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Phase 3a > Neuro 2 - Movement, Autoimmune, Spinal cord > Flashcards

Neuro 2 - Movement, Autoimmune, Spinal cord Flashcards

1
Q

What is the inheritance pattern in Huntington’s disease?

A

Autosomal dominant
Chromosome 4 mutation
Repeated CAG expression

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2
Q

What is the mean age of onset of Huntington’s disease?

A

30-50 years

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3
Q

What is the pathophysiology of Huntington’s disease?

A

Cerebral atrophy
Loss of neurones in the caudate nucleus and putamen of basal ganglia
Decreased ACh and GABA in striatum

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4
Q

What occurs in the prodromal phase of HD?

A

Mild psychotic and behavioural symptoms

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5
Q

What is chorea?

A

Progressive, jerky, explosive movements

May begin as restlessness and lack of co-ordination

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6
Q

What are the late symptoms of HD?

A

Rigidity, dysarthria, dysphagia, abnormal eye movements

Behavioural change and aggression

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7
Q

Give three causes of chorea

A

Huntington’s disease
Syndenham’s chorea (rheumatic fever)
CJD

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8
Q

How is chorea treated symptomatically?

A

BDZs
Valproic acid
Tetrabenazine

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9
Q

What is the mechanism of action of tetrabenazine?

A

Dopamine depleting agent

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10
Q

What is motor neurone disease?

A

Progressive degeneration of motor neurones in the anterior horns, CN motor nuclei, and within the cortex

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11
Q

What percentage of MND is hereditary?

A

10%

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12
Q

What are the four types of MND?

A

Amytrophic lateral sclerosis
Progressive bulbar palsy
Progressive muscular atrophy
Primary lateral sclerosis

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13
Q

What is affected in ALS?

A

Both UMN and LMN

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14
Q

What neurones are affected in PLS, PMA, and PBP?

A

PLS - UMN
PMA - LMN
PBP - UMN and LMN of lower cranial nerves

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15
Q

What are the three patterns of onset in MND?

A

Limb
Bulbar
Respiratory

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16
Q

What investigations are required in MND?

A

Clinical, EMG/nerve conduction studies

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17
Q

What drug is licensed for treatment of MND?

A

Riluzole

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18
Q

Give three UMN signs

A 
Weakness
Hypertonia
Brisk reflexes
Positive Babinski
Clonus
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19
Q

Give three LMN signs

A 
Weakness
Decreased reflexes
Hypotonia
Wasting
Fasciculations
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20
Q

What is a risk factor for MS?

A

Male

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21
Q

What is the pathophysiology of MS?

A

T-cell mediated demyelination at multiple CNS sites - discrete plaques
Autoantibodies

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22
Q

What are the subtypes of MS?

A

Relapsing-remitting (80%)
Secondary progressive
Primary progressive

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23
Q

What is the difference between primary and secondary progressive MS?

A

Secondary - follows from relapsing-remitting MS

Primary - progressive from the start

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24
Q

What is Uhthoff’s phenomenon?

A

Symptoms worsen with heat

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25
Q

Give three symptoms of MS

A 
Unilateral optic neuritis
Motor/sensory disturbance
Brainstem/cerebellar symptoms
UMN signs
Fatigue
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26
Q

What are the symptoms of optic neuritis?

A

Visual impairment (exaggerated by hot bath)
Eye pain worse on movement
Dyschromatopsia (especially for red)

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27
Q

What are brainstem/cerebellar symptoms

A

Diplopia
Ataxia
Vertigo
Dysphagia

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28
Q

What is Lhermitte’s sign?

A

Neck flexion causes electric shocks in trunk and limbs

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29
Q

What suggests demyelination in electrophysiology studies in MS?

A

Delayed nerve conduction

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30
Q

What investigations are required in MS?

A

MRI

LP: increase protein, oligoclonal bands of increased ig concentration

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31
Q

What is the diagnostic criteria for MS?

A

2 or more CNS lesions disseminated in time and space

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32
Q

What is the treatment of an MS relapse?

A

Oral methylprednisolone 5 days

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33
Q

What is disease modifying agents in MS?

