinalysis for glycosuria intellectual deterioration

Question 243

Friedreich's ataxia; differential diagnosis for spasticity, cerebellar and dorsal column loss?

Answer 243

multiple sclerosis - usually has inceased reflexes compared to friedreich's ataxia which has absent reflexes

Question 244

what is subacute combined degeneration of the cord?

Answer 244

progressive disorder due to Vit B12 deficiency with degeneration of the corticospinal tracts and dorsal column of the spinal cord

Question 245

causes of B12 deficiency

Answer 245

low intake, vegan impaired absorption: - gastrectomy - pernicious anaemia: anti-IF, anti-parietal cell antibody - small intestines: crohns, coeliac, SIBO

Question 246

ix of subacute combined degeneration of the cord?

Answer 246

FBC - macrocytic anaemia B12, folate Anti-IF, parietal cell antibodies Schilling test screen with OGD if pernicious anaemia, or higher risk of gastric Ca MRI spinal cord- degeneration of lateral corticospinal tracts and dorsal columns

Question 247

management of subacute combined degeneration of the cord?

Answer 247

B12 replacement - symptoms may recover if treated early, otherwise damage is usually permanent

Question 248

what is taboparesis?

Answer 248

neurosyphilis infection - caused by treponema pallidum infection

Question 249

features of taboparesis?

Answer 249

spasticity with dorsal column loss (high steppage gait) absent ankle jerks charcot joints, trophic ulcers LL before UL affected incontinence, constipation argyll-robertson pupils

Question 250

stages of syphilis infection?

Answer 250

primary: painless chancre secondary: maculopapular rash, acute syphilitic meningitis tertiary: neurosyphilis, cardio syphilis, gummatous syphilis

Question 251

ix of taboparesis?

Answer 251

TPHA: specific once positive, will remain positive even after treatment RPR: non specific, more to monitor treatment and reinfection

Question 252

management of taboparesis?

Answer 252

penicillin symptomatic management: parasthesiae: analgesia, carbamazepine ataxia: PTOT Bladder: avoid anticholinergics, self catheterisation

Question 253

what does MND not involve?

Answer 253

no sensory deficit no ccerebellar disorder no ocular involvement

Question 254

what is lhermittes phenomenon

Answer 254

tingling or electric sensation that passes down the spine with flexion of the neck causes include: MS, cervical myelopathy (reversed lhermitte's - occurs on extension of neck), SACD

Question 255

what causes fasciculation?

Answer 255

sign of LMN disease with denervation spontaneous firing of large motor units formed by branching fibres of surviving axons striving to innervate the muscles that have lost their nerve supply

Question 256

clinical features of cervical myelopathy?

Answer 256

spasticity exceeds weakness, symptoms exceeds signs look for wasting and weakness of C5/6 as this is the most common area for spondylosis passive abduction of the little finger (myelopathy hand sign) inverted supinator jerk sensory - often no sensory loss, but may have loss of propioception and vibration

Question 257

generic neurolocalisation groups

Answer 257

muscle NMJ nerve anterior horn cell spinal cord brain/ brainstem

Question 258

myopathies causing flaccid paraparesis?

Answer 258

inflammatory myopathy (poly, dermatomyositis) rhabdomyolysis periodic paralysis (hypoK) metabolic derangements (hypomg, hypoCa, HypoPO4) thyroid or steroid myopathy acute alcoholic necrotizing myopathy

Question 259

NMJ causes of flaccid paraparesis?

Answer 259

myasthenia gravis botulism tick paralysis organophosphate toxicity LEMS: lambert eaton

Question 260

nerve causes of flaccid paraparesis?

Answer 260

GBS multifocal motor neuropathy Lyme disease, HIV Sarcoidosis drugs, toxins vasculitis including lupus, polyarteritis nodosa paraneoplastic, paraproteinaemias Cauda equina syndrome brachial, lumbosacral plexopathy

Question 261

anterior horn cell disease causing flaccid paraparesis?

