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Polymyositis With Facioscapulohumeral Distribution
Ted L. Rothstein, MD;
Coldeven B. Carlson, MD;
S. Mark Sumi, MD
Arch Neurol. 1971;25(4):313-319.
Abstract
An 11-year-old girl rapidly developed weakness limited to facial and shoulder muscles with associated myoedema. The legs were not involved. Electromyography demonstrated myopathic motor unit potentials and increased muscle irritability. Serum enzyme levels were markedly elevated and returned towards normal with steroid therapy although clinical improvement was slight. Two muscle biopsies showed inflammatory reaction and necrosis. The short clinical course, absence of affected family members, and laboratory findings were consistent with a restricted polymyositis. Restricted muscle involvement simulating facio-S scapulohumeral dystrophy is an unusual variation of polymyositis.
Author Affiliations
Seattle
From the departments of pediatrics and medicine (neurology) and pathology (neuropathology) (Dr. Sumi), University of Washington School of Medicine, and Children's Orthopedic Hospital and Medical Center, Seattle.
Footnotes
Accepted for publication March 29, 1971.
Read before the American Academy of Neurology, Bal Harbour, Fla, May 1, 1970.
Reprint requests to Children's Orthopedic Hospital and Medical Center, 4800 Sand Point Way NE, Seattle 98105 (Dr. Carlson).
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