Neuro Flashcards

1
Q

WHat causes lateral medullary syndrome? What is this supplied by

A

Infarction of the lateral portion of the medulla oblingata
Posterior inferior cerebellar artery or vertebral artery

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2
Q

Features of lateral medullar syndrome

A

Loss of pain and temp sensation on the contralateral side of the body and on the ipsilateral side of the face (dysphagia, vertigo, dysarthria, horners syndrome and nystagmus)

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3
Q

Definition of parkinsonism

A

Collective term for a clinical syndrome that includes bradykineasia alongisde one other of
- tremor
- rigidity
- gait disturbance

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4
Q

Causes of parkinsonism

A

PD
Drug induced
Cerebrovascular disease
Dementia with lewy bodies
MSA
Progressive supranuclear palsy

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5
Q

Pathology of PD

A

Chronic progressive neurodegenerative disease linked to decreased dopamine production in the substrantia nigra

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6
Q

What gait is seen in PD

A

Shuffling

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7
Q

Diagnosis of PD

A

Mainly clinical
MRI
SPECT

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8
Q

What are parkinsons plus syndromes

A

A group of neurodegenerative diseases that consit of cardinal features of PD, alongside additional symptoms which include a reduced response to levodopa, dysarthria, autonomic dysfunction and supranuclear gaze palsy

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9
Q

Examples of the parkinsons plus syndromes

A

Multiple system dystrophy
Dementia with lewy odies
Progressive supranuclear palsy
Corticobasal degeneration

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10
Q

MEdications that are known to cause parkinsonism

A

Antipsychotics
metoclopramide
prochloperazine
tetrabenazine
sodium valproate
lithium

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11
Q

Signs and symptoms more consistent to the diagnosis of PD

A

Unilateral onset and patient asymmetry
Resting tremor
good response to levodopa
Levodopa induced dyskinea
Long disease course >10 yrs

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12
Q

Treatment of PD

A
  1. Levodopa or co-carledopa
  2. pramipexole or ropinorole
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13
Q

What drug can you use to improve dyskinesia affects of anti-parkinson medications?

A

Amantadine

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14
Q

What is the triad of NPH

A

Gait disturbance
Memory loss
Urinary Incontinence

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15
Q

Symptoms of L5/S1 disc prolapse

A

Low back pain
Sciatica
Limited straight leg raisiing
Loss of ankle reflex
Weakness of plantar flexion
Sensory loss in affected dermatome

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16
Q

A young otherwise healthy person who suddenly develops a stroke is likely to be caused by what?

A

Paradoxical embolus
Due to a patent foramen ovale

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17
Q

Imaging of choice if you were suspecting a paradoxical emboli secondary to patent FO

A

transoesophageal ECHO with doppler colour imaging

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18
Q

What is a common S/E of levodopa Tx

A

Dark urine

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19
Q
A
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20
Q

What is found in the CSF of patients with MS

A

Unpaired oligoclonal bands

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21
Q

S/E of topiramate

A

Weight loss
Renal stones
Congitive/behavioural changes

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22
Q

What is neuroepileptic malignant syndrome

A

Complication of treatment with antipsychotic drugs or withdrawl of dopamine agonists

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23
Q

Presentation of neuroepileptic malignant syndrome

A

Muscle rigidity
Autonomic instability
Hyperthermia
Altered mental state

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24
Q

Treatment of neuroepileptic malignant syndrome

A

Supportive measures
Ceasing of the responsible drug

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25
Q

Risk factors for neuroepileptic malignant syndrome

A

Dopamine disorders e.g. PD
Structural brain abnormalities
Genetics

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26
Q

Pathology of GBS

A

Segmental demyelination

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27
Q

Where do anti-Yo antibodies primarily occur?

A

In patients with paraneoplastic cerebellar degeneration (PCD) who have breast cancer or tumours of the ovary, endometrium and fallopian tube

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28
Q

Pathology of paraneoplasic cerebellar degeneration

A

Tumour cells express proteins normally expressed in the cerebellum, triggering an autoimmune reaction

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29
Q

S/Es phenytoin

A

Fatigue
Bone pain
Tingling and numbness in lower limbs
Gum hypertrophy

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30
Q

Antibodies of lambert eaten myasthenic syndrome (LEMS)

A

Anti voltage gated calcium channel Ab

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31
Q

Antibodies of myasthenia gravis

A

Acetylcholine receptor ab

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32
Q

When does labert eaten syndrome most often occur

A

As a paraneoplastic phenomenon - particular assosiation with small cell lung cancer

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33
Q

Reflexes - LEMS vs MG

A

LEMS - absence of reflexes
MG - reflexes unaffected

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34
Q

Autonomic features - LEMS vs MG

A

LEMS - autonomic features present
MG - uncommon

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35
Q

Fatgiue - LEMS vs MG

A

In contrast to MG, LEMS - temporary increase in muscle power post exercise

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36
Q

Treatment of LEMS

A

2,4-diaminopyradie (DAP)
IV immunoglobulins
and/or plasma exchange
Treatment of underlying cancer

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37
Q

Features of idiopathic intracranial HTN

A

High pressure headache
Worse on lying flat
Worse in the morning
Assosiated with papilloedema