Answer 261

poliomyelitis kennedys disease (spinobulbar atrophy) MND other spinomuscular atrophies

Question 262

spinal cord lesions causing flaccid paraparesis?

Answer 262

Affecting anterior horn cells (LMN) inflammatory: transverse myelitis Spinal cord infarct

Question 263

brain/brainstem causes of flaccid paraparesis?

Answer 263

pontine lesions e.g central pontine myelinolysis

Question 264

Features of poliomyelitis?

Answer 264

Usually would present with the paralytic form in paces Flaccid Paralysis and wasting in bulbar or spinal distribution

Question 265

Pathophysiology of poliomyelitis

Answer 265

Polio virus is an enterovirus spread via oro faecal route or contaminated water Replicates in the nasopharyngeal and GIT then haematological spread with predilection to the anterior horn cells of the spinal cord or brain stem with flaccid paralysis in spinal or bulbar distribution

Question 266

Ix of polio

Answer 266

Viral cultures or PCR from stool, throat, csf Poliovirus antibodies

Question 267

Management of polio

Answer 267

Education and counselling PTOT Care of limbs Medical: treat complications Eg pain, respiratory failure, clear bowels

Question 268

Management of polio

Answer 268

Education and counselling PTOT Care of limbs Medical: treat complications of pain, respiratory failure, clear bowels

Question 269

Prevention of polio

Answer 269

Inactivated polio vaccine- Salk vaccine which is administered parenteral Oral live vaccine- can result in poliomyelitis so contraindicated in immunodeficient individuals

Question 270

What is spina bifida?

Answer 270

Incomplete closure of the bony vertebral canal Usually in lumbosacral region, can also involve the cervical region and associated with hydrocephalus

Question 271

Features of spina bifida?

Answer 271

- scars, tufts of hair, dimples, sinus, naevus, lipoma - asymmetric LMN signs of LLs

Question 272

Features of spina bifida?

Answer 272

- scars, tufts of hair, dimples, sinus, naevus, lipoma - asymmetric LMN signs of LLs - L5, S1 dermatomal loss - bladder involvement

Question 273

Causes of spina bifida

Answer 273

Multifactorial aetiologies: folic deficiency, maternal use of sodium valproate, family history of spina bifida Prevented with use of folic acid in early pregnancy

Question 274

Features of cauda equina syndrome

Answer 274

Low back pain Unilateral/ bilateral sciatica Saddle anaesthesia Bladder and bowel disturbances Variable motor and sensory LL abnormalities

Question 275

Features of cauda equina syndrome on examination?

Answer 275

Bilateral knee and ankle jerks absent Flaccid paralysis, asymmetrical Saddle anaesthesia Sphincter disturbance occurs late

Question 276

Features of conus medullary lesion on examination

Answer 276

Spastic paraparesis, symmetrical More LBP Knee jerks preserved, ankle absent Perianal loss of sensation Frequent impotence Sphincter disturbance early

Question 277

Features of guillain barre syndrome

Answer 277

Progressive ascending muscle weakness Variable patchy sensory loss Hyporeflexia Autonomic disturbances such as tachycardia and labile bp Complications: Respiratory failure

Question 278

Features of L5 radiculopathy?

Answer 278

Weakness of hip abduction and internal rotation, Weak foot dorsiflexion (foot drop) loss of foot inversion and eversion plantar flexion preserved Loss of ankle reflex Sensory loss over dorsum of foot and lateral calf + big toe

Question 279

Features of common peroneal nerve palsy?

Answer 279

Weak ankle and toe dorsiflexion, foot eversion Wasting of anterior tibialis muscles Foot inversion and plantar flexion normal (innervated by posterior tibial nerve) Reflexes all normal Sensory loss over lateral calf and dorsum of foot

Question 280

Features of palsy of deep peroneal nerve

Answer 280

Deep branch supplies the ankle + toe dorsiflexion (foot drop) - eversion spared (supplied by superficial branch) Normal reflexes Sensory loss only over first interdigital web space

Question 281

Causes of common peroneal nerve palsy?

