Stiff-Person Syndrome and Related Autoantibodies Naoko Matsui 1 , Keiko Tanaka 2 , Ryuji Kaji 1 1Department of Clinical Neuroscience, Institute of Biomedical Sciences, Tokushima University Graduate School 2Department of Cellular Neurobiology, Brain Research Institute, Niigata University Keyword: 筋硬直 , 筋痙攣 , 自己免疫異常 , 傍腫瘍症候群 , 自己抗体 , muscle rigidity , spasm , autoimmune disorders , paraneoplastic syndrome , autoantibody pp.357-362
Published Date 2018/4/1
DOI https://doi.org/10.11477/mf.1416201008
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Stiff-person syndrome (SPS) is a disorder characterized by fluctuating muscle rigidity and painful spasms that occur spontaneously or are triggered by diverse stimuli. Partial or segmental forms of the disorder, such as stiff-limb syndrome (SLS) and a more severe disease called progressive encephalomyelitis with rigidity and myoclonus (PERM), are usually considered within the spectrum of SPS. SPS responds to immunotherapies, and several autoantigens have been identified. Most patients with SPS have a high-titer of antibodies against glutamic acid decarboxylase (GAD), the rate-limiting enzyme for the synthesis of γ-aminobutyric acid (GABA), and up to 15% have antibodies to the glycine receptor α-subunit. This review explains milestones in defining SPS including autoantibodies.

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