Neurology Flashcards

1
Q

Which type of motor neuron disease carries the worst prognosis?

A

Progressive bulbar palsy

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2
Q

where is the median longitudinal fasciculus?

A

paramedian area of the midbrain and pons

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3
Q

MOA of baclofen?

A
  • GABA receptor agonist

- used to treat muscle spasticity in conditions such as multiple sclerosis, cerebral palsy and spinal cord injuries.

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4
Q

features of Von Hippel Lindau?

A

auto dom condition -> neoplasia

  • cerebellar haemangiomas -> subarachnoid haemorrhage
  • retinal haemangiomas
  • renal cysts
  • phaeochromocytoma
  • clear cell renal cell carcinoma
  • extra renal cysts (hepatic, pancreatic, epididymal)
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5
Q

hearing loss: vestibular neuronitis/ viral labyrinthitis?

A

vestibular neuronitis: NO hearing loss or tinnitus

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6
Q

features of syringomyelia?

A

‘cape-like’ (neck and arms) loss of sensation to temperature, with preservation of light touch, proprioception and vibration

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7
Q

Ix of syringomyelia?

A

full spine MRI with contrast to exclude a tumour or tethered cord.
+ brain MRI to exclude a Chiari malformation.

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8
Q

DVLA time off driving after first unprovoked seizure?

A
  • 6 months if brain imaging and EEG normal

- 12 months if any abnormality in ix

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9
Q

DVLA time off driving after stroke/ TIA?

A

1 month off driving, may not need to inform DVLA if no residual neurological deficit

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10
Q

DVLA time off driving after multiple TIAs?

A

3 months off driving and inform DVLA

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11
Q

management of respiratory distress in MND?

A

NIV e.g. BIPAP

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12
Q

current evidence based management of Bell’s palsy?

A

oral prednisolone

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13
Q

which management options in MND prolongs survival?

A
  • NIV: 7 months

- Riluzole: 3 months

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14
Q

poor prognostic features of Guillain-Barre?

A
  • age > 40 years
  • poor upper extremity muscle strength
  • previous history of a diarrhoeal illness (specifically Campylobacter jejuni)
  • high anti-GM1 antibody titre
  • need for ventilatory support
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15
Q

topiramate and glaucoma?

A

topiramate can precipitate acute closed angle glaucoma

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16
Q

Internuclear ophthalmoplegia: ie. Left eye unable to ADDUCT, where is the lesion?

A

LEFT medial longitudinal fasciculus

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17
Q

first line of medical management for drooling of saliva in people with Parkinson’s disease

A

glycopyrronium bromide

*If not effective/ tolerated or contraindicated, consider botulinum toxin A.

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18
Q

management of myoclonic seizures?

A

sodium valproate

2nd line: clonazepam, lamotrigine

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19
Q

what anti epileptic might exacerbate myoclonic seizures?

A

carbamazepine

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20
Q

Management of focal seizures?

A

carbamazepine or lamotrigine

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21
Q

what antiepilpetic might exacerbate absence seizures?

A

carbamazepine

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22
Q

management of absence seizures?

A

sodium valproate or ethosuximide

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23
Q

management of generalised tonic clonic seizures

A

sodium valproate

2nd line: lamotrigine, carbamazepine

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24
Q

features of anterior spinal artery occlusion?

A
  1. Bilateral spastic paresis

2. Bilateral loss of pain and temperature sensation

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25
Q

what tracts are affected in anterior spinal artery occlusion?

A
  1. Lateral corticospinal tracts

2. Lateral spinothalamic tracts

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26
Q

what anti-emetics might cause QTc prolongation?

A

ondansetron

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27
Q

1st line mx of brain abscess?

A
  • IV 3rd-generation cephalosporin (Ceftriaxone) + metronidazole
  • dexamethasone to reduce pressure
  • surgery to drain abscess
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28
Q

renal angiomyolipoma assoc w?

A

tuberous sclerosis

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29
Q

features of Miller Fischer syndrome?

A
  • ophthalmoplegia, areflexia and ataxia.

The eye muscles are typically affected first, “descending paralysis”

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30
Q

What antibodies are assoc w Miller Fischer syndrome?

A

anti-GQ1b antibodies

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31
Q

1st line management of benign essential tremor?

A

propranolol

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32
Q

when can you consider stopping antiepileptic drugs in epilepsy ?

A

if seizure free for >2 years. stop drugs over 2-3 months

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33
Q

1st line ix in suspected stroke?

A

non contrast CT head

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34
Q

features of parietal lobe brain lesion?

A
  • sensory inattention
  • apraxias (following commands)
  • astereognosis (tactile agnosia)
  • inferior homonymous quadrantanopia
  • Gerstmann’s syndrome (lesion of dominant parietal): alexia, acalculia, finger agnosia and right-left disorientation
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35
Q

features of occipital lobe lesion?

A
  • homonymous hemianopia (with macula sparing)
  • cortical blindness
  • visual agnosia (not recognising objects)
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36
Q

features of temporal lobe lesion?

A
  • Wernicke’s aphasia
  • superior homonymous quadrantanopia
  • auditory agnosia
  • prosopagnosia (difficulty recognising faces)
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37
Q

features of frontal lobe lesion?

A
  • expressive (Broca’s) aphasia
  • disinhibition
  • perseveration
  • anosmia
  • inability to generate a list
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38
Q

features of cerebellar lesion?

A
  • midline lesions: gait and truncal ataxia

- hemisphere lesions: intention tremor, past pointing, dysdiadokinesis, nystagmus

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39
Q

what area of the brain is implicated in Wernickes/ Korsakoffs?

A

Medial thalamus and mammillary bodies of the hypothalamus

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40
Q

What area of the brain is implicated in Huntington’s chorea?

A

Striatum (caudate nucleus) of the basal ganglia

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41
Q

features of Kluver-Bucy syndrome

A

hypersexuality, hyperorality, hyperphagia, visual agnosia

  • amygdala affected
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42
Q

what drugs might cause idiopathic intracranial HTN?