Answer 281

Trauma Surgery Compression at fibula head Mononeuritis multiplex Leprosy CIDP Ischaemia/ vasculitis

Question 282

How to complete examination in patient with foot drop?

Answer 282

Gait Walking aids Look for cause of foot drop (scars, fibular head lesion, spinal lesions) Offer Dre to assess for anal tone and spinal anaesthesia

Question 283

What motor function does the posterior tibial nerve control ?

Answer 283

Foot inversion and plantarflexion

Question 284

In common peroneal nerve palsy, need to differentiate further how to differentiate between common peroneal nerve or deep branch only or superficial branch only

Answer 284

Superficial branch: weak foot eversion, sensation lost over lateral calf and dorsum of foot Deep: weak ankle and big toe dorsiflexion, sensation lost over first interdigital web space

Question 285

Ix common peroneal nerve palsy?

Answer 285

NCS and EMG

Question 286

Mx of common peroneal nerve palsy?

Answer 286

PTOT- 90 degrees splint at night Surgery: for severed nerve or excision of ganglion

Question 287

How are reflexes affected in sciatic nerve palsy?

Answer 287

l4, L5, S1/2 Knee jerk intact Ankle jerks and plantar response absent

Question 288

How are reflexes affected in L5 radiculopathy?

Answer 288

Loss of ankle reflex

Question 289

What differentiates L5 nerve root compression from common peroneal nerve palsy

Answer 289

l5 nerve root, Hip abduction and IR affected Foot inversion affected Loss of ankle reflex

Question 290

What differentiates sciatic nerve palsy from common peroneal nerve palsy?

Answer 290

Loss of ankle reflex and plantars absent Knee flexion weak Foot plantarflexion weak

Question 291

Features of anterior cord syndrome?

Answer 291

Ventral 2/3 of spinal cord affected Dorsal columns spared Bilateral weakness with LMN signs at level of lesion and UMN signs distal to lesion Bilateral loss of pin prick and temp sense -> commonly seen in anterior spinal artery infarct

Question 292

Features of complete transection of spinal cord eg cord trauma/ transverse myelitis

Answer 292

LMN signs at level of lesion UMN signs caudal to level of lesion

Question 293

dorsal columns + corticospinal tracts affected?

Answer 293

Subacute combined degeneration of the cord

Question 294

Dorsal columns + corticospinal tracts + spinocerebellar tracts affected?

Answer 294

Spinocerebellar degeneration: Proprioception and vibration sensory loss weakness Cerebellar dysfunction

Question 295

Central cord syndrome eg syringomyelia, hydromyelia, tumour. Features

Answer 295

Usually bilateral weakness with LMN signs at level of lesion and UMM signs caudal to level of lesion (spastic paraparesis) Loss of pin prick and temp sense with sparing of dorsal columns

Question 296

Causes of dorsal column loss of function ?

Answer 296

Tabes dorsalis DM B12 deficiency Local lesion trauma infarct tumour haemorrhage Posterior spinal artery infarct-> would cause ipsilateral dorsal column dysfunction

Question 297

Brown sequard syndrome features

Answer 297

Ipsilateral weakness Ipsilateral loss of proprioception and vibration Contralateral loss of pain and temp Usually anaesthesia at level of lesion and area of hyperaesthesia just below level of lesion

Question 298

Causes of brown sequard syndrome

Answer 298

Syringomyelia Cord tumour Trauma: Bullet/ stab wounds, haematomyelia Degenerative disease of spine: cervical spondylosis Multiple myeloma Infection: abscess Immune: MS

Question 299

What are the different types of movement disorders?

Answer 299

Tremors Chorea Athetosis Hemiballismus Orofacial dyskinesias

Question 300

What are the different types of tremors you may see?

