異なる臨床経過を示し，非定型皮質基底核変性症が疑われた4症例

Four Cases with Suspected Atypical Corticobasal Degeneration, Showing Different Clinical Courses

抄録

　皮質基底核変性症は比較的まれな神経変性疾患で，錐体外路症状や不随意運動よりなる多彩な運動障害と失行・失語・前頭葉症状などの大脳皮質局在症状がみられ，症状はしばしば非対称性を示す。病変の局在により臨床症状の出現様式は様々で，一側性の運動障害で発症し臨床診断基準を満たす定型例に対し，非定型例では診断が困難であることがある。今回，前頭葉症状・非流暢性失語・パーキンソニスムを含む非定型な臨床像を示し，皮質基底核変性症が疑われた4症例の臨床経過と画像所見を提示した。このような非定型例に遭遇することはまれではなく，皮質基底核変性症は精神科領域でも診断上念頭に置くべき疾患と考えられる。

Summary

　Corticobasal degeneration (CBD) is a relatively rare neurodegenerative disease characterized by various movement disorders such as extrapyramidal signs, involuntary movements, cerebral cortical dysfunctions such as apraxia, aphasia and frontal signs. It also frequently shows asymmetric symptoms and signs. The apparent pattern of CBD symptoms varies according to the location of lesions. Typical cases initially develop unilateral movement disorders, and satisfy clinical diagnostic criteria of CBD. However, it is difficult to diagnose atypical cases, because they do not satisfy the diagnostic criteria. In this report, we demonstrate the clinical courses and neuroimaging features of four suspected atypical cases of CBD, showing different the clinical courses including frontal signs, non-fluent aphasia and parkinsonism. It is not unusual to encounter such atypical cases, so CBD is a disease to be further considered in the psychiatric field.