A
  • COCP
  • steroids
  • tetracyclines
  • vitamin A/ retinoids
  • lithium
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43
Q

what medication options might be beneficial in idiopathic intracranial HTN?

A
  • diuretics e.g. acetazolamide, topiramate

- topiramate

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44
Q

what management for idiopathic intracranial HTN?

A
  • weight loss
  • repeated LPs
  • Diuretics e.g. acetazolamide, topiramate
  • surgery (ie. VP shunt, endovascular shunt in patients with transverse sinus stenoses)
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45
Q

what leukaemia is most associated with gingival hyperplasia?

A

AML

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46
Q

management of myasthenia crisis?

A

plasmapheresis + intravenous immunoglobulins

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47
Q

1st line management of myasthenia gravis?

A

pyridostigmine (acetylcholinesterase inhibitor)

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48
Q

management of myasthenia gravis?

A
  • pyridostigmine
  • immunosuppression: prednisolone initially (azathioprine, cyclosporine, mycophenolate mofetil may also be used)
  • thymectomy
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49
Q

Ix of myasthenia gravis?

A
  • single fibre electromyography: high sensitivity (92-100%)
  • CT thorax to exclude thymoma
  • CK normal
  • autoantibodies
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50
Q

bromocriptine, ropinirole, cabergoline, apomorphine

A

dopamine agonists

- for parkinsons

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51
Q

selegiline

A

MAO-B inhibitors

- for parkinsons

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52
Q

entacapone, tolcapone

A

COM-T inhibitor

- COMT is an enzyme involved in the breakdown of dopamine, and hence used as an adjunct to levodopa therapy

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53
Q

what class of drug is topiramate?

A

carbonic anhydrase inhibitor

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54
Q

most common autonomic symptoms seen in Guillain Barre?

A

tachycardia, urinary retention

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55
Q

LP in Guillain Barre - what findings?

A

rise in protein with a normal white blood cell count (albuminocytologic dissociation) - found in 66%

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56
Q

Nerve conduction studies in Guillain Barre - what findings?

A
  • decreased motor nerve conduction velocity (due to demyelination)
  • prolonged distal motor latency
  • increased F wave latency
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57
Q

which parkinsonian medication is associated with pulmonary/ cardiac/ retroperitoneal fibrosis?

A

ergot-derived dopamine receptor agonists (bromocriptine, cabergoline)

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58
Q

features of multiple system atrophy?

A
  • parkinsonism
  • autonomic disturbance
  • —– erectile dysfunction: often an early feature
  • —– postural hypotension
  • —– atonic bladder
  • cerebellar signs
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59
Q

Features of Neurofibromatosis type 1 (aka von Recklinghausen’s syndrome)?

A
  • Café-au-lait spots (>= 6, 15 mm in diameter)
  • Axillary/groin freckles
  • Peripheral neurofibromas
  • Iris hamatomas (Lisch nodules) in > 90%
  • Scoliosis
  • Pheochromocytomas
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60
Q

what chromosome is affected in NF1?

A

chromosome 17

  • gene mutation that affects neurofibromin
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61
Q

Features of neurofibromatosis type 2?

A
  • Bilateral vestibular schwannomas

- Multiple intracranial schwannomas, mengiomas and ependymomas

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62
Q

What chromosome is affected in NF2?

A

chromosome 22

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63
Q

management of migraines in pregnancy?

A
  • 1st line paracetamol
  • 2nd line: NSAIDs in the first and second trimester

AVOID aspirin and opioids e.g. codeine

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64
Q

what artery is affected in Lateral medullary syndrome?

A

posterior inferior cerebellar artery

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65
Q

features of Lateral medullary syndrome / Wallenburg syndrome?

A

Cerebellar features: ataxia, nystagmus

Brainstem features:

  • ipsilateral: dysphagia, facial numbness, cranial nerve palsy e.g. Horner’s
  • contralateral: limb sensory loss
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66
Q

In patients with Guillain-Barre syndrome, respiratory function should be monitored with:

A

FVC (Force Vital Capacity) to monitor respiratory function

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67
Q

right congruous homonymous hemianopia with macula sparing.

where is the lesion

A

LEFT occipital cortex (opposite side!)

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68
Q

contraindications for triptan use?

A

ischaemic heart disease or cerebrovascular disease

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69
Q

acute management of cluster headache?

A

s/c sumatriptan + 100% O2

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70
Q

Prophylaxis of cluster headache?

A

verapamil

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71
Q

Complications of aneurysmal SAH?

A
  • Re-bleeding: usually in first 12h
  • Vasospasm: typically 7-14 days after
  • Hyponatraemia (SIADH)
  • Seizures
  • Hydrocephalus
  • Death
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72
Q

1st line management of spasticity in multiple sclerosis?

A

gabapentin or baclofen.

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73
Q

management of neuroleptic malignant syndrome?

A
  • stop antipsychotic
  • IV Fluids
  • dantrolene- directly relaxes muscles by inhibiting calcium release from the sarcoplasmic reticulum
  • bromocriptine, dopamine agonist
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74
Q

MOA of ondansetron

A

5-HT3 (serotonin) antagonist

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75
Q

SEs of ondansetron

A

most common- constipation

- Prolonged QT

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76
Q

Neurological features of Friedreich’s ataxia

A

Gait ataxia + kyphoscoliosis are most common

  • absent ankle jerks/extensor plantars
  • cerebellar ataxia
  • optic atrophy
  • spinocerebellar tract degeneration
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77
Q

Associations of Friedrich’s ataxia?

A
  • HOCM (90%, most common cause of death)
  • High arched palate
  • diabetes mellitus
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78
Q

Features of Ataxic telangiectasia?

A
  • Telangiectasia
  • cerebellar ataxia
  • IgA deficiency -> recurrent infections
  • increased risk of leukaemia/ lymphomas
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79
Q

drug causes of gingival hyperplasia?

A

phenytoin,
ciclosporin,
calcium channel blockers (especially nifedipine)

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80
Q

Cutaneous features of tuberous sclerosis?

