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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
Give three symptoms of MS
``` Unilateral optic neuritis Motor/sensory disturbance Brainstem/cerebellar symptoms UMN signs Fatigue ```
26
What are the symptoms of optic neuritis?
Visual impairment (exaggerated by hot bath) Eye pain worse on movement Dyschromatopsia (especially for red)
27
What are brainstem/cerebellar symptoms
Diplopia Ataxia Vertigo Dysphagia
28
What is Lhermitte's sign?
Neck flexion causes electric shocks in trunk and limbs
29
What suggests demyelination in electrophysiology studies in MS?
Delayed nerve conduction
30
What investigations are required in MS?
MRI | LP: increase protein, oligoclonal bands of increased ig concentration
31
What is the diagnostic criteria for MS?
2 or more CNS lesions disseminated in time and space
32
What is the treatment of an MS relapse?
Oral methylprednisolone 5 days
33
What is disease modifying agents in MS?
Alemtuzumab (CD52 monocloncal antibody) Dimethyl fumarate Natalizumab
34
How is myasthenia gravis diagnosed?
Anti-AchR or anti-MUSK antibodies CT of thymus for hyperplasia Tensilon test Repeat nerve stimulation - decreased evoked muscle AP
35
What is the tensilon test?
IV edrophonium given - power increases within seconds
36
What is the treatment of MG?
Pyridostigmine Prednisolone for relapses Thymectomy
37
What are some cholinergic side effects?
Increased salivation Lacrimation V+D miosis
38
Where is the most common disc herniation?
L4/L5
39
What is myelopathy?
Spinal cord compression with UMN signs
40
What is radiculopathy?
Spinal root compression with LMN signs
41
L4/L5 herniation leads to what root nerve compression and why?
L5 | Nerve root comes out above the disc
42
What is the gold standard diagnosis of spinal cord compression?
MRI
43
What is cauda equina?
Nerve root compression caudal to the termination of the cord - usually S1-S5
44
What are the signs of cauda equina syndrome?
``` Pain in legs Variable leg weakness Saddle anaesthesia Poor anal tone Erectile dysfunction Bladder/bowel dysfunction ```
45
What is the diagnosis and management of cauda equina?
MRI | Refer to neurosurgery ASAP risk of permanent damage
46
In which conditions is carpal tunnel syndrome seen (median nerve entrapment at the wrist)
Pregnancy 3rd trimester Hypothyroidism Acromegaly Rheumatoid arthritis
47
Give some general symptoms of neuropathy
``` Sensation change Changes in sweating Orthostatic hypotension Constipation Impotence Trophic changes, pale feet ```
48
What test are used to diagnose carpal tunnel syndrome?
Phalen's | Tinel's - tapping elicits tingling
49
What is the treatment of carpal tunnel syndrome?
Wrist splint Local steroid injection Surgical decompression - division of flexor retinaculum
50
What investigations must you do in syncope?
ECG - heart block or arrhthmias Cardiac enzymes Sitting and standing blood pressure
51
What are the symptoms of Brown-Sequard syndrome?
Ipsilateral weakness Ipsilateral impaired fine touch, proprioception, vibration Contralateral impaired pain and temperature
52
When is the peak incidence of Bell's palsy?
20-40 years
53
What is the management of Bell's palsy?
Prednisolone | Artificial tears and eye taping at night (prevents conjunctivitis from lack of blinking)
54
How can you tell the difference between Bell's palsy and an upper motor neuron palsy?
The forehead is affected in Bell's palsy
55
What are the medications licensed for neuropathic pain?
Duloxetine Pregabalin Amitryptyline Gabapentin
56
What is mononeuritis multiplex?
Sequential involvement of non-contiguous nerve trunks | Acute loss of motor/sensory function of nerves
57
What is a contraindication of triptans?
Ischaemic heart disease or cerebrovascular disease
58
What is the treatment of chronic inflammatory demyelinating polyneuropathy?
Steroids
59
What are the triggers of migraine?
``` CHOCOLATE Chocolate Hangovers Orgasms Combined OCP Lie-ins Alcohol Travel Exercise ```
60
What are the risk factors for MS?
