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緩徐に進行する言語表出困難を呈した60歳,女性患者を報告した。発話速度が極端に速く,語音の著しい歪みとともに無関係な語音が混入し反復し,言語が加速する特異な発語障害を認めた。言語理解や書字は保たれ,口部顔面失行やタッピング動作困難も随伴していた。
従来報告されている原発性進行性失語(PPA)の非流暢性タイプでは,発話速度は遅く努力性であるので,本患者の発語障害の特徴が,左シルヴィウス裂周囲の大脳皮質病変による巣症状(アフェミア)のみでなく,大脳基底核病変に起因するリズム障害を反映している可能性が考えられた。
Primary progressive aphasia is a rare disorder of unknown cause. We report a patient with progres-sive loss of speech output, a clinical variant of PPA, characterized by festinating speech.
A 60-year-old right handed woman was admitted to our hospital, because of progressive deterioration of her speech. On admission, she was alert andorientated without dementia. A severe impairment of her articulation was observed : her speech rate was so fast that her speech became almost intelli-gible. The orofacial apraxia and difficulty in tap-ping were also present. The other neurological findings were normal.
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