Summary

Brain Dev. 2012 Nov;34(10):877-80. doi: 10.1016/j.braindev.2012.02.009. Epub 2012 Mar 19.

A pediatric patient with myopathy associated with antibodies to a signal recognition particle.

Abstract:

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.

日本語要旨:

15歳のSRPミオパチー例を報告した。発症後3ヶ月で著明に進行する近位筋有意の筋力低下と筋萎縮ならびに著明なCK値上昇を認めた。SRPミオパチー小児例はまれであるが、早期発見が予後改善に重要である。

PMID:  22436377

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