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症例は56歳男性で,2003年1月より対人関係に頻繁にトラブルが生じ,8月に発熱のエピソードがあり,10月より舞踏病様不随意運動が左優位に出現するとともに関節の痛みを自覚し,以降会話が通じないほどの精神症状が現れた。入院時,痴呆,感情失禁,異常行動および四肢の舞踏病がみられた。頭部MRIでは右優位の尾状核頭の腫脹,脳FDG-PETでは両尾状核,特に右優位にグルコース代謝の亢進を認め,左優位の舞踏病様運動によく合致し,その原因として炎症が考えられた。ガリウムシンチグラムで四肢関節の異常集積亢進を認め,関節痛の改善により軽快した。関節痛,舞踏病,発熱,血沈亢進,CRPの上昇がみられたことよりシデナム舞踏病と考えられた。精神症状を伴った成人発症のシデナム舞踏病は,本例が初の報告であると思われた。
We report a 56-year-old man with adult-onset Sydenham chorea.Since January 2003, he had often troubled other persons, and in October 2003, following an episode of fever in August of the same year, he noticed left shoulder joint pain and involuntary movements of his limbs, especially on the left side.These involuntary movements gradually worsened and he became unable to converse due to psychiatric symptoms.On admission, neurological examination revealed dementia, emotional incontinence, abnormal behavior and chorea in four limbs.Brain MRI disclosed swelling of bilateral caudate heads that was more marked on the right side.Hypermetabolism in bilateral caudate nuclei, especially on the right, was found on FDG-PET study, which was compatible with his left side-dominant chorea and might reflect inflammation as a nature.A gallium scintigram demonstrated excess accumulations in the plural joints of his extremities, which gradually decreased in parallel with joint pain relief.The present case was diagnosed as Sydenham chorea, because of the presence of arthritis, chorea, fever, increased erythrocyte sedimentation rate and elevated CRP.We believe that this is a first report of adult-onset Sydenham chorea accompanied with psychiatric symptoms.
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