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
Q

what is myotonia

A

failure to relax muscle after voluntary use

26
Q

intrinsic muscle disease causes proximal or distal muscle wasting?

A

proximal

27
Q

does muscular dystrophy cause sensory loss?

A

no

28
Q

is muscular dystrophy inflammatory?

A

no

29
Q

what condition can cause muscle weakness, cataracts, frontal balding, heart defects and ophthalmoplegia?

A

myotonic dystrophy

30
Q

polymyositis is an inflammatory condition causing acute asymmetric proximal muscle weakness. T or F

A

F.

polymyositis is an inflammatory condition causing symmetric proximal muscle weakness over weeks-months

31
Q

blood abnormality in polymyositis?

A

raised CK

32
Q

management of polymyositis

A

CCS

33
Q

how does the presentation of polymyositis and dermatomyositis differ?

A

dermatomyositis same but + heliotropic facial rash

34
Q

what condition is dermatomyositis assoc. with?

A

underlying malignancy (in 50% PTx)

35
Q

inclusion body myositis is an inflammatory disorder that usually presents in young patients with progressive muscle weakness. T or F

A

F. inclusion body myositis is NOT inflammatory! usually presents in PTx over 60yr with progressive muscle weakness

36
Q

what drug has a necrotising effect on muscle, causing myalgia

A

statins

37
Q

what conditions arises in muscle injury due to muscle dissolution

A

rhabdomyolysis

38
Q

an old lady lying on the floor for days after a fall could develop rhabdomyolysis due to what complication

A

DIC

39
Q

what conditions is rhabdomyolysis assoc. with?

A

DIC, AKI, post-ictal, crush injuries

also marathon runners

40
Q

what is the classic triad of rhabdomyolysis?

A

myalgia +
weakness +
myoglobulinuria

41
Q

are tendon reflexes impaired in disorders of the NMJ

A

no

42
Q

what is the peak age range and gender of MND onset

A

M>F

50-75yr

43
Q

is MND familial?

A

only in 10% of cases. 90% sporadic

44
Q

does MND cause UMN or LMN signs?

A

either

45
Q

name 4 types of MND

A

amyotrophic lateral sclerosis
bulbar
primary lateral sclerosis
frontotemporal

46
Q

what is the commonest type of MND

A

ALS

47
Q

what type of MND does not cause weakness

A

frontotemporal

48
Q

what type of MND is more common in women aged 60-80yr? It causes problems with speech, chewing and swallowing

A

bulbar

49
Q

what type of MND only effects UMN? it causes problems with distal spinal muscles

A

PLS primary lateral sclerosis

50
Q

does MND cause sensory signs?

A

no

51
Q

is ALS more prominent in the upper or lower limbs?

A

upper

52
Q

does ALS affect cognition

A

yes, can cause a mild fronto-temporal dementia

53
Q

investigations for MND

A
EMG
MRI (exclude structural cause)
54
Q

how might ALS initially present?

A

weak grip, foot drop, stumbling gait

OE: upgoing plantar, brisk reflexes

55
Q

what is the definitive test for MND

A

there isn’t one

56
Q

according to the diagnostic criteria, what features diagnose ALS

A

LMN signs + UMN signs + progressive spread + no evidence other disease

57
Q

what is the mean prognosis of MND

A

3 years

58
Q

what drug prolongs life expectancy in MND

A

riluzole

only by 3 months though