多数のKuru斑を伴なった亜急性海綿状脳症の1例

A case of subacute spongiform encephalopathy with numerous Kuru plaques in the cerebral and cerebellar cortices

I．はじめに

　Kuru斑を伴なう亜急性海綿状脳症は，とくにわが国でかなりの報告がある。これらの例はいずれも，歩行障害で発してのち，次第に痴呆が進行してくるという共通の臨床経過を示し，神経病理学的にも海綿状脳症のほかにKuru病でみられるplaque（Kuru斑）が多数出現するという共通の所見を持っており，Creutzfeld-Jakob病群中の興味ある一群である。私どもは最近，類似の1剖検例を検索しえたので報告する。

CLINICAL DESCRIPTION

　The case is a Japanese male aged 49 years at his death. In his family three of six siblings have progressive cerebellar ataxia since childhood.

　In the summer of 1971 the patient noticed that his gait was becoming unsteady. In January 1973 he found continuous burning sensation in his both feet and then his gait disturbance became far much worse. By February 1973 he was unable to do fine works because of clumsiness and progressed unsteady gait.