Anti-signal recognition particle myopathy in the first decade of life

Shigeaki Suzuki, Masayasu Ohta, Yuko Shimizu, Yukiko K. Hayashi, Ichizo Nishino

研究成果: Article査読

25 被引用数 (Scopus)


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.

ジャーナルPediatric Neurology
出版ステータスPublished - 2011 8月

ASJC Scopus subject areas

  • 小児科学、周産期医学および子どもの健康
  • 神経学
  • 発達神経科学
  • 臨床神経学


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