病初期に運動性失語を呈した認知症を伴う筋萎縮性側索硬化症の1例

A Case of Amyotrophic Lateral Sclerosis Presenting as Motor Aphasia in its Early Stage

　要旨　76歳女性。言語の緩慢さと呂律困難を発症し，運動性失語を指摘された。以後徐々に認知症と全身の筋萎縮が進行。神経所見および電気生理学的に認知症を伴う筋萎縮性側索硬化症 （ALSD） と診断。発症1年6カ月後に呼吸不全で死亡した。剖検所見では，大脳皮質のグリオーシス，皮質浅層の小径ニューロンを中心としたユビキチン陽性封入体，海馬CA1細胞から内嗅野皮質にかけての神経原線維変化，脊髄前角細胞の脱落などが認められ，ALSDと合致する所見であった。ALSDのサブタイプとして運動性失語で発症する症例があり，注意を要する。

　A 76-year-old right-handed woman complained of speech disturbance and difficulity of singing was admitted to our hospital. Examination showed motor aphasia and mild cognitive impairment. After she was discharged, dementia and weakness of the extremities had rapidly progressed. She was readmitted eight month after the first visit, when she was almost abulic, her skeletal and bulbar muscles were remarkably atrophic, and hyperreflexia of the extremities were seen. Electromyographcal study showed neurogenic pattern. These findings suggest amyotrophic lateral sclerosis （ALS） with dementia. Pathological findings were atrophy at the anterior horn of the spinal cord.The brain was diffusely atrophic. The extent of degenerative change was not lateralized. This case is a discriminative type of ALS with dementia, that its first symptom is motor aphasia.