A
  • epigmented ‘ash-leaf’ spots which fluoresce under UV light
  • roughened patches of skin over lumbar spine (Shagreen patches)
  • adenoma sebaceum (angiofibromas): butterfly distribution over nose
  • fibromata beneath nails (subungual fibromata)
  • café-au-lait spots* may be seen
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81
Q

Management of Ramsay Hunt syndrome?

A

oral acyclovir and steroids

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82
Q

MOA of procyclidine?

A

antimuscarinic.

  • help tremor and rigidity
  • treats drug induced parkinonism
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83
Q

management of absence seizures in pregnancy?

A

lamotrigine

  • ethosuximide / sodium valproate would not be first line in pregnancy/ breast feeding
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84
Q

Features of juvenile myoclonic epilepsy?

A

triad of:

  • Myoclonic seizures
  • Generalised tonic-clonic seizures
  • Absence seizures
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85
Q

Management of juvenile myoclonic epilepsy?

A

sodium valproate

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86
Q

Lambert Eaton myasthenic disorder assoc w?

A

Small cell lung ca + less commonly- ovarian/ breast ca

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87
Q

anti-NMDA receptor encephalitis assoc w?

A

ovarian teratoma

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88
Q

Ix of anti-NMDA receptor encephalitis?

A
  • anti-NMDA receptor antibodies
  • US abdo for ovarian teratoma
  • MRI head / CSF may be normal
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89
Q

Treatment of NMDA encephalitis?

A
  • immunosuppression with IV steroids, Ig, rituximab, cyclophosphamide or plasma exchange, alone or in combination.
  • Resection of teratoma
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90
Q

drug causes of tinnitus?

A
  • Aspirin/NSAIDs
  • Aminoglycosides - gentamicin
  • Loop diuretics
  • Quinine
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91
Q

CT findings in HSV encephalitis?

A

medial temporal and inferior frontal changes (e.g. petechial haemorrhages)
- normal in one-third of patients

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92
Q

inheritance pattern of Facioscapulohumeral muscular dystrophy

A

auto dom

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93
Q

what is juvenile myoclonic epilepsy classically triggered by?

A
  • in the morning/following sleep deprivation

- photosensitivity

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94
Q

What is parinaud syndrome?

A
  • Upward gaze palsy, often manifesting as diplopia (can’t look up)
  • Pupillary light-near dissociation (Pseudo-Argyll Robertson pupils)
  • Convergence-retraction nystagmus
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95
Q

Where is the lesion in Parinaud syndrome (can’t look up)?

A

Dorsal midbrain

  • rostral interstitial nucleus of MLF controls vertical gaze
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96
Q

What infection can cause spastic paraparesis?

A

HIV -> transverse myelitis

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97
Q

features of conduction aphasia?

A

speech fluent but repetition is poor. aware of the mistakes they are making. comprehension is normal.

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98
Q

Where is the lesion in conduction aphasia?

A

arcuate fasiculus - the connection between Wernicke’s and Broca’s area

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99
Q

Features of global aphasia?

A

severe expressive and receptive aphasia, may still be able to communicate using gestures.

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100
Q

Features of wernicke’s aphasia?

A

receptive aphasia

  • Comprehension impaired.
  • Speech fluent but makes no sense (Word salad)
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101
Q

where is the lesion in wernicke’s aphasia?

A

superior temporal gyrus

  • usually supplied by inferior division of Left MCA
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102
Q

features of Broca’s aphasia?

A

Expressive aphasia.

  • Speech is non fluent, laboured, halting.
  • repetition impaired
  • comprehension normal
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103
Q

Where is the lesion in Broca’s aphasia?

A

Inferior frontal gyrus.

  • typically supplied by superior division of the left MCA.
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104
Q

speech fluent but repetition poor. Comprehension intact.

A

Conduction aphasia

- classically due to stroke affecting the arcuate fasciculus

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105
Q

What distinguishes between Neuromyelitis optica and Multiple sclerosis?

A

NMO-IgG seropositive

- Antibodies against aquaporin 4 antigen

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106
Q

absolute criteria for diagnosis of neuromyelitis optica?

A

optic neuritis, acute myelitis

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107
Q

What is Neuromyelitis optica?

A
  • aka Devic’s disease
  • autoimmune disorder attacking optic nerves and cervical cord
  • vomiting is common
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108
Q

Diagnosis of neuromyelitis optica?

A
  • requires bilateral optic neuritis, myelitis and 2/3 criteria:
    1. spinal cord lesion involving >/=3 Spinal levels
    2. Initially normal MRI brain
    3. Aquaporin 4 positive serum antibody
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109
Q

what is the autoantibody in neuromyelitis optica?

A

NMO-IgG - aquaporin 4 positive antibody

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110
Q

Causes of bilateral facial nerve palsy?

A
  • sarcoidosis
  • Guillain-Barre syndrome
  • Lyme disease
  • bilateral acoustic neuromas (NF2)
  • Bell’s palsy (can be b/l)
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111
Q

Causes of LMN unilateral facial n palsy?

A
  • bilateral causes
  • Bell’s palsy
  • Ramsay-Hunt syndrome
  • acoustic neuroma
  • parotid tumours
  • HIV
  • multiple sclerosis*
  • diabetes mellitus
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112
Q

Causes of UMN unilateral facial n palsy?

A

Stroke, MS

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113
Q

what medication does paroxysmal hemicranial and hemicrania continua respond well to?

A

indomethacin

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114
Q

SE of Lamotrigine?

A

Stevens-Johnson syndrome

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115
Q

what areas of the spinal cord are affected in subacute combined degeneration of the cord?

A

dorsal columns + lateral corticospinal tracts

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116
Q

features of subacute combined degeneration of the cord?

A
  • joint position and vibration sense lost first then distal paraesthesia
  • UMN signs typically develop in the legs, classically extensor plantars, brisk knee reflexes, absent ankle jerks
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117
Q

DVLA advice for Meniere’s disease?

A

patients should inform the DVLA.

cease driving until satisfactory control of symptoms is achieved

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118
Q

prevention of attacks in meniere’s diesese?

