Congenital inflammatory myopathy: a demonstrative case and proposed diagnostic classification.
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.
McNeil
Stephen M
SM
Department of Physical Medicine and Rehabilitation, McMaster University, Hamilton, Ontario, Canada.
Woulfe
John
J
Ross
Catherine
C
Tarnopolsky
Mark A
MA
D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D004576:Electromyography; D017809:Fatal Outcome; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D018482:Muscle, Skeletal / Q000473:pathology / Q000503:physiopathology; D009220:Myositis / Q000151:congenital* / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology; D011241:Prednisone / Q000627:therapeutic use
D000893:Anti-Inflammatory Agents; D000324:Adrenocorticotropic Hormone; D011241:Prednisone