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38
Q

What should be excluded if you are suspecting idiopathic intracranial HTN

A

Cerebral venous sinus thrombosis

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39
Q

Diagnosis of spontaenous intracranial hypotension

A

CSF opening pressure at LP (low)
CSF analysis NORMAL
MRI - diffuse pachymengineal enhancement, frequently in assosiation with sagging of the brain, tonsilar descent and posterior fossa crowding

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40
Q

Treatment of spontaenous intracranial hypotension

A

Conservative
Epidural blood patch

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41
Q

Is the headache better or worse lying down in spontaenous intracranial hypotension

A

Better lying down

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42
Q

Test for bradykinesia

A

Finger pinch test

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43
Q

Treatment of essential tremor

A

Propranolol
Primidone

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44
Q

What is tinels sign

A

Symptoms reproduced when tapping over the medial nerve
Seen in carpal tunnel syndrome

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45
Q

3 rules of the effects of paresis on diplopia

A
  1. Direction in which the distance between the images is at a maximum in the direction of the action of the paretic muscles
  2. Paresis of the horizontally acting muscles tends to produce mainly horizontal diplopia
  3. The image projected further from the centre belongs to the paretic eye
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46
Q

Which arteries are most commonly affected by thromboembolic disease in lateral medually syndrome

A

Vertebral or posterior inferior cerebellar arteries

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47
Q

What is multifocal motor neuropathy with conduction block closely related to

A

Chronic inflammatory demyelinating polyneuropathy

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48
Q

Antibodies of multifocal motor neuropathy with conduction block

A

Anti-GM1 antibodies

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49
Q

Presentation of multifocal motor neuropathy with conduction block

A

Asymmetrical motor neuropathies - usually in the upper limb
Reflexes may be preserved or slightly elevated at the onset of disease (causing frequent misidentification with MND)

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50
Q

Electro diagnostic hallmark of multifocal motor neuropathy

A

Motor conduction block

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51
Q

When would you do a sural nerve biopsy

A

If suspecting a vasculitc neuropathy

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52
Q

Presentation of progressive muscular atrophy (subtype of MND)

A

Lower motor neurone weakness distal upper and lower limbs
Without sensory loss

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53
Q

What does an upper motor neurone area involve

A

Brain
Spinal cord

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54
Q

What does a lower motor neurone area involve

A

Anterior horn cell
Motor nerve roots
Peripheral motor nerve

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55
Q

Signs of an UMN lesion

A

Disuse atrophy (minimal) or contractures
Increased tone (spasticity/rigidity) +/- ankle clonus
Pyramidal pattern of weakness (extensors weaker than flexors in arms, vise versa in legs)
Hyperreflexia
Upgoing plantars (Babinskis sign)

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56
Q

Signs of a LMN lesion

A

Marked atrophy
Fasiculations
Reduced tone
Variable pattern of weakness
Reduced or absent reflexes
Downgoing plantars or absent response

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57
Q

Presentation of primary lateral sclerosis (subtype of MND)

A

UMN weakness
Spascitity
Hyperreflexia
More likely to have bulbar involvement

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58
Q

Predominant symptoms of progressive bulbar palsy (subtype of MND)

A

Speech and swallowing difficulties

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59
Q

Presentation of sagital sinus thrombosis

A

Seizures
Focal neurological deficits

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60
Q

Predisposing factors for saggital sinus thrombosis

A

Dehydration
Haematological disorders
- anti-phospholipid, thrombophilia, protein S deficency, antithrombin III deficiency, thrombocythaemia, polycythaemia
Systemic conditions
- SLE, carcinoma, Behects disease, sarcoidosis, nephrotic syndrome
Local infections e.g. mastoiditis

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61
Q

What does botulism show on electromyography

A

Repeitive nerve stimulatio

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62
Q

What gene is mutated in huntingtons disease

A

Huntington gene (HTT)

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63
Q

Features of cavernous sinus syndrome

A

Eye muscle weakness
Proptosis
Chemosis (swelling of conjunctiva)
Horner syndrome
and/or facial sensory loss

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64
Q

Key structures that run through the cavernous sinus

A

CN III (oculomotor)
CN IV (trochlear)
CN V1 and V2 (opthalmic and maxillary divisions of the trigeminal nerve)
CN VI (abducent)
Sympathetic plexus surrounding the internal carotid artery
Cavernous part of the internal carotid artery

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65
Q

Presentation of vertebral artery dissection

A

Neck pain
Symptoms of posterior circulation stroke

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66
Q

What is the most common hereditary ataxia

A

Friedrichs ataxia

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67
Q

Pathology of friedrichs ataxia

A

Degeneration of the cerebellum, certain spinal cord tracts and peripheral nerves

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68
Q

Presentation of fredrichs ataxia

A

Ataxia
Dysarthria
Optic atrophy
Hearing impairment
LDs
Sensory neuropathy
Extensor plantar responses
Diabetes
Cardiomyopathy
Pes cavus
Kyphoscolosis