A

betahistine and vestibular rehabilitation exercises

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119
Q

1st line for newly diagnosed Parkinson’s who have motor symptoms affecting their quality of life?

A

Levodopa

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120
Q

management of Bells palsy if paralysis does not improve within 3 wks?

A

refer urgently to ENT

  • referral to plastic surgery may be appropriate for patients with more long-standing weakness e.g. several months
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121
Q

Infections assoc w autonomic neuropathy?

A

HIV, Chagas’ disease, neurosyphilis

122
Q

what conditions in which one should start anti-epileptics after first seizure?

A

if any of the following present:

  • neurological deficit
  • brain imaging shows a structural abnormality
  • EEG shows unequivocal epileptic activity
  • patient or family/ carers consider the risk of having a further seizure unacceptable
123
Q

features of restless legs syndrome?

A
  • akathisia: uncontrollable urge to move legs
  • paraesthesias e.g. ‘crawling’ or ‘throbbing’ sensations
  • periodic limb movements of sleeps (PLMS)
124
Q

Causes/ associations of restless legs sydnrome?

A
  • idiopathic (50% have +ve FHx)
  • iron deficiency anaemia
  • uraemia
  • diabetes mellitus
  • pregnancy
125
Q

Diagnosis of restless legs syndrome?

A

clinical, ferritin to rule out IDA

126
Q

Management of restless legs syndrome?

A
  • simple measures: walking, stretching, massaging affected limbs
  • treat any iron deficiency
  • 1st line: dopamine agonists (e.g. Pramipexole, ropinirole)
  • benzodiazepines
  • gabapentin
127
Q

1st line medical management of restless legs syndrome?

A

dopamine agonists e.g. pramipexole, ropinirole

128
Q

absence seizures: what is the probability of the patient becoming seizure-free during adolescence?

A

90-95%. good prognosis

129
Q

dementia pugilistica?

A
  • assoc w boxers

- chronic traumatic encephalopathy

130
Q

a scale that measures disability or dependence in activities of daily living in stroke patients

A

Barthel scale

131
Q

features of medication overuse headache?

A
  • present for 15 days or more per month
  • developed or worsened whilst taking regular symptomatic medication
  • highest risk: using opioids and triptans
  • may be psychiatric co-morbidity
132
Q

management of simple analgesics and triptans in medication overuse headache?

A

stop abruptly (may initially worsen headaches)

133
Q

management of opioids in medication overuse headache?

A

gradually withdrawn

134
Q

confusion, behavioural change, ataxia, hemiparesis and visual deficits. in pt on natalizumab/ other HIV or MS drugs

A

Progressive multifocal leukoencephalopathy

- due to reactivation of JC virus

135
Q

what is transverse myelitis?

A

inflammation of the spinal cord. can be uni/ bilateral. characterized by neurologic dysfunction in motor and sensory tracts.
-> causes UMN weakness in lower limbs

136
Q

auditory agnosia - which lobe

A

temporal

137
Q

Palatal myoclonus - where is the lesion

A

in the triangle of Guillain and Mollaret (triangle linking the inferior olivary nucleus, red nucleus and the contralateral dentate nucleus)
- specific feature of hypertrophic olivary degeneration

138
Q

causes of Predominately sensory Peripheral neuropathy

A
  • diabetes
  • uraemia
  • leprosy
  • alcoholism
  • vitamin B12 deficiency
  • amyloidosis
139
Q

causes of Predominately motor Peripheral neuropathy

A
  • Guillain-Barre syndrome
  • porphyria
  • lead poisoning
  • hereditary sensorimotor neuropathies (HSMN) - Charcot-Marie-Tooth
  • chronic inflammatory demyelinating polyneuropathy (CIDP)
  • diphtheria
140
Q

causes of Demyelinating Peripheral neuropathy

A
  • Guillain-Barre syndrome
  • chronic inflammatory demyelinating polyneuropathy (CIDP)
  • amiodarone
  • hereditary sensorimotor neuropathies (HSMN) type I
  • paraprotein neuropathy
141
Q

causes of Peripheral neuropathy of axonal pathology

A
alcohol
diabetes mellitus*
vasculitis
vitamin B12 deficiency*
hereditary sensorimotor neuropathies (HSMN) type II
142
Q

Gerstmann’s syndrome: alexia, acalculia, finger agnosia, R-L disorientation — where is the lesion?

A

Lesion of Dominant parietal

-> dominant = opposite of dominant hand

143
Q

SE of Vigabatrin (AED)?

A

V for Visual field defects.

40% of patients develop visual field defects, which may be irreversible

visual fields should be checked every 6 months

144
Q

genetics of Charcot-Marie-Tooth disease (hereditary sensorimotor neuropathy type I)

A

auto dom, defect in PMP-22 gene (which codes for myelin)

145
Q

features of Charcot-Marie-Tooth disease (hereditary sensorimotor neuropathy type I)?

A
  • features often start at puberty
  • motor symptoms predominate
  • distal muscle wasting, pes cavus, clawed toes
  • foot drop, leg weakness often first features
146
Q

what is myotonic dystrophy?

A

an inherited myopathy with features developing at 20-30 years old.

  • affects skeletal, cardiac and smooth muscle.
  • 2 main types: DM1 and DM2.
147
Q

Genetics of myotonic dystrophy?

A

auto dom, trinucleotide repeat disorder

148
Q

genetics of DM1 (Myotonic dystrophy type 1)

A

Chr 19, CTG repeat at the end of DMPK (Dystrophia Myotonica-Protein Kinase) gene

149
Q

Genetics of DM2 (myotonic dystrophy type 2)?

A

Chr 3, repeat expansion of the ZNF9 gene

150
Q

Difference between DM1 and DM2?

A

DM1: distal weakness more prominent
DM2: proximal weakness more prominent, severe congenital form not seen

151
Q

General features of myotonic dystrophy?

A
  • myotonic facies (long, ‘haggard’ appearance)
  • frontal balding
  • bilateral ptosis
  • cataracts
  • dysarthria
  • myotonia (tonic spasm of muscle)
152
Q

urinary incontinence + gait abnormality + dementia

A

normal pressure hydrocephalus

153
Q

triad of features in normal pressure hydrocephalus?

