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Y3 Neurology > Neuromuscular Disorders > Flashcards

Neuromuscular Disorders Flashcards

1
Q

what is MRC muscle power grade 1

A

no movement at all

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2
Q

what is MRC muscle power grade 2

A

flicker of movement when attempting to contract muscle

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3
Q

what is MRC muscle power grade 3

A

some muscle movement if gravity removed by none against gravity

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4
Q

what is MRC muscle power grade 4

A

movement against resistance but not full strength

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5
Q

what is MRC muscle power grade 5

A

normal strength

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6
Q

what is the disease mechanism of lambert eaton synd LEMS?

A

autoimmune antibody to presynaptic voltage gated Ca channels

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7
Q

what condition is lambert eaton syndrome LEMS strongly associated with

A

small cell carcinoma (almost always lung)

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8
Q

what is the presentation of lambert eaton syndrome LEMS

A

proximal limb weakness

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9
Q

what is the treatment for lambert eaton synd LEMS

A

3-4 diaminopyridine

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10
Q

what are the peaks of myasthenia gravis onset in terms of age and gender

A

25yr female

80yr male

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11
Q

what is the general disease mechanism of myasthenia gravis

A

autoimmune antibody to acetylcholine receptors

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12
Q

what condition is strongly associated with myasthenia gravis

A

thymoma or thymus hyperplasia

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13
Q

what autoantibodies are associated with myasthenia gravis

A

MUSK, ACh

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14
Q

what is the general early presentation of myasthenia gravis

A

extraocular weakness, facial weakness, ptosis THAT IS WORSE THROUGHOUT THE DAY

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15
Q

what are late onset features of myasthenia gravis

A

proximal limb weakness, respiratory muscle weakness

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16
Q

investigations for myasthenia gravis

A

autoantibodies, EMG +- ice test for ptosis

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17
Q

what is the acute management of myasthenia gravis

A

acetylcholinesterase inhibitor pyridostigmine, IV immunoglobulin

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18
Q

what is the long term management of myasthenia gravis

A

high dose CCS or steroid sparing azathioprine/ mycophenolate.

+- thymectomy!

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19
Q

what drug should be avoided in patients with myasthenia gravis?

A

genta

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20
Q

tendon reflexes are normal in myasthenia gravis. true or false

A

true

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21
Q

presentation of botulism

A

rapid onset weakness, no sensory loss

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22
Q

botulism is cause by a clostridium toxin and is associated with alcoholism. T or F

A

F. botulism is cause by a clostridium toxin and is associated with PWIDs

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23
Q

what is a fasciculation

A

fast spontaneous muscle twitch

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24
Q

what are fasciculations associated with

A

UMN disease, caffeine, fatigue

25
what is myotonia
failure to relax muscle after voluntary use
26
intrinsic muscle disease causes proximal or distal muscle wasting?
proximal
27
does muscular dystrophy cause sensory loss?
no
28
is muscular dystrophy inflammatory?
no
29
what condition can cause muscle weakness, cataracts, frontal balding, heart defects and ophthalmoplegia?
myotonic dystrophy
30
polymyositis is an inflammatory condition causing acute asymmetric proximal muscle weakness. T or F
F. | polymyositis is an inflammatory condition causing symmetric proximal muscle weakness over weeks-months
31
blood abnormality in polymyositis?
raised CK
32
management of polymyositis
CCS
33
how does the presentation of polymyositis and dermatomyositis differ?
dermatomyositis same but + heliotropic facial rash
34
what condition is dermatomyositis assoc. with?
underlying malignancy (in 50% PTx)
35
inclusion body myositis is an inflammatory disorder that usually presents in young patients with progressive muscle weakness. T or F
F. inclusion body myositis is NOT inflammatory! usually presents in PTx over 60yr with progressive muscle weakness
36
what drug has a necrotising effect on muscle, causing myalgia
statins
37
what conditions arises in muscle injury due to muscle dissolution
rhabdomyolysis
38
an old lady lying on the floor for days after a fall could develop rhabdomyolysis due to what complication
DIC
39
what conditions is rhabdomyolysis assoc. with?
DIC, AKI, post-ictal, crush injuries | also marathon runners
40
what is the classic triad of rhabdomyolysis?
myalgia + weakness + myoglobulinuria
41
are tendon reflexes impaired in disorders of the NMJ
no
42
what is the peak age range and gender of MND onset
M>F | 50-75yr
43
is MND familial?
only in 10% of cases. 90% sporadic
44
does MND cause UMN or LMN signs?
either
45
name 4 types of MND
amyotrophic lateral sclerosis bulbar primary lateral sclerosis frontotemporal
46
what is the commonest type of MND
ALS
47
what type of MND does not cause weakness
frontotemporal
48
what type of MND is more common in women aged 60-80yr? It causes problems with speech, chewing and swallowing
bulbar
49
what type of MND only effects UMN? it causes problems with distal spinal muscles
PLS primary lateral sclerosis
50
does MND cause sensory signs?
no
51
is ALS more prominent in the upper or lower limbs?
upper
52
does ALS affect cognition
yes, can cause a mild fronto-temporal dementia
53
investigations for MND
``` EMG MRI (exclude structural cause) ```
54
how might ALS initially present?
weak grip, foot drop, stumbling gait | OE: upgoing plantar, brisk reflexes
55
what is the definitive test for MND
there isn't one
56
according to the diagnostic criteria, what features diagnose ALS
LMN signs + UMN signs + progressive spread + no evidence other disease
57
what is the mean prognosis of MND
3 years
58
what drug prolongs life expectancy in MND
riluzole | only by 3 months though

Y3 Neurology (23 decks)

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