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69
Q

What is the most common type of partial seizure

A

Temporal lobe epilepsy

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70
Q

Presentation of temporal lobe seizures

A

Subjective abdo symptoms
Staring
Lip smacking
Period of disorientation

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71
Q

How is the vestibulo-ocular reflex tested

A

Caloric test
- monitoring of eye movements during or following the slow injection of at least 50ml of ice cold water over 60s into each external auditory meatus in turn

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72
Q

Where is the lesion if the vestibulo ocular reflex is negative

A

Brainstem

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73
Q

Treatment for generalised tonic clonic seizures

A

Sodium valpriate
Carbamazepine
Lamotrigine

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74
Q

Treatment for focal seizures

A

Carbamazepine
Lamotrigine

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75
Q

Treatment for abscence seizures

A

Ethosuximide

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76
Q

Treatment of GBS

A

Plasma exchange therapy
IV immunoglobulin

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77
Q

CSF analysis of GBS

A

Albuminocytolocigal dissociation (i.e. elevated protein)
However may be entirely normal

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78
Q

What features make up aphasia

A

Fluency
Repetition
Comprehension

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79
Q

Where is Brocas area and what is it for

A

Inferior frontal lobe
Centre for the motor part of speech and sentence formation
EXPRESSIVE DYSPHASIA
lesions - imparied fluency, intact comprehension and impaired repetition

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80
Q

Where is wernickes area and what is it for

A

Posterior, superior temporal lobe
Centre for comprehension and planning words
Lesions - cannot understand written or spoken words, speech remains fluent but meaningless RECEPTIVE DYSPHASIA

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81
Q

Treatment of filles de la tourette sydnrome (rare - mutliple tics with both motor actions and vocalisations)

A

Risperidone

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82
Q

Visual field testing results of indiopathic intracranail HTN

A

Loss of visual acuity when changing posture
Enlargment of blind spots and circumferential restriction in visual fields
Papilloedema

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83
Q

Most common cause and subsequant treatmnet of a 6th CN palsy

A

Microvascular nerve palsy (benign prognosis)
Vascular risk be addressed
Review in 4 weeks

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84
Q

What CNs pass through the pons

A

Facial nerve
Abducens

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85
Q

What does cervical radiculopathy refer to

A

One nerve root

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86
Q

What is synringomyelia

A

Sensory loss over a cape like distribution over the upper body, rather than peripheral sensory loss

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87
Q

What happens to cause syngobulbia

A

A synrinx forms in rhe brainstem

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88
Q

Typical CT findings of HSE

A

Hypodense areas in temporal lobes

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89
Q

Features of an extradural haemorrahage

A

Injury
Brief loss of consciousness
Recovery (lucid interval)
Delayed deterioration

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90
Q

What causes an extradural haemorrhage

A

Ruptures the middle meningeal artery (trauma)

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91
Q

Features of von hippel lindau (VHL) syndrome

A

Cerebellar haemangioma
Retinal angioma
Pancreatic and/or hepatic cysts
Adrenal and renal tumours

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92
Q

WHen do cluster headaches often occur

A

Nighttime

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93
Q

Assosiated symptoms with cluster headache

A

Runny nose
Watery eye
ptosis

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94
Q

WHat can bilateral bells paly sometimes be the first presenting symptom of

A

guillian barre syndrome

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95
Q

What condition is interferon B liscened for

A

RRMS who are able to walk unaided

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96
Q

Definition of RRMS

A

At least 2 attacks of neurological dysfunction (relapses) over the previous two years

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97
Q

How to test for CJD

A

LP

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98
Q

Presentation of new variant CJD in young adults

A

Psychiatric symptoms
followed by
- non specific painful sensory symptoms - most often in lower limbs
Cognitiv impairment
pyramidal signs
myoclonus
primitive relfexes

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99
Q

MRI findings of new variant CJD

A

High signal on T2 weighed imagines in the pulvinar (posterior aspect of the thalamus)

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100
Q

What are dorsal midbrain lesions assosiated with

A

Failure of vertical gaze

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101
Q

WHat controls vertical eye movements

A

Bilateral control of the cortex and upper brainsteam

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102
Q

Treatment of trigeminal neuralgia

A

Anti convulsants e.g. carbamazepine

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103
Q

What is hemibaslism

A

kinetic movement disorder
violent involuntary limb movements on one side of the body

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104
Q

WHat does hemibasilsm occur secondary to

A

Damage to the subthalamic nucleus (stroke, tumour, abscess)

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105
Q

Treatment of hemibasilsm

A

Dopamine antagonists

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106
Q

Treatment of cluster headache attacks

A

Sumatriptan injection
Oxygen inhalation

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107
Q

Prevention of cluster headaches

A

Verapamil
Lithium

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108
Q

Presentation of classic synringomyelia

A

Comonly assosiated with Arnold CHairai malformation and presents at 20-30 yrs of age
Upper limb pain excerbated by coughing or laughing
Pain and temp loss leads to painless upper limb burns and trophic changes
Difficulty in walking and paraparesis develop later
Gradually progressive condition