A
  • urinary incontinence
  • dementia, bradyphenia (slowed thinking)
  • gait abnormality (may be similar to Parkinson’s disease)
154
Q

Ix of Normal pressure hydrocephalus?

A

Imaging showing hydrocephalus with ventriculomegaly in the absence of, or out of proportion to, sulcal enlargement

155
Q

Mx of normal pressure hydrocephalus?

A

VP shunting.

156
Q

what antibiotics may exacerbate myasthenia gravis?

A

gentamicin

  • macrolides (clari/erythromycin), quinolone (ciprofloxacin), tetracyclines (doxy)
157
Q

what are some drugs that may exacerbate myasthenia?

A

penicillamine, procainamide, BB, lithium, phenytoin, quinidine, antibiotics e.g. gentamicin

158
Q

what class of drug are triptans?

A

5-HT1 agonists

159
Q

CI for triptans?

A

history of, or RF for, ischaemic heart disease/ Cerebrovascular disease

160
Q

features of CJD?

A
  • dementia (rapid onset)

- myoclonus (e.g. “notable jerk” usually provoked by startle)

161
Q

Ix of CJD?

A

CSF usually normal

  • EEG: Biphasic, high amplitude sharp waves (in sporadic CJD)
  • MRI: hyper intense signals in the basal ganglia and thalamus
162
Q

most common presenting features of new variant CJD?

A

usually younger patients (average age. = 25y)

- anxiety, withdrawal, dysphonia

163
Q

causes of miosis?

A
  • Horner’s syndrome
  • Argyll-Robertson pupil
  • senile miosis
  • pontine haemorrhage
  • congenital
164
Q

what is pituitary apoplexy?

A

Sudden enlargement of a pituitary tumour (usually non-functioning macroadenoma) secondary to haemorrhage or infarction.

165
Q

Features of pituitary apoplexy?

A
  • sudden onset headache similar to that seen in subarachnoid haemorrhage
  • vomiting
  • neck stiffness
  • visual field defects: classically bitemporal superior quadrantic defect
  • extraocular nerve palsies
  • features of pituitary insufficiency
    e. g. hypotension/hyponatraemia secondary to hypoadrenalism
166
Q

Diagnostic ix of pituitary apoplexy?

A

MRI

167
Q

Mx of pituitary apoplexy?

A
  • urgent steroid replacement due to loss of ACTH
  • careful fluid balance
  • Surgery
168
Q

congruous vs incongruous visual field defect?

A

A congruous defect simply means complete or symmetrical visual field loss and conversely an incongruous defect is incomplete or asymmetric.

incongruous defects = optic tract lesion; congruous defects= optic radiation lesion or occipital cortex

169
Q

what hormone levels are low in narcolepsy?

A

orexin (hypocretin)- a protein responsible for controlling appetite and sleep patterns

170
Q

Ix of narcolepsy?

A

multiple sleep latency EEG

171
Q

Mx of narcolepsy?

A

Daytime stimulants e.g. modafinil, and nighttime sodium oxybate

172
Q

restless legs syndrome- single most important blood test?

A

ferritin - exclude IDA

173
Q

haemorrhage disease of the newborn - which AED may cause this?

A

phenytoin

174
Q

DMARDS in Multiple sclerosis?

A
  1. Beta interferon - reduces relapse rates
  2. glatiramer acetate - immunomodulating drug
  3. natalizumab - antagonises Alpha-4 beta-1-integrin, inhibits migration of leucocytes across the BBB
  4. fingolimod: sphingosine 1-phosphate receptor modulator
175
Q

Froins syndrome?

A

xanthochromia, high protein level and marked coagulation of CSF

-> caused by meningeal irritation and CSF flow blockage by tumour mass/ abscess

176
Q

features of multiple system atrophy?

A
  • parkinsonism
  • autonomic disturbance: erectile dysfunction: often an early feature, postural hypotension, atonic bladder
  • cerebellar signs
177
Q

What is CADASIL?

A

cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy

  • rare cause of multi-infarct dementia
  • often present with migraine
178
Q

genetics of CADASIL?

A

NOCTH3 gene

179
Q

features of CADASIL?

A

usually presents with migraines in middle age, followed by recurrent TIAs/ strokes -> neuro-cognitive decline, psych problems and dementia.

MRI- multiple widespread hyper intense lesions in the white matter, basal ganglia, thalamus and pons

180
Q

what antibody is implicated in peripheral neuropathy in breast cancer?

A

Purkinje cell antibody

181
Q

what antibody is implicated in ocular opsoclonus-myoclonus (assoc w breast/ small cell lung ca)?

A

Anti-Ri antibody

182
Q

what antibody is implicated in stiff person’s syndrome/ diffuse hypertonia (assoc w breast, colorectal, small cell lung ca)?

A

Anti-GAD antibody

183
Q

what antibody is implicated in cerebellar syndrome assoc w ovarian/ breast cancer?

A

anti-Yo antibody

184
Q

what antibody is assoc with small cell lung ca and neuroblastomas and may cause sensory neuropathy (may be painful), cerebellar syndrome and encephalomyelitis?

A

anti-Hu antibody

185
Q

Ix of wernicke’s encephalopathy?

A
  • decreased red cell transketolase

- MRi brain

186
Q

What is the most specific finding on brain MRI for this Wernicke’s encephalopathy?

A

enhancement of the mamillary bodies due to petechial haemorrhages

187
Q

what is the cardiac features associated with myotonic dystrophy?

A

heart block, cardiomyopathy

188
Q

What AED is assoc with the rare SE of Steven Johnson syndrome?

A

lamotrigine

189
Q

common peroneal nerve palsy?

A

foot drop, weakness of foot dorsiflexion, foot eversion, extensor hallucis longus

  • sensory loss over the dorsum of the foot and lower lateral part of the leg
  • wasting of the anterior tibial and peroneal muscles
190
Q

features of acute disseminated encephalomyelitis?

