What is it about?
In order to reveal the present status of an extremely rare disorder stiff-person syndrome (SPS) in Japan, the nationwide survey was conducted in 3 consecutive years starting in 2015.
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Why is it important?
The prevalence of STS was estimated to be 0.11 per 100,000 population. The median age of onset was 51 years, while thee quarters were female. The outcome of patients was generally favorable, however, it was found that aggressive immunotherapies were necessary for GAD65-positive patients with SPS.
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This page is a summary of: Prevalence, Clinical Profiles, and Prognosis of Stiff-Person Syndrome in a Japanese Nationwide Survey, Neurology Neuroimmunology & Neuroinflammation, November 2023, Wolters Kluwer Health,
DOI: 10.1212/nxi.0000000000200165.
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