シミズ　ユウコ   SHIMIZU Yuko   清水　優子    所属   医学部 医学科（東京女子医科大学病院）    職種   教授
論文種別	症例報告
言語種別	英語
査読の有無	査読あり
表題	Anti-signal recognition particle myopathy in the first decade of life.
掲載誌名	正式名：Pediatric neurology 略　 称：Pediatr Neurol ISSNコード：18735150/08878994
掲載区分	国外
巻・号・頁	45(2),pp.114-6
著者・共著者	Suzuki Shigeaki, Ohta Masayasu, Shimizu Yuko, Hayashi Yukiko K, Nishino Ichizo
発行年月	2011/08
概要	Autoantibodies to signal recognition particle have been associated with juvenile and adult-onset necrotizing myopathy. However, only a few teenage patients with anti-signal recognition particle myopathy have been reported, and to date, to our knowledge, no patient younger than 10 years has been documented. We describe 2 Japanese girls with anti-signal recognition particle myopathy who developed symptoms from the ages of 5 and 9 years, respectively. Both patients had progressive muscle weakness and atrophy without myalgia. Facioscapulohumeral muscular dystrophy was initially suspected because of asymmetric shoulder girdle muscle involvement in one patient, and limb girdle muscular dystrophy due to proximal limb muscle weakness in the other. There were no extramuscular manifestations, including fever or arthritis. Serum creatine kinase levels were elevated to 2,467-4,629 IU/L. Results of muscle biopsy revealed necrotizing myopathy with minimal to mild endomysial fibrosis but without inflammatory infiltrates. Immunosuppressive agents were not effective for muscle weakness, resulting in marked disability. Anti-signal recognition particle myopathy can occur in the first decade of life and should be included in the differential diagnosis for children with progressive limb girdle muscle weakness and high creatine kinase levels.
DOI	10.1016/j.pediatrneurol.2011.04.009
PMID	21763952