A
  • autoimmune demyelinating disease
  • can occur following infection
  • acute onset of multifocal neurological symptoms
191
Q

mx of acute disseminated encephalomyelitis?

A

IV steroids, consideration of IVIG where this fails

192
Q

1st line mx of restless legs syndrome?

A

dopamine agonists e.g. ropinirole, pramipexole

193
Q

what kind of anaemia is phenytoin assoc w?

A

folate deficiency -> megaloblastic anaemia.

194
Q

SEs of levodopa?

A
- dyskinesia
'- on-off' effect
- postural hypotension
- cardiac arrhythmias
- nausea & vomiting
- psychosis
- reddish discolouration of urine upon standing
195
Q

1st line management of parkinsons disease if the motor symptoms are not affecting the patient’s quality of life?

A

dopamine agonist (non-ergot derived) e.g. ropinirole, levodopa or monoamine oxidase B (MAO‑B) inhibitor e.g. selegiline

196
Q

management of drug induced Parkinsonism?

A

antimuscarinics e.g. procyclidine, benzotropine, benzhexol (trihexyphenidyl)

197
Q

what part of the hypothalamus is affected with a craniopharyngioma?

A

ventromedial area of the hypothalamus

198
Q

management of benign essential tremor when propranolol is CI?

A

primidone

199
Q

Lead poisoning vs acute intermittent porphyria?

A

both cause increase in urinary delta-aminolevulinic acid, only lead poisoning causes basophilic stippling

200
Q

Features of nitrous oxide toxicity?

A
  • predominantly sensory neuropathy and assoc myelopathy (leg weakness, increased AND decreased reflexes)
  • similar to B12 deficiency as NO interacts w Vitamin B12 causing selective inhibition of methionine synthase
201
Q

Features of orbital apex syndrome?

A

optic neuropathy, proptosis, Horner’s syndrome, cheimoses (swelling of the ocular surface membranes), ophthalmoplegia, involvement of first branch of the trigeminal nerve.

202
Q

Classic features of temporal lobe epilepsy?

A

ascending epigastric aura
olfactory + gustatory hallucinations,
ictal fear,
automatism: lip-smacking, chewing, swallowing + fiddling/ tapping

203
Q

Most common cause of temporal lobe epilepsy?

A

hippocampal sclerosis

dx: MRI brain.
Tx: surgery

204
Q

mx of primary cervical dystonia/ torticollis?

A

Botulinum toxin injection

205
Q

What can you measure to discriminate between organic and psychogenic seizures?

A

serum prolactin levels.

most seizures cause rise in serum prolactin whereas psychogenic seizures do not.

206
Q

what type of nystagmus is associated with difficulty in reading down a menu/ text? and is usually better when reading a menu at coffee shops (usually positioned above your head)

A

downbeat nystagmus

207
Q

Diagnosis of TB meningitis?

A

CSF PCR

208
Q

differences in reflexes in conus medullaris vs cauda equina syndrome?

A

Cauda equina: Both ankle/ knee jerks affected

Conus medullaris: Knee jerks preserved, ankle jerks affected

209
Q

Mx of Tourette syndrome?

A

Risperidone

210
Q

what are the 5 classic presentations of lacunar strokes?

A
  1. pure motor hemiparesis
  2. pure sensory
  3. sensorimotor
  4. ataxic hemiparesis
  5. clumsy-hand dysarthria
211
Q

What nerve conduction study finding is assoc w Axonal neuropathy??

A

reduced compound muscle action potential amplitude

212
Q

What nerve conduction study finding is assoc with myelin disorders?

A

reduction in conduction velocity/ conduction block

213
Q

features of inclusion body myositis?

A
  • most common acquired myopathy in >50y, esp M.
  • serum CK can be normal
  • idiopathic inflammatory myopathy
  • affects quads/ long finger flexors. often asymmetrical
214
Q

cavernous sinus thrombosis affects which nerves?

A

CN 3, 4, 6, V1

215
Q

Ipsilateral CNIII palsy + contralateral hemiparesis and hemiparkinsonism

A

Weber syndrome

- occlusion of paramedian branches of the posterior cerebral artery

216
Q

mx of miller-fisher syndrome?

A

IVIG +/- plasma exchange

217
Q

SEs of topiramate?

A

weight loss + renal stones + cognitive / behavioural changes

218
Q

Compression of anterior interosseous nerve / aka Kiloh-Nevin syndrome
- features?

A

pain in form + weakness of pincer movement of thumb and index finger

219
Q

Parietal lesions in the dominant lobe can produce:

A
  • disorders of language
  • agnosia
  • Gerstmann Syndrome (L-R confusion, acalculia, agraphia, finger agnosia)
220
Q

Non dominant parietal lesions may cause:

A
  • visuospatial dysfunction
  • constructional apraxia (ie. clock face)
  • anosognosia (lack of insight) and dressing apraxia
221
Q

Triad of kearn sayers syndrome?

A

chronic progressive external ophthalmoplegia, pigmentary retinopathy and cardiac conduction defects

222
Q

Diagnosis of kearn sayers?

A

muscle biopsy - characteristic ‘ragged red fibres’ (due to increased number of mitochondria)

  • often supplemented by PCR analysis of mitochondrial DNA for mutation detection
223
Q

Presumed BZD overdose: management?

A

Supportive care

It is now advised that flumazenil should only be used to reverse benzodiazepines in anaesthesia. (Due to side effect profile)

224
Q

Features of alcohol hallucinosis?

A
  • psychosis of < 6 mo duration
  • auditory hallucinations, often of persecutory or derogatory nature
  • occurs in clear consciousness
225
Q

Which antiepileptic exhibits autoinduction ie. return of seizures after 3-4 weeks of treatment?

A

Carbamazepine

226
Q

Anterior uveitis: management?

A

topical corticosteroids and cycloplegic (mydriatic) drops E.g. atropine/ cyclopentolate

227
Q

When should statins be started post acute stroke?

A

48 hours post-event

  • potential for haemorrhagic transformation in early administration of statins.
228
Q
A

Dawson fingers: often seen on FLAIR images - hyperintense lesions penpendicular to the corpus callosum

-> seen in multiple sclerosis

229
Q

Drugs causing a peripheral neuropathy?