Answer 300

Resting tremors in Parkinsonism Intention tremor of cerebellar Postural tremor of outstretched hands (anxiety, thyrotoxicosis, ETOH withdrawal, alcohol, drug induced ie salbutamol/ theophylline, Bzd withdrawal, familial)

Question 301

Causes of hemiballismus

Answer 301

Subthalamic nucleus affected - infarct - abscess, tumour, AVM, MS

Question 302

Chorea occurs when which part of the brain is affected?

Answer 302

Globus pallidus Can be stroke/ SOL/ MS etc

Question 303

spastic paraparesis - what are the 3 things to test?

Answer 303

1) sensation: normal? Glove and stocking loss? Sensory level? 2) cerebellar signs 3) UL: pronator drift, tone, reflexes

Question 304

spastic paraparesis with dorsal column loss but pinprick sensation intact?

Answer 304

spinocerebellar degeneration MS SACD taboparesis

Question 305

young patient pes cavus, distal wasting, spastic LL (pyramidal weakness pattern) gait and limb ataxia

Answer 305

Friedreich's ataxia

Question 306

differential for spasticity, cerebellar, dorsal column loss

Answer 306

freidreichs ataxia: usually absent knee +/- ankle jerks multiple sclerosis: usually assoc with increased reflexes

Question 307

unilateral foot drop, LMN pattern of weakness, both knee flexion and extension are weak

Answer 307

Anterior horn cell polyradiculopathy lumbosacral plexopathy

Question 308

how to differentiate between plexopathy and radiculopathy?

Answer 308

Nerve conduction studies and needle electromyography are particularly helpful in distinguishing radiculopathy from plexopathy. Sensory nerve action potentials are generally abnormal in plexopathy, but spared in most radiculopathies.

Question 309

approach to a unilateral ptosis?

Answer 309

first to rule out pseudoptosis (droopy eyelids) see if there is a horner's syndrome (miosis)

Question 310

unilateral Horner's syndrome - examine the other cranial nerves to look for? potential causes

Answer 310

Cavernous sinus syndrome Superior orbital syndrome Lateral medullary syndrome Syringobulbia (V, VII, IX-> XII) Multiple sclerosis (INO, RAPD, cerebellar)

Question 311

unilateral horner's syndrome what to examine in the upper limbs?

Answer 311

- pronator drift then cerebellar signs (lateral medullary syndrome) - Wasting of ipsilateral small muscles of hands (T1) - Clubbing - Sensory loss T1 - Dissociated sensory loss (syringomyelia) - Contralateral loss to pain and temperature (lateral medullary syndrome) - axilla: trauma to brachial plexus

Question 312

unilateral horner's syndrome: what to request for for patient history?

Answer 312

loss of sweating and level

Question 313

main differentials for bilateral ptosis?

Answer 313

1) Muscle: - Myopathy e.g. myotonic dystrophy, CPEO, ocular myopathy, oculopharyngeal myopathy - Local infiltration 2) NMJ: Myasthenia gravis 3) III nerve bilaterally: GBS/ Miller fisher syndrome, Midbrain (Nuclear III) bilateral horner's (syringomyelia)

Question 314

patient with suspected myasthenia gravis, what to examine for in the eyes?

Answer 314

ptosis with fatiguability variable strabismus and diplopia that occurs after some time check for hyperthyroid and thyroid eye disease check for anaemia check for malar rash of SLE

Question 315

patient with suspected myasthenia gravis, what to examine for in the face?

Answer 315

VII- show teeth assessment of speech: count 1-20, nasal voice (bulbar palsy) masseter weakness but pterygoids normal check neck for goitre and scars

Question 316

patient with suspected myasthenia gravis, what to examine for in the ULs?

Answer 316

normal deep tendon reflexes (Lambert eaton and miller fisher assoc reduced reflexes) normal sensation fatiguability with weakness signs of RA, SLE features

Question 317

patient with suspected myasthenia gravis, what to examine for in the chest?