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109
Q

What is suggestive of an axonal neuropathy on a nerve conduction studies

A

Reduced compound muscle action potential amplitude

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110
Q

Myelin disorders nerve conduction studies

A

Reduction in velocities or blocks

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111
Q

Axonal disorders nerve conduction studies

A

Loss of action potential size/amplitude

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112
Q

WHat drugs commonly exacerbates weakness in myasthenia gravis

A

D-penicillaemine
Aminoglycoside and fluroquinolone antibiotics
Antiarrythmics e.g. quinidine, procainamide
B blockers e.g. propranolol
Neuromuscular blocking agents e.g. succinycholine

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113
Q

What is highly suggestive of spinal bulbar muscular atrophy (kennedy syndrome)

A

Perioral fasciculations
Androgen insensitivity

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114
Q

WHat is the safest anti epileptic to use in pregnancy

A

Lamotrigine

115
Q

WHat nerve is commonly injured in THRs

A

Sciatric nerve

116
Q

WHo is at higher risk of cavernous sinus thrombosis

A

Pregnancy
Acne

117
Q

Treatment of tourette syndrome

A

Risperidone

118
Q

Ocular pathology assosiated wit arnold chiari and formaen magnum lesions

A

Downbeat nystagmus

119
Q

Typical features of fascioscapulohumeral muscular dystrophy (FSHMD)

A

Variable condition
May go unrecognised until later in life
Facial weakness
Winging of the scapula
Proximal upper limb weakness
Foot drops

120
Q

What would distal wasting and pes cavus indicate

A

Very chronic neuromuscular disorder with axonal loss

121
Q

Presentation of subacute combined degeneration of the cord due to B12 deficiency

A

Selective involvement of the dorsal columns and the pyramidal tracts
Evidence of peripheral nerve invovlement
50% absent ankle jerks with hyperreflexia at the knees
Plantars inititally flexor, then extensor

122
Q

WHat is fibromuscular dysplasia

A

Non atherosclerotic, non inflammatory condition that principally affects the carotid and renal arteires

123
Q

Assosiations of fibromuscular dysplasia

A

Ehler Danlos
Marfans
Phaechromocytoma
Takayasus disease

124
Q

MRI appearance of fibromuscular dysplasia in carotid arteries

A

String of beads appearance (medial hyperplasia)

125
Q

Causes of both UMN and LMN signs

A

Vitamin B 12 deficiency (subacure combined degeneration of the cord with peripheral neuropathy)
Tertirary syphillis (taboparssis)
MND
Friedrichs ataxia
Conus medullaris lesion
DM

126
Q

WHat would indicate a lacunar infarct affecting the corticospinal tract at the level of the internal capsule or the upper pons

A

Weakness inolcing face, arm and legs
Absence of language disturbance, hemineglect or visual field deficit

127
Q

What does mild sensory changes in temperature and touch sensations favour

A

A small infarct

128
Q

What is multifocal motor neuropathy

A

Inflammatory neuropathy that causes multi focal demyelination with conduction block

129
Q

Features of mutlifocal motor neuropathy

A

Progressive weakness and wasting
Intermittent fasiculations
Nil sensory features
Nerve conduction studies - demyelination and conduction block, normal sensory conduction

130
Q

Poor prognostic factors of GBS

A

> 40
Rapid symptom progression
Requirment for ventilatory support
Previous Hx diarhroeal illness (specifically campylobacter)
High Anti-GBM antibody titre

131
Q

Presentation of GBS

A

Ascending paralysis
Flaccid muscle tone
Hyporeflexia/areflexia
Autonomic disturbances
Neuromuscular ventinatory failure

132
Q

CSF findings of GBS

A

High protein
normal otherwise

133
Q

Presentation of common peroneal nerve injury

A

Failure to dorsiflex and evert foot
Senosry loss over dorsum of foot and anterolateral leg

134
Q

WHat is internuclear opthalmoplegia caused by

A

Lesion in the medical longitudinal fasiculus (MLF) which connects the sixth nerve nuclei (supplying the lateral rectus muscle, which controls abduction) to the contralateral third nerve nuclesus (supplying medial rectus, which controls adduction)

135
Q

What does unilateral INO (internuclear opthlamoplegia) cause

A

Loss of adduction of the ipsulateral medial rectus on attempted conjugate gaze
Abducting nystagmus of the contralateral eye

136
Q

What is bilateral INO strongly assosiated with

A

MS

137
Q

Causes of internuclear opthamoplegia (IMO)

A

MS
Cerebrovascular disease

138
Q

Prophylaxis of migraine

A

Propranolol
Topiramate

139
Q

Causes of rapidly progressive dementing syndrome

A

Sporadic CJD
HIV related disease
CNS vasculitis
Limbic encephalitis

140
Q

Is sensation preserved or affected in MND

A

Preserved
(unless co exists with another pathology e.g. diabetic neuropathy)

141
Q

What makes up neuropathies

A

Reflex loss
Sensory disturbance

142
Q

Where are roth spots found and what are the causes

A

FOUND IN THE EYES - white cenetered haemorrhages consisting of perivascular collections of lympchytes
Carbon monoxide poisoning
Myeloma
Trauma
Hypoxia
Anaestheia
PET
HIV retinopathy

143
Q

Abnormality in which vessels are most frequently asosiated with lateral medually syndrome