A
amiodarone
isoniazid
vincristine
nitrofurantoin
metronidazole
230
Q

Acute dystonia: management?

A

Procyclidine (anticholinergic)

Usually occurs as an extra pyramidal side effect to dopamine antagonist

231
Q

Gold standard imaging option to pick up brain tumour?

A

MRI brain with contrast

- offers excellent resolution and can be used to distinguish between other pathologies (such as an abscess)

232
Q

What kind of brain tumour may be associated with polycythaemia?

A

haemangioblastomas release erythropoietin so can cause a rise in haemoglobin

233
Q

thunderclap headache with normal CT brain and normal or near-normal lumbar puncture results ?

A

Reversible cerebrovascular vasoconstriction syndrome (RCVS)

234
Q

Reversible cerebrovascular vasoconstriction syndrome (RCVS) - diagnosis?

A

finding of diffuse arterial beading on angiography (CTA, MRA or catheter)

235
Q

Reversible cerebrovascular vasoconstriction syndrome (RCVS): management?

A

no evidence-based treatments for RCVS

although bed-rest (avoid exercise) and nimodipine/verapamil are usually prescribed. manage hypertension

236
Q

First line diagnostic test for delirium?

A

Confusion Assessment Method

237
Q

MRI findings in CJD?

A

hyperintense signals in the basal ganglia and thalamus

238
Q

Most sensitive investigation to diagnose myasthenia gravis?

A

Single fibre electromyography (92-100% sensitivity)

Serum ACh receptor antibodies (85% sensitivity)

239
Q

Optic neuritis treatment?

A

IV methylprednisolone

  • Optic neuritis treatment trial: increased speed of visual recovery and reduced risk of conversion to multiple sclerosis at 2 years compared to oral prednisolone
240
Q

Parkinson’s dementia: management?

A

Rivastigmine

- licensed treatment demonstrated in a randomised clinical trial

241
Q

Contraindications to thrombolysis in stroke?

A
  • stroke or traumatic brain injury in last 3 months
  • previous intracranial haemorrhage
  • seizure at onset of stroke
  • intracranial neoplasm
  • suspected subarachnoid haemorrhage
  • LP in last 7 days
  • GI Bleed in last 3 wks
  • active bleeding
  • pregnancy
  • oesophageal actives
  • uncontrolled hypertension >200/120
242
Q

Hoarseness post thyroidectomy: how to investigate?

A

Hoarseness indicates recurrent laryngeal nerve damage.

1st line: laryngoscopy to assess for vocal cord paralysis and evaluate motility

243
Q

acute disseminated encephalomyelitis?

A

post infectious encephalomyelitis

  • autoimmune demyelinating disease of the CNS
    e. g. post measles, mumps, rubella, varicella

tx: steroids +/- consideration of IVIg

244
Q

what blood test might be raised in true seizure compared to pseudo seizure?

A

raised serum prolactin

245
Q

features of Multifocal motor neuropathy?

A

acquired autoimmune demyelinating Moto neuropathy

  • motor conduction block
  • slowly progressive, distal motor neuropathy which progresses over many years
  • usually normal sensation and reflexes
  • asymmetric
246
Q

multifocal motor neuropathy: what antibodies are usually raised?

A

Anti-GM1 antibodies

247
Q

what is POEMS syndrome?

A
polyneuropathy
organomegaly
endocrinopathy
M-protein band from a plamacytoma
Skin pigmentation
248
Q

most effective imaging modality for TIA?

A

diffusion-weighted MRI head which is very sensitive to small ischaemic lesions.

249
Q

features of spinocerebellar ataxias?

A
  • autosomal dominant
  • progressive development of ataxia features e.g. gait disturbance, nystagmus, tremor
  • usually begins between 30-40 yo
250
Q

up to what percentage of individuals with Guillain Barre will experience long term weakness?

A

15%

251
Q

what anti-epileptic drug may cause hyperammonaemic encephalopathy?

A

sodium valproate

252
Q

management of valproate-induced hyperammonaemic encephalopathy?

A

L-carnitine

- may reduce ammonia levels

253
Q

HSV encephalitis LP findings?

A

Raised lymphocytes

High protein

254
Q

Prognosis of HSV encephalitis?

A

dependent on whether aciclovir is commenced early

> If started promptly mortality is 10-20%. > Left untreated mortality approaches 80

255
Q

EEG findings of HSV encephalitis?

A

lateralised periodic discharges at 2 Hz

256
Q

Myasthenia gravis:

Situations in which thymectomy is generally not done?

A
when patients have 
1. antibodies to MUSK
2. late onset disease 
Or
3. purely ocular disease
257
Q

Multiple sclerosis relapse rates in pregnancy?

A

relapse rates may reduce during pregnancy and may increase 3 to 6 months after childbirth before returning to pre-pregnancy rates

258
Q

Chronic inflammatory demyelinating polyneuropathy: management?

A

Steroids and immunosuppressants

259
Q

Management of off episodes in Parkinsons?

A

Can use subcut apomorphine to alleviate symptoms

260
Q

ipsilateral optic atrophy and contralateral papilloedema

A

Foster- Kennedy syndrome

typically caused by a frontal lobe mass, normally a meningioma, which compresses on the ipsilateral optic nerve and olfactory nerve, while increasing intracranial pressure for the contralateral optic nerve sheath

261
Q

Features of foster Kennedy syndrome?

A

optic atrophy + central scotoma in the ipsilateral eye
papilloedema in the contralateral eye
anosmia

262
Q

neurological syndrome in which trivial trauma to a peripheral nerve e.g. sleeping on a limb, results in a mononeuropathy which may take weeks to resolve

A

Hereditary Neuropathy with Liability to Pressure Palsy (HNPP)

263
Q

Ix of Hereditary Neuropathy with Liability to Pressure Palsy

A

deletion in the peripheral myelin protein 22 gene on chromosome 17
> autosomal dominant

264
Q

treatment of hepatotoxicity or hyperammonemic encephalopathy in the context of an acute or chronic overdose of sodium valproate?

