title: A case of childhood stiff-person syndrome with striatal lesions: a possible entity distinct from the classical adult form.
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.
authors.affiliation: Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Japan. sane26@pediatr.med.kyushu-u.ac.jp
authors.forename: Masafumi
authors.initials: M
authors.lastname: Sanefuji
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Torisu
authors.forename: Ryutaro
authors.initials: R
authors.lastname: Kira
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Yamashita
authors.forename: Kazuna
authors.initials: K
authors.lastname: Ejima
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Shigeto
authors.forename: Yui
authors.initials: Y
authors.lastname: Takada
authors.forename: Keiko
authors.initials: K
authors.lastname: Yoshida
authors.forename: Toshiro
authors.initials: T
authors.lastname: Hara
mesh_terms: D002648:Child; D003342:Corpus Striatum / Q000000981:diagnostic imaging / Q000473:pathology*; D038524:Diffusion Magnetic Resonance Imaging; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D053609:Lethargy / Q000150:complications; D008279:Magnetic Resonance Imaging; D009771:Obsessive-Compulsive Disorder / Q000150:complications; D016750:Stiff-Person Syndrome / Q000150:complications / Q000473:pathology* / Q000628:therapy; D015899:Tomography, Emission-Computed, Single-Photon
chemical_list: D016756:Immunoglobulins, Intravenous