Neuromuscular junction disease, Raynaud, scleroderma Flashcards

(91 cards)

1
Q

Myasthenia Gravis: frequency?

A

most common NMJ disorder

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Myasthenia Gravis: pathphysiology?

A

antibodies to postsynaptic ACh receptor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Myasthenia Gravis: symptoms?

A

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

spared reflexes
worsens with muscle use

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Myasthenia Gravis: symptoms eye?

A

ptosis, diplopia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Myasthenia Gravis: symptom respiratory?

A

dyspnea

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Myasthenia Gravis: reflexes?

A

normal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Myasthenia Gravis: worsens or improves with muscle use?

A

worsens

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Myasthenia Gravis: what kind of muscle weaknes?

A

fatigable

proximal muscles
ptosis
diplopia
dyspnea
dysphagia, difficulty chewing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Myasthenia Gravis: bulbar muscle involvement leads to .. ?

A

dysphagia, difficulty chewing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what is dysphagia?

A

difficulty swallowing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Myasthenia Gravis: respiratory muscle involvement leads to .. ?

A

dyspnea

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

most common NMJ disorder

A

myasthenia gravis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

autoantibodies against postsynaptic ACh receptor

A

myasthenia gravis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

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

what is this sugggestive oft?

A

Myasthenia Gravis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Myasthenia Gravis: associated with?

A

thymoma, thymic hyperplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

which NMJ disease is associated with thymoma?

A

Myasthenia Gravis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

which NMJ is associated with thymic hyperplasia?

A

Myasthenia Gravis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Myasthenia Gravis: AChE inhibitor administrator effect?

A

reverses symtomps

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Myasthenia Gravis: treatment?

A

pyridostigmine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Pyridostigmine: treatment for what?

A

Myasthenia Gravis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

AChE inhibitor is short for what?

A

achetylcholine esterase inhibitor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Lambert-Eaton myasthenic syndrome: frequency?

A

uncommon

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Lambert-Eaton myasthenic syndrome: pathophysiology?

A

autoantibodies to presynaptic Ca2+ channels -> less ACh release

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Lambert-Eaton myasthenic syndrome: symptoms?

