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

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

Research output: Contribution to journalArticle

22 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)114-116
Number of pages3
JournalPediatric Neurology
Volume45
Issue number2
DOIs
Publication statusPublished - 2011 Aug

Fingerprint

Signal Recognition Particle
Muscular Diseases
Muscle Weakness
Creatine Kinase
Extremities
Facioscapulohumeral Muscular Dystrophy
Limb-Girdle Muscular Dystrophies
Muscles
Muscular Atrophy
Myalgia
Immunosuppressive Agents
Autoantibodies
Arthritis
Differential Diagnosis
Fibrosis
Fever
Biopsy
Serum

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Developmental Neuroscience
  • Neurology

Cite this

Anti-signal recognition particle myopathy in the first decade of life. / Suzuki, Shigeaki; Ohta, Masayasu; Shimizu, Yuko; Hayashi, Yukiko K.; Nishino, Ichizo.

In: Pediatric Neurology, Vol. 45, No. 2, 08.2011, p. 114-116.

Research output: Contribution to journalArticle

Suzuki, Shigeaki ; Ohta, Masayasu ; Shimizu, Yuko ; Hayashi, Yukiko K. ; Nishino, Ichizo. / Anti-signal recognition particle myopathy in the first decade of life. In: Pediatric Neurology. 2011 ; Vol. 45, No. 2. pp. 114-116.
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