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症例は死亡時60歳男性で1994年5月頃(58歳)より歩行時ふらつき,構音障害が出現し,全経過2年で死亡。神経学的に小脳症状,錐体路徴候,自律神経症状を認め,自律神経機能検査,123I-metaiodobenzyl-guanidine心筋シンチグラフィーで交感・副交感神経障害を確認,脳MRIで小脳・橋の萎縮を認め多系統萎縮症と臨床診断した。神経病理学的にはオリーブ橋小脳病変,自律神経病変,glial cytoplasmic inclusionなどを認めたことより多系統萎縮症と確定診断した,本例では123I-metaiodobenzylguanidine心筋シンチグラフィーで非常に急速な心臓交感神経の障害が確認され,このような急速な自律神経障害の進展が罹病期間の短さの原因の一つであると考えられた。
We report an autopsy case of multiple system atro-phy (MSA) presenting with rapid progression of auto-nomic disturbance. He was admitted to our hospital because of gait disturbance and dysarthria.
The patient was a Japanese man, who first noticed gait disturbance and dysarthria at age 58, followed by syncope 3 months later. He developed urinary inconti-nence and frequency of urination 8 months after the disease onset. His gait disturbance, dysarthria, syn-cope, and urinary symptoms progressed, and he was admitted to the department of neurology 1 year after the onset of the disease.
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