A

Alemtuzumab (CD52 monocloncal antibody)
Dimethyl fumarate
Natalizumab

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34
Q

How is myasthenia gravis diagnosed?

A

Anti-AchR or anti-MUSK antibodies
CT of thymus for hyperplasia
Tensilon test
Repeat nerve stimulation - decreased evoked muscle AP

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35
Q

What is the tensilon test?

A

IV edrophonium given - power increases within seconds

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36
Q

What is the treatment of MG?

A

Pyridostigmine
Prednisolone for relapses
Thymectomy

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37
Q

What are some cholinergic side effects?

A

Increased salivation
Lacrimation
V+D
miosis

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38
Q

Where is the most common disc herniation?

A

L4/L5

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39
Q

What is myelopathy?

A

Spinal cord compression with UMN signs

40
Q

What is radiculopathy?

A

Spinal root compression with LMN signs

41
Q

L4/L5 herniation leads to what root nerve compression and why?

A

L5

Nerve root comes out above the disc

42
Q

What is the gold standard diagnosis of spinal cord compression?

A

MRI

43
Q

What is cauda equina?

A

Nerve root compression caudal to the termination of the cord - usually S1-S5

44
Q

What are the signs of cauda equina syndrome?

A 
Pain in legs
Variable leg weakness
Saddle anaesthesia
Poor anal tone
Erectile dysfunction
Bladder/bowel dysfunction
45
Q

What is the diagnosis and management of cauda equina?

A

MRI

Refer to neurosurgery ASAP risk of permanent damage

46
Q

In which conditions is carpal tunnel syndrome seen (median nerve entrapment at the wrist)

A

Pregnancy 3rd trimester
Hypothyroidism
Acromegaly
Rheumatoid arthritis

47
Q

Give some general symptoms of neuropathy

A 
Sensation change
Changes in sweating
Orthostatic hypotension
Constipation
Impotence
Trophic changes, pale feet
48
Q

What test are used to diagnose carpal tunnel syndrome?

A

Phalen’s

Tinel’s - tapping elicits tingling

49
Q

What is the treatment of carpal tunnel syndrome?

A

Wrist splint
Local steroid injection
Surgical decompression - division of flexor retinaculum

50
Q

What investigations must you do in syncope?

A

ECG - heart block or arrhthmias
Cardiac enzymes
Sitting and standing blood pressure

51
Q

What are the symptoms of Brown-Sequard syndrome?

A

Ipsilateral weakness
Ipsilateral impaired fine touch, proprioception, vibration
Contralateral impaired pain and temperature

52
Q

When is the peak incidence of Bell’s palsy?

A

20-40 years

53
Q

What is the management of Bell’s palsy?

A

Prednisolone

Artificial tears and eye taping at night (prevents conjunctivitis from lack of blinking)

54
Q

How can you tell the difference between Bell’s palsy and an upper motor neuron palsy?

A

The forehead is affected in Bell’s palsy

55
Q

What are the medications licensed for neuropathic pain?

A

Duloxetine
Pregabalin
Amitryptyline
Gabapentin

56
Q

What is mononeuritis multiplex?

A

Sequential involvement of non-contiguous nerve trunks

Acute loss of motor/sensory function of nerves

57
Q

What is a contraindication of triptans?

A

Ischaemic heart disease or cerebrovascular disease

58
Q

What is the treatment of chronic inflammatory demyelinating polyneuropathy?

A

Steroids

59
Q

What are the triggers of migraine?

A 
CHOCOLATE
Chocolate
Hangovers
Orgasms
Combined OCP
Lie-ins
Alcohol
Travel
Exercise
60
Q

What are the risk factors for MS?

A

Vitamin D deficiency
Previous infectious mononucleosis
Genetics
Smoking

61
Q

Where is Broca’s area found?

A

Inferior frontal gyrus

62
Q

Where is Wernicke’s area found?

A

Superior temporal gyrus

63
Q

What type of dysphasia do you get with strokes affecting Wernicke’s and Broca’s areas?

A

Broca’s - expressive

Wernicke’s - receptive`

64
Q

What is the treatment of a myasthenic crisis?

