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Congenital inflammatory myopathy: a demonstrative case and proposed diagnostic classification.

Authors
  • McNeil, Stephen M
  • Woulfe, John
  • Ross, Catherine
  • Tarnopolsky, Mark A
Type
Published Article
Journal
Muscle & nerve
Publication Date
Feb 01, 2002
Volume
25
Issue
2
Pages
259–264
Identifiers
PMID: 11870695
Source
Medline
License
Unknown

Abstract

There have been few reports of congenital inflammatory myopathy in the literature, and most of these have been associated with congenital muscular dystrophy. We review the literature and present a case with electromyographic and muscle biopsy evidence of congenital inflammatory myopathy with onset in the perinatal period and no evidence of a congenital muscular dystrophy. There was evidence of subjective improvement following corticosteroid administration (approximately 1 mg/kg per day) with a concomitant normalization of the serum creatine kinase activity. Of particular interest in the case was the history of maternal infection, suggesting a possible postinfectious molecular mimicry as the etiology of the muscle inflammation. This case suggests that a rare form of congenital inflammatory myopathy does exist that is not associated with a congenital muscular dystrophy. A preliminary classification scheme is proposed to separate true congenital cases from those acquired after birth and those cases associated with congenital dystrophy.

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