A

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

improves with muscle use

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Lambert-Eaton myasthenic syndrome: associated with?
small cell lung cancer
26
whatLambert-Eaton myasthenic syndrome: reflexes?
lower
27
Lambert-Eaton myasthenic syndrome: autonomic symptoms?
dry mouth, constipation, impotence
28
Lambert-Eaton myasthenic syndrome: weakness in which muscles?
proximal
29
Lambert-Eaton myasthenic syndrome: how do symptoms improve?
with muscle use
30
autoantibodies against presynaptic Ca2+ channels, effect?
Less ACh release (Lambert-Eaton myasthenic syndrome)
30
proximal muscle weakness, dry mouth, hyporeflexes improves with muscle use suggestive of?
Lambert-Eaton myasthenic syndrome
30
which NMJ is associated with small cell lung cancer?
Lambert-Eaton myasthenic syndrome
30
which NMJ is uncommon?
Lambert-Eaton myasthenic syndrome
31
autoantibodies against presynaptic Ca2+ channels, less Ach release which disease?
Lambert-Eaton myasthenic syndrome
32
which NMJ disorder has lowered reflexes?
Lambert-Eaton myasthenic syndrome
33
which NMJ disorder has normal reflexes?
Lambert-Eaton myasthenic syndrome
34
Lambert-Eaton myasthenic syndrome: AChE inhibitor effect?
minimal
35
AChE inhibitor minimal effect in which disease?
Lambert-Eaton myasthenic syndrome
36
Raynaud: mechanism
cold or stress -> arteriolar (small vessel) vasospasmen -> color change (white to blue to red)
37
what can cause symptoms of Raynaud disease?
cold or stress
38
what causes the color changes in Raynaud?
white - ischemia blue - hypoxia red - reperfusion
39
what causes white color in raynaud?
ischemia
40
what causes blue color in raynaud?
hypoxia
41
what causes red color in raynaud?
reperfusion
42
what are the color changes and order in raynaud?
white -> blue -> red
43
what happens to the blood vessels in raynaud?
vasospasm due to cold/stress
44
in which body part raynaud phenomenon?
fingers and toes
45
when is raynaud called 'raynaud disease'?
when it's primary/idiopathic
46
when is raynaud called 'raynaud phenomenon'?
2nd to a disease process
47
raynaud can be secondary to which disease?
SLE Systemic sclerosis (CREST) mixed connective tissue disease
48
digital ulceration seen in which forms of raynaud?
secondary (SLE, SS, MCTD)
49
what causes digital ulceration in raynaud
critical ischemia
50
raynaud: treatment?
calcium channel blockers (promotes vasodilatation)
51
what are calcium channel blockers?
antihypertensive drugs which promote vasodilatation
52
scleroderma: other name?
systemic sclerosis
53
systemic sclerosis: other name?
scleroderma
54
systemic sclerosis: triad?
autoimmunity vasculopathy collagen deposition with fibrosis
55
triad of: autoimmunity vasculopathy collagen deposition with fibrosis what is this?
systemic sclerosis
56
systemic sclerosis: what happens to the skin?
collagen deposition with fibrosis
57
systemic sclerosis: how does the skin look?
puffy, taut (stretched tight) skin without wrinkles fingertip pitting
58
systemic sclerosis: what happens to fingertips?
fingertip pitting
59
puffy, taut (stretched tight) skin without wrinkles. fingertip pitting suggestive of?
systemic sclerosis
60
systemic sclerosis: can involve which systems? (besides skin)
renal pulmonary GI cardiovascular
61
systemic sclerosis: what can happens to renal system?
scleroderma renal crisis
62
how to treat scleroderma renal crisis?
ACE inhibitors
63
systemic sclerosis: possible pulmonary pathology?
interstitial fibrosis, pulmonary hypertension
64
systemic sclerosis: possible GI symptoms?
less peristalsis and LES tone -> dysphagia and heart burn
65
what is the percentage of females with systemic sclerosis?
75%
66
what are the 2 major types of systemic sclerosis?
diffuse and limited (CREST)
67
Diffuse scleroderma: skin involvement?
widespread
68
diffuse scleroderma: how fast if progression?
rapid
69
diffuse scleroderma: visceral involvement early or late?
early
70
diffuse scleroderma: characteristics?
widespread skin involvement, rapid progression, early visceral involvement (in sum: widespread skin, fast and organs also)
71
diffuse scleroderma: antibodies?
anti-Scl-70 antibody anti-RNA polymerase III
72
anti-Scl-70 antibody anti-RNA polymerase III associated with what?
SS diffuse scleroderma type
73
what is another name for anti-Scl-70 antibody?
anti-DNA topoisomerase-I antibody
74
anti-DNA topoisomerase-I antibody another name?
anti-Scl-70 antibody
75
widespread skin involvement, rapid progression, early visceral involvement characteristics of which type of SS?
diffuse
76
limited scleroderma: skin involvement?
limited to face and fingers
77
limited scleroderma: also with which syndrome?
CREST
78
what is the CREST syndrome?
Calcinosis cutis, anti-centromere antibody Raynaud Esophageal dysmotility Sclerodactylyl Telangiectasia
79
what is the C in CREST syndrome?
calcinosis cutis anti-centromere antibody
80
what is the R in CREST syndrome?
raynaud
81
what is the E in CREST syndrome?
esophageal dysmotility
82
what is the S in CREST syndrome?
sclerodactyly
83
what is the T in CREST syndrome?
telangiectasia
84
Sclerodactyly, what is this?
a hardening of the skin of the hand fingers curl inward (claw-like shape)
85
what are telangiectasia?
small, widened blood vessels on the skin
86
limited scleroderma: clinical course benigne or malignant?
benigne
87
SS: skin involvement only face and fingers. which type?
limited scleroderma
88
CREST syndrome associated with which type of SS?
limited scleroderma