肝脳疾患"類瘢痕脳型"の1剖検例

AN AUTOPSY CASE OF "PSEUDO-ULEGYRIC TYPE" OF HEPATO-CEREBRAL DISEASE

　私たちは，20歳の男子に発病して，全経過約1年，繰り返す意識障害発作と，幼児化の方向への精神的退行を呈し，臨床診断が困難であつた，類瘢痕脳型（白木ら^{9） l0）}）の肝脳疾患の1例を経験した。この種の疾患の報告例はいまだ少なく，目下は臨床，病理像の報告をつみかさねることにより，将来その本態が明らかになるものと思うので，私たちはここに1例を報告して，若干の考察を加えたい。

The authors reported a case of "pseudo-ulegyric type" of hepato-cerebral disease.

1) Clinical course : A male aged 21 at the time of death. The patient had unbalanced diet. The illness began with insomnia and confusional state during the night. Recurring periods of clouding of consciousness lasting several hours were noted. About 5 months after the onset, he became bedrid-den and showed incontinence. In the later stage the intermittent periods of normality became short-er, and muscular rigidity set in in four limbs. Ema-ciation was marked, and he regressed mentally. Liver function test showed a slight degree of damage. Death ocurred about one year after the onset of the disease.

2) Pathological examination showed pseuso-ulegyric change as well as laminar cortical necrosis in the insula, T.2, T.3 and temporo-occipital regions on both sides, which were marked in the bottom of the sulci. Alzheimer's type II of glial cells were found diffusely in the:cerebral cortex, in thenucleus of which Carmin-positive substance was found.

3) The clinicopathological features were dis-cussed, and the comparison was made with 9 cases in the literature.