A
  1. Vertebral artery
  2. Posterior inferior celebellar artery
  3. Superior middle and inferior medually arteries
144
Q

WHat sensory disturbance occurs with common peroneal nerve palsy

A

Dorsum of the foot

145
Q

Where is the lesion to cause a homonymous hemianopia

A

Occipital cortex

146
Q

Presnetaiton of pos ttraumatic synringomyelia

A

Loss of pain and temp sensation
Preservation of light touch
Weakness

147
Q

Pathology of synringomyelia

A

Synrinx is a fluid filled cyst existing in the spinal cord, compresses the anterior horn (LMNs) and spinothalamic tract (pain and temp sensation)
Dorsal colums (light touch and proprioception) are usually spared but as the syrinx expands it may involves these tracts

148
Q

Diagnosis of syringomyelia

A

MRI

149
Q

What may be seen preceeding an enterovirus meningitis

A

Cold / diarrhoeal illnesses

150
Q

Antibodies of paraneoplastic cerebellar degeneration

A

Anti bodies against the ani-Yo antigen

151
Q

Cerebellar signs

A

Dysdiadochinesia
Ataxia (gait and posture)
nystagmus
intention tremor
Slurred, staccato speech
Hypotonia

152
Q

What is ramsay hunt syndrome caused by

A

Infection of the geniculate ganglion of the facial nerve , together with the invasion of the VIIIth nerve ganglia, by the herpes zoster virus

153
Q

Presentation of ramsey hunt syndrome

A

Ear pain
Hearing loss
Vertigo
Facial nerve paralysis
Vesicular rash on outer ear, sometimes also the soft apalate or anterior 2/3rds of the tognue

154
Q

CN II

A

Optic nerve
(NO MOTOR FUNCTION)

155
Q

CN III, IV and VI

A

Oculomotor, trochlear and abducens nerves
MOTOR EYE AND EYELID FUNCTION
oculomotor - parasympathetic fibres for pupillary constrictoon

156
Q

CN V

A

Trigeminal nerve (3 subdivisons)
Senosyr information about facial sensation and motor to the muscles of mastication

157
Q

CN VII

A

Facial nerve
MOTOR TO THE MUSCLES OF FACIAL EXPRESSION
SENSORY COMPONSANT TO ANTEIOR 2/3RDS OF THE TONGUE

158
Q

CN VIII

A

Vestibulocochlear nerve
SENSORY ABOUT SOUND AND BALANCE

159
Q

CN IX AND X

A

Glossopharyneal and vagus
glossopharyneal - elevates pharynx during swallowing and speech. sensory to posterior togue
Vagus - motor to several muscles of the mouth and gag reflex

160
Q

CN XI

A

Accessory nerve (SCM and trapezius muscles)

161
Q

CN XII

A

Hypoglossal nerve
Motor - extrinsic muscles of the tognue

162
Q

Diagnosis and Tx of BPPV

A

Diagnosis - Dix hallpike
Treatment - Epley

163
Q

Presentation of subacute degeneration of the spinal cord (i.e. vit B12 deficiency)

A

Myeloneuropathy
Consists of spinal cord signs and peripheral neuropathic signs

164
Q

Cuases of mononeuritis multiplex

A

Vasculitis
Diabetes
Sarcoidosis
Paraneoplastic syndrome
Amyloidosis

165
Q

Presentation of brown sequard sundrome (hemisection of the cord)

A

Ipsilateral paralysis
Ipsilateral loss of vibration and position sense
Hyperreflexia below level of the lesion
Contralteral loss of pain and temp sensation (usually begin 2 or 3 segements below the level of the lesion)
(In a clinical situtaiton - a mix of these signs is usually seen as a hemisection is rarely complete)

166
Q

Features of post infectious transverse myelitis

A

Complete cord syndrome with involvement at sensory level, vibration loss and UMN signs
Predominatly lymphocytes in CSF, with normal protein and glucose

167
Q

Driving restriction for both strokes and TIAs

A

1 month

168
Q

What does amyloidosis assosiated neuropathy classically assosiated with

A

Autonomic neuropathy

169
Q

Causes of autonomic neuropathy

A

MSA
PD
Wernickes encephalopathy
Syndrinmyelia/bulbia
Famial dysautonmia
Familal amyloidosis
GBS
DM
Tabes dorsalis
Alcoholic nutritional neuropathy
LEMS

170
Q

Mechanism of action of ropinirole

A

Dopamine receptor agonist

171
Q

What is webers syndrome and presentation

A

A midbrain stroke syndrome usually occuring as a result of occlusion of the paramedian branches of the posterior cerebral artery
Presents as
- ipsilateral CNIII palsy
- contralaterla hemiparesis and hemiparkinsonism due to the involvement of the corticospinal tract and substanstia nigra, respectively The crossed nature of these ffeatures (i.e. contralteral to the CNIII palsy) indicates that the lesion is within the brainstem and above the medually decussation

172
Q

is mononeuritis painful?

