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 1 2013

Fingerprint

Stiff-Person Syndrome
Corpus Striatum
Glutamate Decarboxylase
Diffusion Magnetic Resonance Imaging
Intravenous Immunoglobulins
Spasm
Single-Photon Emission-Computed Tomography
Autoantibodies
Steroids
Magnetic Resonance Imaging
Antibodies
Therapeutics

All Science Journal Classification (ASJC) codes

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

Cite this

A case of childhood stiff-person syndrome with striatal lesions : A possible entity distinct from the classical adult form. / Sanefuji, Masafumi; Torisu, Hiroyuki; Kira, Ryutaro; Yamashita, Hiroshi; Ejima, Kazuna; Shigeto, Hiroshi; Takada, Yui; Yoshida, Keiko; Hara, Toshiro.

In: Brain and Development, Vol. 35, No. 6, 01.06.2013, p. 575-578.

Research output: Contribution to journalArticle

Sanefuji, Masafumi ; Torisu, Hiroyuki ; Kira, Ryutaro ; Yamashita, Hiroshi ; Ejima, Kazuna ; Shigeto, Hiroshi ; Takada, Yui ; Yoshida, Keiko ; Hara, Toshiro. / A case of childhood stiff-person syndrome with striatal lesions : A possible entity distinct from the classical adult form. In: Brain and Development. 2013 ; Vol. 35, No. 6. pp. 575-578.
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