A

Plasmaphresis

IVIG

65
Q

What are the two types of seizures?

A

Partial/focal

Generalized

66
Q

What is the difference between a simple partial seizure and a complex partial seizure?

A

Simple: no loss of consciousness
Compex: loss of consciousness

67
Q

What is a secondary generalised seizure?

A

Partial seizures may spread after a few seconds due to failure of inhibitory mechanisms

68
Q

What is a generalised seizure?

A

Simultaneous involvement of both hemispheres, always loss of consciousness/awareness

69
Q

What are two possible characteristics of simple partial seizures?

A

Jacksonian march

Todd’s paralysis

70
Q

From where do complex partial seizures usually arise?

A

Temporal (60%) or frontal lobe

71
Q

What are the characteristics of a complex partial seizure?

A

Aura
Loss of awareness
Post-ictal confusion

72
Q

What are the types of generalised seizure?

A 
Absence
Tonic-clonic
Myoclonic
Tonic
Atonic
73
Q

What are the characteristics of absence seizures?

A

3Hz spike and wave activity on EEG
Loss of awareness, vacant expression for 10 seconds
No motor manifestations except eyelid flutter

74
Q

What are the characteristics of tonic-clonic seizures?

A 
Stiffening of the limbs, then synchronous jerking of limbs
Eyes open
Tongue bitten
Incontinent of urine/faeces
Post-ictal phase
75
Q

What occurs in the post ictal phase?

A

Flaccid unresponsiveness
Gradual return to awareness
Confusion and drowsiness
Lasts 15m - hour

76
Q

What are the two main types of primary generalised epilepsy?

A

Childhood absence epilepsy

Juvenile myoclonic epilepsy

77
Q

What is childhood absence epilepsy?

A

Begins 5-7 years

Absence seizures

78
Q

What is juvenile absence epilepsy?

A

Begins in older children/teenagers
Absence seizures
Longer seizures

79
Q

What is juvenile myoclonic epilepsy?

A

Begins in teenage years
GTCS, myoclonic, absence
Occur early in the morning

80
Q

What are some triggers for seizures in JME?

A

Strobe lighting
Alcohol
Decreased sleep

81
Q

What EEG finding is seen in JME?

A

Spike and wave activity

82
Q

What drug is likely to worsen myoclonic seizures?

A

Lamotrigine

83
Q

What is the treatment of myoclonic seizures?

A

Valproate or levetiracetam

84
Q

What is the treatment of tonic/atonic seizures?

A

Sodium valproate

85
Q

What is genetic anticipation?

A

Hereditary diseases have an earlier age of onset through generations
Trinucleotide repeat disorders - Huntington’s, myotonic dystrophy

86
Q

What is the inheritance pattern in essential tremor?

A

Autosomal dominant

87
Q

What are the characteristics of essential tremor?

A

Bilateral

Worsens when both arms are outstretched

88
Q

What is the first line treatment of essential tremor?

A

Propanolol

89
Q

What is cataplexy?

A

Sudden and transient weakness after strong emotions

90
Q

What is associated with cataplexy?

A

Narcolepsy

91
Q

What are three side effects of levodopa?

A

Postural hypotension
On-off effect/dyskinesia
Cardiac arrhythmias
Psychosis

92
Q

What common class of drug is associated with precipitating a myasthenic crisis?

A

Beta-blockers

93
Q

What is ataxia caused by?

A

Lesions in the cerebellum:
Gait ataxia without limb ataxia: cerebellar vermis
General ataxia: cerebellum

94
Q

What is limb ataxia?

A

Past pointing, finger-nose ataxia etc

95
Q

What do lesions of the parietal lobe produce?

A

Sensory symptoms, dyslexia, dysgraphia

96
Q

What are the symptoms of a vestibular schwannoma?

A

Unilateral deafness
Tinnitus
Absent corneal reflex

97
Q

Give three causes of tremor, other than Parkinson’s disease, and essential tremor

A

Carbon dioxide retention - flap when outstretched
Thyrotoxicosis - thyroid signs
Cerebellar - intention tremor
Anxiety

Phase 3a (18 decks)

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