A

YES

173
Q

What is holmes aide pupil thought to be secondary to

A

A mild autonomic neuropathy

174
Q

What is addies tonic pupil commonly assosiated with

A

Areflexia (loss of deep tendon reflexes)

175
Q

RFs for alzheimers

A

Vascular risk factors
FH
APOE4 genotype
Traumatic brain injury
Downs syndrome

176
Q

What level does the spinal cord end at

A

L1

177
Q

What is miller fischer syndrome

A

Rare variant of guillane barre syndrome
Begins with proximal muscle weakness, affecting the eye muscles then spreading distally
(Contrast to GBS which begins peripherally in the lower limbs)

178
Q

Features of fascioscapulohumeral muscular dystrophy

A

Facial, proximal liimb and abdominal muscle weakness
Winged scapulae
Footdrop
Elevated CK

179
Q

What should be used i nthe context of SAH to reduce chances of cerebral artery vasospasm, risk of cerbral infarct and worsening neurological outcomes

A

Nimodipine

180
Q

Presentation of cauda equina vs cons medullaris syndrome

A

Cauda equina
- gradual and unilateral onset
- both ankle and knee jerks affected
- More severe radicular pain
- Less lower back pain
- Numbness tends to be more localised to the saddle area, asymmetrical and may be unilateral. No sensory dissociation, Loss of specific dermatomes in lower limbs
- Asymmetric areflexic paraplegia, fasciculations rare, atrophy common
- urinary retention tends to occur late course of the disease

Cons medullaris syndrome
- Sudden onset with bilateral symptoms
- Knee jerks preserved by ankle jerks affected
- Less severe radicular pain but more severe back pain
- Numbness tends to be more perianal area, symmetrical and bilateral, sensory dissociation occurs
- Symmetrical hyperreflexic distal paresis of lower limbs, fasciulations may be present
- Urinary and faecal incontinence

181
Q

Presentation of inclusion body myositis

A

Slowly progressive weakness
Wasting of finger flexor and quadriceps

182
Q

10-15% of people with myasthenia gravis have a what

A

Thymoma

183
Q

What should you consider when exercise has caused a stroke

A

Dissection of a carotid artery
Cardioembolism from an ASD

184
Q

Most common cause of stroke in patients < 40 with no vascular risk factors

A

Carotid artery dissection

185
Q

What is meralgia paraesthetica

A

A syndrome which includes numbness, paraesthesia and pains in the anterolateral thigh, resulting from either entrapment neuropathy or neuroma of the lateral femoral cutaneos nerve
Obsesity / weight gain a risk factor

186
Q

People with MS commonly feel their symptoms get worse with what

A

Exercise
Heat

187
Q

Multifocal motor neuropathy is most commonly assosiated with which of the following

A

Anti-GMB1 antibodies

188
Q

What does the sciatic nerve divide into

A

Tibial nerve
Common peroneal nerve

189
Q

What innervates flexion of the hip

A

Femoral nerve

190
Q

What is the major determinant of the need for intubation and ITU admission in GBS

A

Forced vital capacity (FVC)

191
Q

WHat is menieres disease

A

Paroxysmal episodes of vertigo, N/V, and deafness lasting for hours
Audiogram - unilateral low frequeny sensorineural deafness

192
Q

Treatment of menieres disease

A

Acute attacks - prochloperazine, cyclizine
Prophylaxis - betahistine, low salt diet

193
Q

Presentation of vestibular schwannoma

A

Unilateral hearing loss
Tinnitus
Trigeminal neuropathy

194
Q

Treatment of an acute attack of ocular myasthenia gravis

A

Pyridostigmine - initially 30mg QDS PO for 2-4 days, titrate up to find the lowest effective dose

195
Q

What innervates the intrinsic muscles of the hand

A

T1 nerve root

196
Q

What artery supplies brocas area

A

Brocas area is in the dominant frontal lobe
Middle cerebral artery

197
Q

What does a history of coming down the stairs and the prescence of downbeat nystamus highly suggestive of

A

Structural lesion at the foramen magnum .e.g chairi malformation

198
Q

What post op visual field deficit may be seen in a unilateral temporal lobe lobectomy

A

Superior homonymous quadrantonopia

199
Q

MRI signs of new CJD variant disease

A

Increased signal in the pulvinar of the thalamus (pulvinar sign)

200
Q

Where usually is the site of damage in dysarthria clumsy hand syndrome

A

Internal capsule
Pons

201
Q

Key clue to P dementia with lewy bodies

A

Hallucinations

202
Q

What does the radial nerve supply and why is it particularly susceptible to injury

A

muscles controlling elbow, wrist and finger extension
Sensation over the posterior forearm and small patch at the base of the thumb

Susceptible to compression or traumatic damage as it winds around the humerus

203
Q

Nerve conduction studies results in peripheral neuropathy secondary to DM

A

Decreased sensory nerve amplitude responses

204
Q

WHat is the most common form of genetic vascular dementia

A

Cerebral autosomal dominant arteriopathy with subcottical infarcts and leukoencepalopathy (CADASIL)

205
Q

Presentation of CADASIL

A

Variable phenotype
High prevalnce of migraine with aura (often atyical aura)
Strokes at young age
Easy vascular subcortical dementia

206
Q

How does parietal lobe damage manifest

A

Defect of inattention in the contralateral visual field
Asteregnosis
Constructional apraxia
Dressing apraxia
Ideomotor aprazia
Right hemisphere (i.e. non dominant) parietal lesions particularly prone to producing visual neglect

