Journal
BRAIN & DEVELOPMENT
Volume 34, Issue 10, Pages 877-880Publisher
ELSEVIER SCIENCE BV
DOI: 10.1016/j.braindev.2012.02.009
Keywords
Signal recognition particle; Anti-SRP myopathy; Muscular dystrophy; Inflammatory myopathy; Myositis; Muscle pathological findings
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We report the case of a 15-year-old Japanese girl with myopathy associated with antibodies to a signal recognition particle (anti-SRP myopathy). The patient presented with progressive symmetrical proximal muscle weakness that caused difficulty in walking within 3 months, and marked elevation of the serum creatine kinase levels. A skeletal muscle biopsy revealed active necrotic and regenerating processes, with mild inflammatory changes. Based on the above findings, the patient was diagnosed as having anti-SRP myopathy. Only a limited number of pediatric patients with anti-SRP myopathy has been reported previously, with usually a poor prognosis. Early diagnosis is important for obtaining a better prognosis in patients with anti-SRP myopathy. (C) 2012 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
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