Vitamin D deficiency Previous infectious mononucleosis Genetics Smoking
61
Where is Broca's area found?
Inferior frontal gyrus
62
Where is Wernicke's area found?
Superior temporal gyrus
63
What type of dysphasia do you get with strokes affecting Wernicke's and Broca's areas?
Broca's - expressive | Wernicke's - receptive`
64
What is the treatment of a myasthenic crisis?
Plasmaphresis | IVIG
65
What are the two types of seizures?
Partial/focal | Generalized
66
What is the difference between a simple partial seizure and a complex partial seizure?
Simple: no loss of consciousness Compex: loss of consciousness
67
What is a secondary generalised seizure?
Partial seizures may spread after a few seconds due to failure of inhibitory mechanisms
68
What is a generalised seizure?
Simultaneous involvement of both hemispheres, always loss of consciousness/awareness
69
What are two possible characteristics of simple partial seizures?
Jacksonian march | Todd's paralysis
70
From where do complex partial seizures usually arise?
Temporal (60%) or frontal lobe
71
What are the characteristics of a complex partial seizure?
Aura Loss of awareness Post-ictal confusion
72
What are the types of generalised seizure?
``` Absence Tonic-clonic Myoclonic Tonic Atonic ```
73
What are the characteristics of absence seizures?
3Hz spike and wave activity on EEG Loss of awareness, vacant expression for 10 seconds No motor manifestations except eyelid flutter
74
What are the characteristics of tonic-clonic seizures?
``` Stiffening of the limbs, then synchronous jerking of limbs Eyes open Tongue bitten Incontinent of urine/faeces Post-ictal phase ```
75
What occurs in the post ictal phase?
Flaccid unresponsiveness Gradual return to awareness Confusion and drowsiness Lasts 15m - hour
76
What are the two main types of primary generalised epilepsy?
Childhood absence epilepsy | Juvenile myoclonic epilepsy
77
What is childhood absence epilepsy?
Begins 5-7 years | Absence seizures
78
What is juvenile absence epilepsy?
Begins in older children/teenagers Absence seizures Longer seizures
79
What is juvenile myoclonic epilepsy?
Begins in teenage years GTCS, myoclonic, absence Occur early in the morning
80
What are some triggers for seizures in JME?
Strobe lighting Alcohol Decreased sleep
81
What EEG finding is seen in JME?
Spike and wave activity
82
What drug is likely to worsen myoclonic seizures?
Lamotrigine
83
What is the treatment of myoclonic seizures?
Valproate or levetiracetam
84
What is the treatment of tonic/atonic seizures?
Sodium valproate
85
What is genetic anticipation?
Hereditary diseases have an earlier age of onset through generations Trinucleotide repeat disorders - Huntington's, myotonic dystrophy
86
What is the inheritance pattern in essential tremor?
Autosomal dominant
87
What are the characteristics of essential tremor?
Bilateral | Worsens when both arms are outstretched
88
What is the first line treatment of essential tremor?
Propanolol
89
What is cataplexy?
Sudden and transient weakness after strong emotions
90
What is associated with cataplexy?
Narcolepsy
91
What are three side effects of levodopa?
Postural hypotension On-off effect/dyskinesia Cardiac arrhythmias Psychosis
92
What common class of drug is associated with precipitating a myasthenic crisis?
Beta-blockers
93
What is ataxia caused by?
Lesions in the cerebellum: Gait ataxia without limb ataxia: cerebellar vermis General ataxia: cerebellum
94
What is limb ataxia?
Past pointing, finger-nose ataxia etc
95
What do lesions of the parietal lobe produce?
Sensory symptoms, dyslexia, dysgraphia
96
What are the symptoms of a vestibular schwannoma?
Unilateral deafness Tinnitus Absent corneal reflex
97
Give three causes of tremor, other than Parkinson's disease, and essential tremor
Carbon dioxide retention - flap when outstretched Thyrotoxicosis - thyroid signs Cerebellar - intention tremor Anxiety

Phase 3a (18 decks)

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