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Stiff-Person Syndrome Naoko Matsui 1 , Keiko Tanaka 2 , Yuishin Izumi 1 1Department of Neurology, Tokushima University Hospital 2Department of Comparative and Experimental Medicine, Brain Research Institute, Niigata University Keyword: スティッフパーソン症候群 , 筋硬直 , 筋痙攣 , 傍腫瘍症候群 , 自己抗体 , stiff-person syndrome , muscle rigidity , spasm , paraneoplastic syndrome , autoantibody pp.640-646
Published Date 2021/5/1
DOI https://doi.org/10.11477/mf.1416201808
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Abstract

Stiff-person syndrome (SPS) is a neurological 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 the 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 high titers of antibodies against glutamic acid decarboxylase (GAD), the enzyme that limits the rate of the synthesis of γ-aminobutyric acid (GABA), and up to 15% have antibodies against the glycine receptor α-subunit.


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電子版ISSN 1344-8129 印刷版ISSN 1881-6096 医学書院

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