myopathy Flashcards

1
Q

at what age can you start recognizing Duchenne Muscular dystrophy vs Becker muscular dystrophy?

A

3-5 yrs old for duchenne; becker: 5-15

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2
Q

what is the main sign of Duchenne?

A

gower sign: patient has to get up using their hands to climb himself up from floor

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3
Q

what is the diff between duchenne myopathy vs becker myopathy with dystrophin?
what does absent of dystrophin indicate in dx

A

Duchenne Myopathy: Absent of dystrophin: dystrophin deficiency will cause muscle protein complex to fall apart

Becker: some dystrophin produced

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4
Q

what is dystrophin

A

large protein part of large complex of muscle protein and stabilizes the sarcolemma

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5
Q

which muscle atrophy requires wheel chair by the time they are 12 vs which is still allowed for walking?

A

becker: walk beyond 15 yrs; duchenne requires wheel chain by 12

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6
Q

what types of muscles are effected most for Dermatomyositis and polymyositis, And Becker

A

proximal muscles

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7
Q

what is the classic symptoms for dermatomyositis

A

rash: heliotrope, Gottron, shawl sign, V sign (v sign rash on the back), mechanic hands

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8
Q

what are grotton papules

A

rashes that sit over the central aspect of fingers in dermatomyositis

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9
Q

which myopathy is increased association with malignancy/cancer? what is the most important thing to do with this myopathy?

A

Dermatomyositis IMPORTANT to GET ALL SCREENING EXAMS FOR CANCER ***

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10
Q

what is a unique histology to inclusion body myositis?

A

Rimmed vacuoles

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11
Q

what type of exams should a male with dermatomyositis get specifically?

A

breast exam, pelvic exam, and prostate exam for male to screen for cancer

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12
Q

what are some physical features of Duchenne?

A

present at birth, apparent 3-5 yrs, freuqnet falls and can’t keep up with friends

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13
Q

Statin and Fibrates adverse side effects? what does elevated CK indicate?

A

myalgia, malaise, muscle tenderness due to exercise
-muscle necrosis leading to severe rxn have rhabdomyolysis and myoglobinuria

  • elvated CK is indicateion of toxicity
  • STOP THE Drug and muscle can improve, and to decrease in CK
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14
Q

what is the definitive test for dermatomyositis? what is the key finding?

A

muscle biopsy: inflammation (key)(inlammatory cells lymphocytes)

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15
Q

Laboratory finding for duchenne

A

creatine kinase levels elevated 20-100x more due to muscle loss

EMG: inflammation/myopathy

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16
Q

what is the definitive dx for dychenne

A

dystrophin deficiency

17
Q

what are the inflammatory myopathies

A

dermatomyositis and polymyositis