A

L-carnitine

> reduces serum ammonia levels

265
Q

irregular low frequency tremor which is a combination of resting, postural and action tremor

+ hx of prev stroke

A

Holmes tremor

caused by a lesion in the red nucleus
Tx: levodopa / thalamotomy or chronic thalamic stimulation

266
Q

Asymmetric bi-brachial motor weakness without sensory or bulbar involvement

+ multi focal areas of demyelination and motor block

A

Multi focal motor neuropathy with conduction block

MMN-CB

267
Q

Tx of multi focal motor neuropathy with conduction block?

Acquired immune mediated demyelinating neuropathy

A

IV Ig

268
Q

What electrolyte replacement can precipitate myasthenia crisis?

A

IV Mg

269
Q

Multiple lung cysts in tuberous sclerosis?

A

Lymphangioleiomyomatosis

270
Q

Difference in size criteria of cafe au lait spots to diagnose neurofibromatosis in children vs adults?

A

Children: >0.5cm
Adults: >1.5cm

271
Q

Diagnosis of spontaneous intracranial hypotension?

A

MRI with gadolinium

> demonstrates distinctive dural gadolinium enhancement and downward displacement of brain on sagittal views.

272
Q

Risk factors for spontaneous intracranial hypotension?

A

connective tissue disorders such as Marfan’s syndrome

273
Q

Key features of spontaneous intracranial hypotension?

A

strong postural relationship with the headache generally much worse when upright. Patients may, therefore, be bed-bound

274
Q

Management of spontaneous intracranial hypotension?

A

usually conservative

> if this fails an epidural blood patch may be tried

275
Q

What MRI brain finding is highly specific for spontaneous intracranial hypotension?

A

MRI brain with contrast:
diffuse, smooth dural thickening with enhancement (in~80%).

others less specific but common:
cerebellar tonsillar descent, subdural fluid collections, decreased ventricular size, engorgement of the pituitary gland

276
Q

What imaging should be considered in trigeminal neuralgia?

A

first line- contrast MRI brain: may detect compressing/ demyelinating cause in ~15%

MRA: in the other ~85%, MRA may identify neurovascular contact between a loop of the superior cerebellar artery and the trigeminal nerve -> causing displacement or atrophy

277
Q

what other 2nd line medications to consider for treatment of trigeminal neuralgia after carbamazepine?

A

oxcarbazepine, baclofen, gabapentin, lamotrigine

for those refractory to two-drug trials: consider surgical intervention

278
Q

prophylactic mx of cluster headaches?

A

verapamil, galcanezumab can reduce freq of cluster headaches

279
Q

mx of SUNCT: short lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing?

A

medically refractory, reports of response to lamotrigine

280
Q

prophylaxis mx of tension type headaches?

A

amitriptyline

281
Q

Most sensitive and specific test for prion proteins in the CSF to diagnose CJD?

A

real-time quaking-induced conversion assay
-uses fluorescent dye to detect prion protein present in the CSF

282
Q

severely asymmetric Parkinsonism, fixed dystonia, myoclonus, cortical sensory deficits, apraxia, cognitive deficits …?

A

corticobasal degeneration

  • one of the Parkinson’s plus syndromes
283
Q

severely asymmetric Parkinsonism, fixed dystonia, myoclonus, cortical sensory deficits, apraxia, cognitive deficits …?

A

corticobasal degeneration

  • one of the Parkinson’s plus syndromes
284
Q

treatment for levodopa-induced dyskinesia?

A

amantadine

285
Q

what autoantibody is positive in NMO?

A

serum aquaporin 4 antibody testing

286
Q

management of intracranial hypotension?

A

epidural blood patch

287
Q

Treatment options of tardive dyskinesia 2’ anti-emetic, antipsychotic meds (dopamine antagonists)?

A

monoamine transporter 2 inhibitors e.g. valbenazine, deutetrabenazine, tetrabenazine

288
Q

1st line treatment for MS relapses?

A

high dose glucocorticoids e.g. IV Methylpred (1g/d for 3-5 days)

289
Q

2nd line treatment for MS relapses refractory to glucocorticoid treatment?

A

IM adenocorticotropin hormone gel, plasmapheresis

290
Q

in Multiple sclerosis, what is defined as “activity”?

A

clinical relapses or MRI evidence of new or enlarging lesions

291
Q

in Multiple sclerosis, what is defined as “activity”?

A

clinical relapses or MRI evidence of new or enlarging lesions

292
Q

in Multiple sclerosis, what is defined as “progression”?

A

gradual accumulation of neurologic deficits independent of relapses

293
Q

in Multiple sclerosis, what is defined as “progression”?

A

gradual accumulation of neurologic deficits independent of relapses

294
Q

what CSF finding is characteristic of Guillain Barre syndrome?

A

albuminocytologic dissociation (high protein level with normal/mildly elevated leucocyte count)

295
Q

what are good first line AEDs to initiate in older adults?

A

lamotrigine, gabapentin, levetiracetam

296
Q

first line treatment for essential tremors?

A

propranolol, primidone, topiramate

297
Q

2nd line treatment for essential tremors?
ie. after trying propranolol, primidone, topiramate

A

clonazepam

298
Q

treatment of refractory essential tremors?

A

deep brain stimulation and focused US thalamotomy

299
Q

what prophylactic agents may be used in migraines?

A
  • propranolol, timolol, metoprolol
  • amitriptyline, venlafaxine
  • topiramate
  • sodium valproate
  • monoclonal abs
300
Q

what investigation is performed in drug resistant epilepsy to evaluate for candidacy for epilepsy surgery?

A

1st step: video EEG
2nd step: advanced brain imaging

301
Q

what monoclonal antibodies may be used for migraine prophylaxis?

A

erenumab, fremanezumab, eptinezumab, galcanezumab

302
Q

features of spinocerebellar ataxias?

A
  • autosomal dominant
  • progressive development of ataxia features e.g. gait disturbance, nystagmus, tremor
  • usually begins between 30-40 yo