A case of childhood stiff-person syndrome with striatal lesions: A possible entity distinct from the classical adult form |
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| Authors: | Masafumi Sanefuji Hiroyuki Torisu Ryutaro Kira Hiroshi Yamashita Kazuna Ejima Hiroshi Shigeto Yui Takada Keiko Yoshida Toshiro Hara |
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| Affiliation: | 1. Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Japan;2. Department of Neuropsychiatry, Graduate School of Medical Sciences, Kyushu University, Japan;3. Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Japan |
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| 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. |
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| Keywords: | DWI, diffusion-weighted imaging GAD, glutamic acid decarboxylase SPECT, single-photon emission computed tomography SPS, stiff-person syndrome |
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