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NEUROLOGY 1990;40:1111
© 1990 American Academy of Neurology

Congenital inflammatory myopathy

M. Shevell, MD, CM, B. Rosenblatt, MD, CM, K. Silver, MD, S. Carpenter, MD and G. Karpati, MD

Department of Neurology (Drs. Shevell, Rosenblatt, and Silver), Montreal Children's Hospital; the Departments of Pathology (Dr. Carpenter) and Neurology (Dr. Karpati), Montreal Neurological Institute, McGill University, Montreal, PQ, Canada.

We present 3 patients with congenital inflammatory myopathy and summarize the literature. CNS involvement (microcephaly/intellectual delay) may or may not be present. Serum creatine kinase activity is elevated, the EMG is myopathic, and the muscle biopsy reveals inflammatory infiltrates, muscle fiber damage, and class I major histocompatibility complex products in muscle sarcolemma. Possible etiologies include intrauterine viral infection or an autoimmune process. Treatment with steroids may result in some motor improvement but has no effect on the CNS involvement. Despite a common time of presentation, these patients have a heterogeneous clinical profile, often suggesting a congenital muscular dystrophy syndrome.

Address correspondence and reprint requests to Dr. B. Rosenblatt, Room A-514, Montreal Children's Hospital, 2300 Tupper Ave., Montreal, PQ, Canada H3H 1P3.

Presented in part at the 24th Canadian Congress of Neurological Sciences, Ottawa, Ontario, Canada, June 1989.

Received September 6, 1989. Accepted for publication in final form December 8, 1989.




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