A case of childhood stiff-person syndrome with striatal lesions: A possible entity distinct from the classical adult form

Masafumi Sanefuji, Hiroyuki Torisu, Ryutaro Kira, Hiroshi Yamashita, Kazuna Ejima, Hiroshi Shigeto, Yui Takada, Keiko Yoshida, Toshiro Hara

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

Parainfectious or autoimmune striatal lesions have been repeatedly described in children. We report a 7-year-old girl with painful muscle spasms, leading to the diagnosis of childhood stiff-person syndrome (SPS). Striatal lesions were demonstrated by diffusion-weighted magnetic resonance imaging (MRI) and single-photon emission computed tomography but not by conventional MRI. Autoantibodies against glutamic acid decarboxylase (GAD) were absent. Steroid pulse therapy and high-dose intravenous immunoglobulin resolved all the symptoms with slight sequelae. Childhood SPS may be characterized by absent anti-GAD antibodies and a transient benign clinical course, and it may have a pathomechanism distinct from that in adult SPS.

Original languageEnglish
Pages (from-to)575-578
Number of pages4
JournalBrain and Development
Volume35
Issue number6
DOIs
Publication statusPublished - Jun 2013

All Science Journal Classification (ASJC) codes

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

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