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20 Cards in this Set

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  • Back
What are signs of Duchenne MD?
proximal>distal muscle weakness presenting in early childhood; have Gower's sign, Trendellenberg's sign, Lordosis, tip toe walking, enlarged calves. Reflexes absent everywhere but ankle, CK around 100,000
A young patient comes with extremely high CK levels and absent reflexes in upper extremitites. His muscle biopsy stain was negative for dystrophin. What would you give this patient to tx disorder and how is it inherited?
Give prednisone, have do PT with heal cord streches, bracing w/ knee-foot orthroses, + pressure vent. at night, genetic counseling to parents as it is X-linked inher.
What are clinical hallmarks, lab findings, and associated features of Becker MD?
Like Duchenne but variable onset with muscle cramping/myalgias w/ exercise, myoglobinuria, hyper-CK-emia, reduced dystrophin, dilated cardiomyopathy
Would you use prednisone for Duchenne MD? Becker MD?
Yes for Duchenne, No for Becker
What is myotonia? dystrophy?
sustained muscle contraxn in response to stimuli. dystrophy is loss of skeletle muscle with fibrosis and fatty filtration
What are the clinical signs of each of mild, classical, and congenital myotonic dystrophy?
Mild: cataracts, myotonia, normal life span. Classical: muscle weakness, atrophy, cataracts, condxn abnormalities, short life span?. Congenital: hypotonia, severe weakness at birth, resp, insufficiency, mental retardation, early death
What are the usual early signs of mytonic dystrophy?
Distal weakness in hands (grip) and feet (footdrop)
What isthe cause of myotonic distrophy and what are some associated features in the heart, eye, sm. muscle, endocrine and CNS?
Trinucleotide repeat, the longer the earlier the onset. Get cardio fibrosis leading to block, cataracts, insulin insensitivity-- so resistant to hypoglycemia, bowel hypomotility w/ megacolon, hypersolmnolent
If a patient comes to you unable to whistle or such through a staw, cannot raise their arms above their head or close their eyes completely what other things should you look for?
Winged scapula, hypodeveloped biceps, possible hearing loss and retinopathy. Chest muscles may be poorly developed
How do you diffinitively diagnose fascio-scapulo-humoral dystrophy?
DNA analysis for linkage to chromo 4q, Southern Blot analysis show 4q deletion
What are the clinical landmarks and associated features of plymyositis?
weakness and myalgias following a myopathic pattern (proximal>>distal), steadily progressive and can show myocarditis w? conductioin defects and CHF as well as interstitial lung dx
How would you be able to definitively diagnose polymyositis? Then how would you treat it?
Elevated CK (50X), muscle biopsy w? inflammatory cell inflitrates and muscle fiber necrosis. Also elevated liver enzymes. Tx with prednisone followed by steroid-sparing agents
If a patient has dermatomyositis, what clinical features would you see?
Blue-purple rash for on upper eyelids, heliotrope rash on face and upper trunk, Gottron rash- erythema of knuckles (SPARES phalanges), muscle weakness. possible CANCER
What are some laboratory findings of dermatomyotitis?
EMG with myopathic potentials, elevated CK, muscle biopsy showing perivascular inflammation and perivascular atrophy
What are some associated features of dermatomyotitis?
mechanic fingernails, cardio condxn defects, interstitial lung dx, subcutaneous calcifications, ulcerations in GI, dysphagia, fever, wt loss, Raynaud's phenomenon
What is the number one thing a physician should be worried about in a patient with dermatomyotitis?
Cancer, especially ovarian cancer
What is a good way to differentiate whether a patient who comes in with muscle weakness has polymyotitis or Inclusion body myotitis?
Inclusion body myotitis will have facial muscle weakness sparing the extraocular muscles.
How do you diagnose inclusion body myositis? tx?
Light micro findings are rimmed vacuoles, endomysial inflammation, swollen nuclei, hypertrophic large fibers. No real tx, cannot tx w/ steroid
How do you distinguish dermatomyositis from lupus both clinically and histologically?
Heliotrope (not malar) rash and Gottron rash- erythema on the knuckles. Get mechanic nails. Histo- you see perivascular inflammation and perifascular atrophy
What can polymyositis occur form?
Viral infxns such as HIV, Lyme disease and in conjxn w/ Chron's, sarcoidosis, Sjorgrens, biliary cirrhosis, ankylosing spondylitis, lupus, RA