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Musculoskeletal system > Neuromuscular junction disease, Raynaud, scleroderma > Flashcards

Neuromuscular junction disease, Raynaud, scleroderma Flashcards

1
Q

Myasthenia Gravis: frequency?

A

most common NMJ disorder

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2
Q

Myasthenia Gravis: pathphysiology?

A

antibodies to postsynaptic ACh receptor

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3
Q

Myasthenia Gravis: symptoms?

A

fatigable muscle weakness (proximal weakness, ptosis, diplopia, dyspnea, dysphagia)

spared reflexes
worsens with muscle use

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4
Q

Myasthenia Gravis: symptoms eye?

A

ptosis, diplopia

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5
Q

Myasthenia Gravis: symptom respiratory?

A

dyspnea

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6
Q

Myasthenia Gravis: reflexes?

A

normal

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7
Q

Myasthenia Gravis: worsens or improves with muscle use?

A

worsens

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8
Q

Myasthenia Gravis: what kind of muscle weaknes?

A

fatigable

proximal muscles
ptosis
diplopia
dyspnea
dysphagia, difficulty chewing

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9
Q

Myasthenia Gravis: bulbar muscle involvement leads to .. ?

A

dysphagia, difficulty chewing

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10
Q

what is dysphagia?

A

difficulty swallowing

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11
Q

Myasthenia Gravis: respiratory muscle involvement leads to .. ?

A

dyspnea

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12
Q

most common NMJ disorder

A

myasthenia gravis

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13
Q

autoantibodies against postsynaptic ACh receptor

A

myasthenia gravis

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14
Q

proximal muscle weakness with ptosis and diplopia, get worse with use. reflexes normal

what is this sugggestive oft?

A

Myasthenia Gravis

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15
Q

Myasthenia Gravis: associated with?

A

thymoma, thymic hyperplasia

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16
Q

which NMJ disease is associated with thymoma?

A

Myasthenia Gravis

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17
Q

which NMJ is associated with thymic hyperplasia?

A

Myasthenia Gravis

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18
Q

Myasthenia Gravis: AChE inhibitor administrator effect?

A

reverses symtomps

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19
Q

Myasthenia Gravis: treatment?

A

pyridostigmine

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20
Q

Pyridostigmine: treatment for what?

A

Myasthenia Gravis

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21
Q

AChE inhibitor is short for what?

A

achetylcholine esterase inhibitor

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22
Q

Lambert-Eaton myasthenic syndrome: frequency?

A

uncommon

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23
Q

Lambert-Eaton myasthenic syndrome: pathophysiology?

A

autoantibodies to presynaptic Ca2+ channels -> less ACh release

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24
Q

Lambert-Eaton myasthenic syndrome: symptoms?

A

proximal weakness
autonomic symptoms (dry mouth, constipation, impotence)
hypoflexia

improves with muscle use

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25
Q

Lambert-Eaton myasthenic syndrome: associated with?

A

small cell lung cancer

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26
Q

whatLambert-Eaton myasthenic syndrome: reflexes?

A

lower

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27
Q

Lambert-Eaton myasthenic syndrome: autonomic symptoms?

A

dry mouth, constipation, impotence

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28
Q

Lambert-Eaton myasthenic syndrome: weakness in which muscles?

A

proximal

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29
Q

Lambert-Eaton myasthenic syndrome: how do symptoms improve?

A

with muscle use

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30
Q

autoantibodies against presynaptic Ca2+ channels, effect?

A

Less ACh release

(Lambert-Eaton myasthenic syndrome)

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30
Q

proximal muscle weakness, dry mouth, hyporeflexes

improves with muscle use

suggestive of?

A

Lambert-Eaton myasthenic syndrome

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30
Q

which NMJ is associated with small cell lung cancer?

A

Lambert-Eaton myasthenic syndrome

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30
Q

which NMJ is uncommon?

A

Lambert-Eaton myasthenic syndrome

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31
Q

autoantibodies against presynaptic Ca2+ channels, less Ach release

which disease?

A

Lambert-Eaton myasthenic syndrome

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32
Q

which NMJ disorder has lowered reflexes?

A

Lambert-Eaton myasthenic syndrome

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33
Q

which NMJ disorder has normal reflexes?

A

Lambert-Eaton myasthenic syndrome

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34
Q

Lambert-Eaton myasthenic syndrome: AChE inhibitor effect?

A

minimal

35
Q

AChE inhibitor minimal effect in which disease?

A

Lambert-Eaton myasthenic syndrome

36
Q

Raynaud: mechanism

A

cold or stress -> arteriolar (small vessel) vasospasmen -> color change (white to blue to red)

37
Q

what can cause symptoms of Raynaud disease?

A

cold or stress

38
Q

what causes the color changes in Raynaud?

A

white - ischemia
blue - hypoxia
red - reperfusion

39
Q

what causes white color in raynaud?

