title: Congenital inflammatory myopathy: a demonstrative case and proposed diagnostic classification.
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.
authors.affiliation: Department of Physical Medicine and Rehabilitation, McMaster University, Hamilton, Ontario, Canada.
authors.forename: Stephen M
authors.initials: SM
authors.lastname: McNeil
authors.forename: John
authors.initials: J
authors.lastname: Woulfe
authors.forename: Catherine
authors.initials: C
authors.lastname: Ross
authors.forename: Mark A
authors.initials: MA
authors.lastname: Tarnopolsky
mesh_terms: 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
chemical_list: D000893:Anti-Inflammatory Agents; D000324:Adrenocorticotropic Hormone; D011241:Prednisone