207
Q

What is amaurosis fugax

A

Painless, transient, monocular vision loss (TIA)

208
Q

Issues with treatment of dementia with lewy bodies

A

It can be hard to treat the parkinsonism, as levodopa therapy may worsen the confusion

209
Q

What surgical procdure is preferred for a cerebral aneurysm

A

Endovascular coil

210
Q

Syringomyelia vs syringobulbia

A

Synribulbia - lower brainstem is involved e.g. tongue fasiculations

211
Q

Presentation of myotonic dystrophy type 1

A

AD condition
Ptosis
Facial weakness
Dysphagia
Sleep apnoea
Distal weakness
atrophy of the temporalis
Frontal balding
Cardiac conduction problems
Cataracts
Impaired mental function

212
Q

Is lamotrigine safe to use in pregnancy

A

Yes

213
Q

Is valoproate safe to use in pregnancy

A

No

214
Q

Is phenytoin safe in pregnancy

A

No

215
Q

WHat does diabetes increase the suscepitbility of your peripheral nerves to

A

Damage/compression

216
Q

What nerve is affected to cause pain and sensory abnormalities in the anterolateral thigh

A

Lateral cutaneous nerve of the thigh

217
Q

Which side is the lesion on in INO regarding symptoms

A

Lesion on the side of the eye that fails to adduct

218
Q

Function of the medial longittudinal fascilucus

A

Brainstem structure connecting the 6th and 3rd nerve nuclei on opposite sides of the brainstem in order to coordination abduction of one eye with the adduction of the other (horizontal conjungate gaze)

219
Q

What is chairari type 1 malformation

A

Elongated cerebellar tonsils are displaced into the upper cervical canal through the formamen magnum
Symptoms develop as a result of disordered anatomy, compression of the medulla and upper spinal cord, compression of the cerebellum and disruption of CSF flow through the formaen magnum
Weakness
Unsteadiness
Headache / neck pain
Nystagmus
Cerebellar signs
Dissosiated sensor loss (cape anaesthesia)

220
Q

What is chairi type 2 malformation

A

Also known as the arnold - chairi malformation
Presents at a younger age than type I with symptoms of brainstem dysfunction

221
Q

90% of patients who have syringomyelia also have what

A

Type 1 chiari malformation

222
Q

Where in the brain is affected in wilsons disease

A

Basal ganglia

223
Q

What do nasal regurgitations and a weak cough point towards

A

Bulbar dysphagia

224
Q

Prophylaxis of cluster headache

A

Verapmil
Lithium
Sodium valproate
Gabapentin

225
Q

Triad of miller fisher syndrome

A

Opthalmoplegia
Ataxia
Areflexia

226
Q

What is miller fiseher syndrome

A

Variant of GBS
Affects the brainstem, Cranial nerves and peripheral nerves
Like GBS, often follows an infective illness

227
Q

CSF findings in miller fisher syndrome

A

CSF protein high
No cellular response (cytoalbuminologic dissosiation)

228
Q

Which antibody is assosiated with miller fisher syndrome

A

Antiganglioside antibody GQ1B

229
Q

Treatment of miller fisher syndrome

A

Immunoglobulins and plasma exchange for those with more severe disease (i.e. non ambulant)

230
Q

What aneurysm is characteristically assosiated with a third nerve palsy

A

A posterior communicating artery aneurysm

231
Q

Features of middle cerebral artery aneurysm

A

Contralateral hemiparesis
Hemi sensory loss in the face, upper and lower extremetiries

232
Q

Features of anterior communicating artery aneurysm

A

Bitemporal heminaopia

233
Q

MRI findings of subacute combined degeneration of the cord

A

Increased signal on T2 weighted imaging, predomiantely in the dorsal columns

234
Q

Features of holmes aide syndrome and prognosis

A

A tonic pupil that is usually larger, fails to react to light, with sluggish reaction to accomodation
Diminished reflexes
BENIGN CONDITION

235
Q

Features of argyll robertson pupil

A

Small, usually bilateral
React to accomodation, but not to direct light

236
Q

What does essential tremor tend to improve with

A

Alcohol

237
Q

What needs checked before immunoglobulin therapy for the diagnosis of miller fisher syndrome

A

IgA levels

238
Q

What is inclusion body myositis

A

Idiopathic inflammatory myopathy
Most common acquired myopathy in those > 50 y/o and more common in men

239
Q

Most common acquired myopathy in patients > 50

A

Inclusion body myositis

240
Q

Features of inclusion body myositis

A

Patterns of muscle weakenss (e.g. quads and long finger flexors)
Asymmetry of signs
Falls
Normal CK

241
Q

Strokes affecting the occipital lobe will cause what

A

Contralateral homoonymous hemaniopia with macular (central field) sparing

242
Q

Criteria for IV steriods rather than oral in patients with MS

A

Oral steriods failed to be tolerated
Who need admitted to hospital for severe relapse or monitoring of medical or psychollogical conditions, such as diabetes or depression

