SHIMIZU Yuko    Department   School of Medicine(Tokyo Women's Medical University Hospital), School of Medicine    Position   Professor
Article types	Case report
Language	English
Peer review	Peer reviewed
Title	Anti-signal recognition particle myopathy in the first decade of life.
Journal	Formal name：Pediatric neurology Abbreviation：Pediatr Neurol ISSN code：18735150/08878994
Domestic / Foregin	Foregin
Volume, Issue, Page	45(2),pp.114-6
Author and coauthor	Suzuki Shigeaki, Ohta Masayasu, Shimizu Yuko, Hayashi Yukiko K, Nishino Ichizo
Publication date	2011/08
Summary	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