運動ニューロン疾患を伴い進行性の失語を呈した痴呆の1例

A Case of Motor Neuron Disease with Progressive Aphasia and Dementia

　症例は71歳女性。痴呆や運動ニューロン疾患の家族歴はなし。構音障害，嚥下障害などの球麻痺症状で発症し，失語が前景に立った痴呆症状が出現し，急速に進行し失外套症候群に至った。本例は，運動ニューロン疾患を伴う痴呆症（湯浅-三山病）や前頭側頭型痴呆と異なり，人格変化などの前頭葉症状は目立たない点，非流暢性失語が前景に出た痴呆症状がみられた点，形態画像と機能画像にて左側頭葉と両側頭頂葉の障害がみられた点で非典型的であった。

We report a 71-year-old woman showing rapidly progressive non-fluent aphasia and dementia accom-panied by motor neuron disease (MND). There was no family history of dementia or motor neuron dis-ease. There was 10 months history of dysarthria and dysphagia. On examination, she showed profound dif-ficulty in articulation. Her comprehension was im-paired in that she was unable to obey three-stage command. Her written language was also impaired with phonological spelling errors, syntactic errors, and perseveration.