243
Q

Treatment of relapse of MS

A

Oral merthylpresnisolone 0.5g daily for 5 days

244
Q

What is the commonest inherited polyneuropathy

A

Charcot marie tooth disease (known as hereditary motor sensory polyneuropathy)

245
Q

Treatment of paroxysmal hemicrania

A

Indomethacin
Salicylates, NSAIDs and prednisolone may help

246
Q

CSF findings of MS

A

Oligoclonal bands
Increased rates of IgG synthesis

247
Q

What is brown sequard syndrome most commonly seen in

A

MS

248
Q

CT head findings that support the diagnosis of NPH

A

Enlarged ventricles

249
Q

1st line treatment of cervical dystonia

A

Botulinum toxin

250
Q

What is picks disease also known as

A

Frontotemporal dementia

251
Q

Features of injury to the posterior interosseous nerve

A

Deep branch of radial nerve
Purely motor syndrrome
- finger drop
- radial wrist deviation on extension

252
Q

Features of ulnar neuropathy

A

finger abduction, adduction and flexion weakness

253
Q

What would a lesion / stroke in the vestibular nuclei cause

A

Vomiting
Vertigo
Nystagmus

254
Q

What would a lesion / stroke in the inferior cerebellar peduncle cause

A

Ipsilateral cerebellar signs
- atazia
- dysmetria
0 dysdiadochokinsea

255
Q

What does a laesion in the lateral spinothalamic tract casuse

A

Contralteral deficits in pain and temp sensation

256
Q

What does a lesion in the spinal trigeminal nucleus and tract cause

A

Ipsilateral loss f pain, temp sensation from face

257
Q

What would a lesion in the dececning sympathetic fibres cause

A

Ipsilateral horners syndrome

258
Q

Presentation of lead poisoning

A

Abdo pain and vomiting
Reduced distal power
Absent reflexes

259
Q

Tests for lead poisoning

A

Blood film - basophilic stripping
Elevation in urinary delta-ALA level

260
Q

What is typically spared in anterior spinal artery occlusion and why

A

Proproiception and vibration sense spared
They are conducted in the posterior columns

261
Q

What is semantic dementia

A

A form of FTD
Very speficic deficic in semantic memory (the ability to asssosiate meaning to objects presented via visual or audotry modalities)
Pathology primarily affects the temporal lobes

262
Q

3 types of frontotemporal dementia

A

Behavioural variant
Semantic dementia
Progressive non fluent aphasia

263
Q

What supplies the thenar eminence

A

Median nerve
(minus the adductor pollicus, which is ulnar)

264
Q

L5 radiculopathy affects 3 nerves;

A

PERONEAL NERVE
- ankle dorsiflexion
- ankle eversion
TIBIAL NERVE
- ankle inversion
SUPERIOR GLUTEAL NERVE
- hip abdcution
- leg internal rotation

Sensation over anterior shin and dorsum of the foot

265
Q

What would indicate an occlusion of the middle superior cerebral artery rather than the anterior cerebral artery

A

Contralateral hemiparesis that affects the face, arm and hands but with RELATIVE SPARING OF THE LEGS
Contralteral hemisensory deficit in the same distribution
No homonymous hemianopia
If dominant hemisphere involved - expressive aphasia

266
Q

Treatment of CVST

A

LWMH
IV fluids

267
Q

What does anticipation mean in terms of inheritance

A

Symptoms begin at an earlier stage in successive generations

268
Q

What does the weakness in LEMS tend to improve with

A

Exertion

269
Q

What nerves are supplied by the S1 nerve root

A

INFERIOR GLUTEAL NERVE
- hip extension
SCIATIC NERVE
- knee flexion (hamstrings)
TIBIAL NERVE
- ankle plantar flexion

Sensation over the plantar aspect of the foot and the skin overt the achilles tendon

270
Q

40% of patients with myotonic dystrophy may develop what

A

LV dysfunction

271
Q

Painful 3rd nerve palsy is what until proven otherwise

A

Posterior communicating artery aneurysm

272
Q

Tendon reflexes in spinal cord compression

A

Increased below the level of the lesion
Absent at the level of the lesion

273
Q

What points towards osteomalacia

A

Low calcium and low phosphate
Raised ALP

274
Q

Cause of osmotic demyelination (when correcting hyponatraemia)

A

Astrocyte apoptosis

275
Q

Neurological findings of Vit B12 deficiency

A

Impaired proprioception, and fine touch

276
Q

Who is vit B12 deficiency seen in

A

Abusers of nitrous oxide

277
Q

Where would the lesion be if a patient had anomia with impaired repetition, but otherwise fluent speech and good reading comprehension

A

Subcortical lesion near the left superior temporal gyrus (i.e. the dominant hemisphere)

278
Q

Pathology of lambert eaton myasthenic syndrome

A

antibodies against voltage gated calcium channels

279
Q

Treatment of lambert eaton myasthenic syndrome

A

Treatment of underlying cancer AND
Amifampridine

280
Q

What produces CSF

A

Choroid plexus

281
Q

Where is CSF absorbed

A

Arachnoid granulations

282
Q

What drugs can precipitate a myasthenia crisis

A

BETA BLOCKERS
Macrolide and quinolone antibiotics
Statins

283
Q
A