A

ischemia

40
Q

what causes blue color in raynaud?

A

hypoxia

41
Q

what causes red color in raynaud?

A

reperfusion

42
Q

what are the color changes and order in raynaud?

A

white -> blue -> red

43
Q

what happens to the blood vessels in raynaud?

A

vasospasm due to cold/stress

44
Q

in which body part raynaud phenomenon?

A

fingers and toes

45
Q

when is raynaud called ‘raynaud disease’?

A

when it’s primary/idiopathic

46
Q

when is raynaud called ‘raynaud phenomenon’?

A

2nd to a disease process

47
Q

raynaud can be secondary to which disease?

A

SLE
Systemic sclerosis (CREST)
mixed connective tissue disease

48
Q

digital ulceration seen in which forms of raynaud?

A

secondary (SLE, SS, MCTD)

49
Q

what causes digital ulceration in raynaud

A

critical ischemia

50
Q

raynaud: treatment?

A

calcium channel blockers (promotes vasodilatation)

51
Q

what are calcium channel blockers?

A

antihypertensive drugs which promote vasodilatation

52
Q

scleroderma: other name?

A

systemic sclerosis

53
Q

systemic sclerosis: other name?

A

scleroderma

54
Q

systemic sclerosis: triad?

A

autoimmunity
vasculopathy
collagen deposition with fibrosis

55
Q

triad of:
autoimmunity
vasculopathy
collagen deposition with fibrosis

what is this?

A

systemic sclerosis

56
Q

systemic sclerosis: what happens to the skin?

A

collagen deposition with fibrosis

57
Q

systemic sclerosis: how does the skin look?

A

puffy, taut (stretched tight) skin without wrinkles

fingertip pitting

58
Q

systemic sclerosis: what happens to fingertips?

A

fingertip pitting

59
Q

puffy, taut (stretched tight) skin without wrinkles. fingertip pitting

suggestive of?

A

systemic sclerosis

60
Q

systemic sclerosis: can involve which systems? (besides skin)

A

renal
pulmonary
GI
cardiovascular

61
Q

systemic sclerosis: what can happens to renal system?

A

scleroderma renal crisis

62
Q

how to treat scleroderma renal crisis?

A

ACE inhibitors

63
Q

systemic sclerosis: possible pulmonary pathology?

A

interstitial fibrosis, pulmonary hypertension

64
Q

systemic sclerosis: possible GI symptoms?

A

less peristalsis and LES tone -> dysphagia and heart burn

65
Q

what is the percentage of females with systemic sclerosis?

A

75%

66
Q

what are the 2 major types of systemic sclerosis?

A

diffuse and limited (CREST)

67
Q

Diffuse scleroderma: skin involvement?

A

widespread

68
Q

diffuse scleroderma: how fast if progression?

A

rapid

69
Q

diffuse scleroderma: visceral involvement early or late?

A

early

70
Q

diffuse scleroderma: characteristics?

A

widespread skin involvement, rapid progression, early visceral involvement

(in sum: widespread skin, fast and organs also)

71
Q

diffuse scleroderma: antibodies?

A

anti-Scl-70 antibody
anti-RNA polymerase III

72
Q

anti-Scl-70 antibody
anti-RNA polymerase III

associated with what?

A

SS
diffuse scleroderma type

73
Q

what is another name for anti-Scl-70 antibody?

A

anti-DNA topoisomerase-I antibody

74
Q

anti-DNA topoisomerase-I antibody

another name?

A

anti-Scl-70 antibody

75
Q

widespread skin involvement, rapid progression, early visceral involvement

characteristics of which type of SS?

A

diffuse

76
Q

limited scleroderma: skin involvement?

A

limited to face and fingers

77
Q

limited scleroderma: also with which syndrome?

A

CREST

78
Q

what is the CREST syndrome?

A

Calcinosis cutis, anti-centromere antibody
Raynaud
Esophageal dysmotility
Sclerodactylyl
Telangiectasia

79
Q

what is the C in CREST syndrome?

A

calcinosis cutis
anti-centromere antibody

80
Q

what is the R in CREST syndrome?

A

raynaud

81
Q

what is the E in CREST syndrome?

A

esophageal dysmotility

82
Q

what is the S in CREST syndrome?

A

sclerodactyly

83
Q

what is the T in CREST syndrome?

A

telangiectasia

84
Q

Sclerodactyly, what is this?

A

a hardening of the skin of the hand

fingers curl inward (claw-like shape)

85
Q

what are telangiectasia?

A

small, widened blood vessels on the skin

86
Q

limited scleroderma: clinical course benigne or malignant?

A

benigne

87
Q

SS: skin involvement only face and fingers. which type?

A

limited scleroderma

88
Q

CREST syndrome associated with which type of SS?

A

limited scleroderma

Musculoskeletal system (17 decks)

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