Answer 317

thymectomy scar plasmapheresis line

Question 318

associated conditions of myasthenia gravis

Answer 318

endocrine: thyroid, DM, pernicious anaemia Connective tissue disorders: RA, SLE, polymyositis

Question 319

myasthenia gravis: how to complete your examination?

Answer 319

checking negative inspiratory force, forced vital capacity temperature chart drug history

Question 320

signs of a pancoast tumour causing ipsilateral horners syndrome

Answer 320

supraclavicular dullness tracheal deviation small muscles of hands -wasting loss of pain sensation in UL clubbing

Question 321

features of isolated VI nerve palsy?

Answer 321

failure of abduction of affected eye convergent strabismus at primary gaze diplopi where image is side by side and further on gaze at affected size, with disappearance of the outer image on covering the affected eye -> suggests lateral muscle weakness and VI nerve palsy

Question 322

isolated CN VI nerve palsy? what to perform on examination

Answer 322

- eye examination: rule out MG, thyroid eye disease - rule out cavernous sinus/ superior orbital fissure syndrome - check other CN for CPA tumour (V, VII, VIII), base of skull lesions (XI, X, XI, XII) examine upper limbs: hemipleigia (long tract signs suggesting brainstem) cerebellar signs (CPA lesion, Miller fisher usually truncal and gait ataxia) reflexes for areflexia (miller fisher)

Question 323

CN VI palsy what to request for on completion of examination?

Answer 323

fundoscopy: papilloedema from raised ICP, optic atrophy for MS, DM or hypertensive retinopathy field testing (bitemporal hemianopia) acuity (reduced in orbital lesions) corneal testing for reduced sensation from V1 involvement (cavernous sinus/ superior orbital syndrome) BP urine dipstick Fever chart- meningitis Ask for retrobulbar pain

Question 324

causes of unilateral VI nerve palsy?

Answer 324

brainstem (pons): infarct, haemorrhage, demyelination (MS), abscess meningitis CPA lesions cavernous sinus / superior orbital syndrome miller fisher syndrome mononeuritis multiplex DM, HTN MG raised ICP most commonly: ischaemic CN VI palsy in adults

Question 325

causes of bilateral CN VI nerve palsy?

Answer 325

leptomeningeal causes: leptomeningeal carcinomatosis, NPC with secondaries, lymphoma, radiotherapy miller fisher syndrome mononeuritis multiplex MG raised ICP wernicke's encephalopathy (opthalmoplegia, confusion, ataxia)

Question 326

central syndromes associated with VI nerve palsy?

Answer 326

raymond's syndrome: ipsilateral VI with contralateral hemiparesis millard-gubler: ipsilateral VI and VII with contraleteral hemiparesis

Question 327

peripheral syndromes associated with VI nerve palsy?

Answer 327

CPA (V, VI, VII, VIII + cerebellar) superior orbital (III, IV, VI + V1) Cavernous sinus (III, IV, VI + V1 and 2) Gradenigo syndrome: inflammation of the tip of the petrous bone from mastoiditis; VI, V and VII

Question 328

how to evaluate diplopia?

Answer 328

1. double vision is maximal in the direction of gaze in the affected muscle 2. false image is the outer image 3. false image arises from the affected eye

Question 329

what is cavernous sinus syndrome?

Answer 329

lesion in the cavernous sinus leading to: ophthalmoplegia, V1, v2, Horners, proptosis, chemosis, pain

Question 330

causes of cavernous sinus syndrome

Answer 330

carotid aneurysm, carotid-cavernous fistula, tumour, thrombosis, tolosa hunt syndrome

Question 331

what is Miller fisher syndrome?

Answer 331

triad of ophthalmoplegia, ataxia, areflexia - presence of antiGQ1B antibodies - variant of GBS

Question 332

causes of mononeuritis multiplex

Answer 332

endocrine: DM, HTN Autoimmune: GPA, eGPA, PAN, Sjogrens, SLE, RA Infective: lyme, leprosy Infiltrative: amyloid, sarcoid

Question 333

ix of VI nerve palsy?

