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Skeletal Muscle and Neuromuscular Junction Disorders Clinical Features

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fuckdaveliu's version from 2018-05-19 09:38

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DermatomyositisBilateral proximal muscle weakness, distal involvement can develop late in disease. Rash of the upper eyelids(heliotrope rahs); malar rash may also be seen. Red papules on the elbows, knuckles, and knees(Gottron papules). Laboratory findings include increased creatine kinase. Positive ANA and anti-Jo-1 antibody. Perimysial inflammation(CD4+T cells) with perifascicular atrophy on biopsy
PolymyositisResembles dermatomyositis clinically, but skin is not involved; endomysial inflammation (CD8+ T cells) with necrotic muscle fibers is seen on biopsy.
Duchenne Muscular Dystrophy Proximal muscle weakness at 1 year of age; progresses to involve distal msucles. Calf Pseudohypertrophy is a characteristic finding. Serum creatine kinase is elevated. Death results from cardiac or respiratory failure, myocardium is commonly involved
Becker Muscular dystrophy Milder disease
Myasthenia Gravis Muscle weakness that worsens with use and improves with rest; classically involves the eyes, leading to ptosis and diplopia. Symptoms improve with anticholinesterase agents. Associated with thymic hyperplasia or thymoma; thymectomy improves symptoms
Lamber-Eaton SyndromProximal muscle weakness that improves with use; eyes are usually spared. Anticholinesterase agents do not improve symptoms. Resolves with resection of the cancer
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