両側側頭葉の軟化を示した1例

A Case of Bilateral Temporal Softening

I．はじめに

　側頭葉機能に関しては，Pick病等の変性疾患，脳外科的臨床経験，動物実験等による多くの報告がなされ注目されてきている。またこの部は組織病理学的にもPick病のほか，急性壊死性脳炎，肝脳疾患類瘢痕脳型等特殊な疾病の好発部位でもある。

　私共は臨床的には問題児として1年余とりあつかわれ，脳剖検では両側々頭葉の軟化と広汎な神経細胞脱落，gliaの変性増殖を示す1例を経験した。臨床病像も従来の側頭葉損傷例に照らして特異的であり，組織病理学的にも多彩な知見を含んでいて簡単に特定の疾患とみなすことは難かしい。ここにその臨床と病理を報告し，今後類似した症例の追加と検討をまちたい。

This is a 10-year-old boy. The initial symptom was character change such as hyperactivity, lying and swindling. One year after the onset of the illness, he had an epileptic convulsion and then became confused and showed oral tendency and stereotypy. The consciousness became more and more distrubed in 2 months and died of an attackof fever.

The postmortem examination revealed the softe-ning in the bilateral temporal lobes. Microscopica-lly the followings were detected: The spongy state in the subcortical white matter in the temporal lo-bes. The disintegration of nerve cells, especially in the temporal-and occipital lobes. The appeara-nce of Alzheimer type Ⅱ glial cells with intranuc-lear PAS positive inclusion's body, widely dissemi-nated in gray and white matter. The appearance of great number of microglial cells in both white and gray matter.

These histopathological findings of the brain are highly similar to the "Pseudoulegyria Type (Shira-ki)"of hepatocerebral disease. But the clinical course of behavior disorder distinguishes this case from other hepatocerebral disease with episodic bursts of conscious disturbance.