Answer 333

Bloods: Fasting glucose, ESR, ANA, VDRL Imaging; CT/MRI brain vascular imaging if proptosis/chemosis looking for cavernous sinus thrombosis or dural carotid-cavernous fistula LP: if bilateral/systemic illness 8-30% remain cryptogenic after workup

Question 334

management of CN VI palsy?

Answer 334

education avoid driving/ operating heavy machinery treat underlying cause: e.g. DM/ HTN symptomatic treatment: patch, prism if chronic, chemodenervation with botox, or strabismus surgery

Question 335

prognosis for CN VI palsy

Answer 335

most either spontaneously improve or have underlying lesion found ischaemic palsies almost always recover completely in 2-4 months while some recovery seen in half of traumatic cases if no recovery over 3-6 months then suspect underlying lesion such as tumour - > follow regularly looking for emergence of new localising signs, ensure adequate neuroimaging and ENT evaluation

Question 336

"examine this patient's speech" -> what to look for

Answer 336

look for unilateral weakness ataxia of UL (cerebellar) tremors (Parkinsonism) Dysphonia: recurrent laryngeal nerve involvement - offer to examine left chest, radiation marks, enlarged cervical LNs, look for horners and wasting of T1 Dysarthria: - British constitution, Bababa, lalala, kakaka Dysphasia: expressive, receptive (give 1,2,3 step instructions), conductive, global if dysphasia found: demonstrate other cortical signs -> visual fields, sensory or visual inattention, graphaesthesia, astereognosis

Question 337

"examine this patient's cortical function"

Answer 337

evaluate for obvious gaze preference or hemiparesis test which side is affected with inattention: visual inattention sensory inattention line bisection test test visual fields for hemianopia test for dysphasia look for pronator drift test functions of each lobe aetiology: pulse, carotid bruit, murmur, hyperlipidaemia, DM dermopathy, tar stains request for BP, urinary analysis for DM, fundoscopy (papilloedema)

Question 338

test for occipital lobe function

Answer 338

cortical blindness hemianopia with macula sparing

Question 339

test for temporal lobe function

Answer 339

superior quadrantonopia receptive dysphasia short and long term memory

Question 340

test for parietal lobe function

Answer 340

gerstmanns: acalculia (serial 7 subctraction), agraphia (inability to write) L-R disorientation finger agnosia constructional (copy a cub) and dressing apraxia, spatial neglect

Question 341

test for frontal lobe function

Answer 341

expressive dysphasia labile emotion, personality changes gait apraxia

Question 342

esotropia ->

Answer 342

6th nerve palsy

Question 343

exotropia

Answer 343

3rd, 4th nerve palsy

Question 344

complex ophthalmoplegia causes

Answer 344

central: INO, wernickes nerves: miller fisher NMJ: MG Myopathies: thyroid eye disease, CPEO

Question 345

LMN unilateral facial droop + CN 5th/ 6th and 8th involvement?

Answer 345

cerebellopontine angle lesion

Question 346

LMN unilateral facial droop + 8th CN palsy?

Answer 346

internal auditory canal lesion

Question 347

LMN unilateral facial droop + cerebellar signs

Answer 347

lateral medullary syndrome or middle cerebellar peduncle

Question 348

LMN unilateral facial droop + long tract signs

Answer 348

pontine lesion

Question 349

UMN facial droop + long tract signs, neurolocalisation

Answer 349

pontine and above

Question 350

cranial nerve examination, how to complete examination

Answer 350

fundoscopy visual field defect proper assessment of visual acuity with snellen chart check corneal reflex- afferent CN V1, efferent VII check gag reflex- afferent CN IX, efferent X

Question 351

CN palsies single or multiple: how to work through?

