老人斑，海綿状態，脱髄など多彩な病理像を示した脳症の1例

A peculiar case of panencephalopathy with widespread distribution of plaques, status spongiosus and demvelination

はじめに

　われわれは最近，臨床的に遺伝性小脳失調症と診断され，神経病理学的にCreutzfeldt-Jakob病（C-J病）に通常みられる所見に加えて，無数のplaqueと広汎な脱髄を示した症例を経験したが，このような症例は過去に例を見ないのでここに報告する。

　The patient was a 56 years old Japanese male whose father and one of his sisters were known to die of the similar disease.

　The onset of the illness was at the age of 46 years with complaints of gait disturbances and sharp pains in the lower extremities. Subsequently, dysarthria and marked ataxia in the trunk and extremities developed. Six years later, apathy, urinary incontinence, ataxic eye movements, bulbar palsy, fasciculation of the muscles and convulsions became manifested.