Answer 351

1) part of CN club? ie. cavernous sinus 2) base of skull/meningeal disease 3) polyneuropathy: GBS/ MFS 4) brainstem (except for II)

Question 352

Unilateral CN III, IV, VI + V1 + II optic nerve palsy

Answer 352

orbital apex syndrome

Question 353

Unilateral CN III, IV, VI + V1 palsy

Answer 353

superior orbital fissure syndrome

Question 354

Unilateral CN III, IV, VI + V1 and V2 palsy

Answer 354

Cavernous sinus syndrome

Question 355

Ipsilateral horners, CN V, IX, X, cerebellar, nystagmus contralateral pain and temp

Answer 355

lateral medullary syndrome

Question 356

ipsilateral CN XII palsy + contralateral dorsal column and pyramidal tract palsy

Answer 356

medial medullary syndrome

Question 357

unilateral CN V, VI, VII, VIII, IX palsy + cerebellar?

Answer 357

cerebellopontine angle syndrome

Question 358

unilateral CN IX, X, XI palsy +/- XII

Answer 358

jugular foramen

Question 359

bilateral UMN CN IX, X, XI, XII palsy

Answer 359

pseudobulbar palsy

Question 360

bilateral LMN CN IX, X, XI, XII palsy

Answer 360

bulbar palsy

Question 361

what is part of the CN clubs

Answer 361

orbital apex superior orbital fissure cavernous sinus lateral medullary syndrome cerebellopontine angle jugular foramen

Question 362

base of skull /meningeal disease causing CN palsies?

Answer 362

NPC meningitis - bacterial, TB carcinomatous meningitis- melanoma, lymphoma, adenoCa arnold chiari malformation infiltrative- sarcoidosis, amyloidosis

Question 363

Horner's + CN V, VII, IX, X, XI, XII palsy usually unilateral can progress to bilateral

Answer 363

syringobulbia

Question 364

signs of bulbar palsy?

Answer 364

slurred / nasal bulbar speech, difficulty with consonants atrophic tongue with fasciculations palatal weakness pooling/ dribbling of saliva weakness/ wasting of SCM jaw jerk normal or absent weakness of soft palate gag reflex absent

Question 365

what signs to look for to suggest aetiology of bulbar palsy?

Answer 365

surgical and radiation scars (NPC) UL and LL examination -look for signs of LMN lesion and fasiculations

Question 366

what is bulbar palsy?

Answer 366

bilateral LMN CN IX, X, XI, XII palsy -lesion at medulla or lower

Question 367

what is pseudobulbar palsy?

Answer 367

UMN bilateral CN IX, X, XI, XII palsy - usually in the internal capsule

Question 368

signs of pseudobulbar palsy?

Answer 368

pseudobulbar "hot potato" speech small spastic tongue palatal weakness pooling of saliva brisk jaw jerk gag reflex may be normal/ brisk look for: surgical and radiation scars (NPC) UL and LL examination- look for signs of UMN lesion AF, xanthelasma, DM dermopathy

Question 369

causes of bulbar palsy?

Answer 369

MND syringobulbia/myelia brainstem tumour e.g. NPC medullary infarct poliomyelitis GBS MG

Question 370

causes of pseudobulbar palsy?

Answer 370

MND/ALS high brain stem lesion/ tumour e.g. NPC MS bilateral hemispheric infarct/ trauma

Question 371

causes of jugular foramen syndrome?

Answer 371

ca of pharynx (most common) basal meningitis pagets disease thrombosis of jugular vein

Question 372

examination of a homonymous hemianopia?

Answer 372

lesion affecting optic radiation or visual cortex of occipital lobe can start with visual fields to confirm CN II- XII - involvement of other CN palsies may be present in posterior circulation stroke causing brainstem pathology along w occipital lobe screen for other long tract signs: sensory or tactile neglect pronator drift UL weakness babinkski for upgoing plantars screen for cerebellar signs: dysmetria screen for cause of stroke: cardiovasc: pulse, cardiac murmur, carotid bruit