肝腦変性疾患特殊型の1例

A case of the particular form of the hepato-cerebral disease.

1．序言

　一般にウイルソン仮性硬化症群と総称される疾患に対し，本邦に於て之と類似の肝脳変性疾患が存在する事は，最近注目されつゝあるところである。之に就いて猪瀨^1）は昭和24年肝脳変性疾患特殊型として，その病理組織学的特徴を詳細に記載し，之をウイルソン仮性硬化症群より区別すべき事を強調した。

　本症に対しては数年来，生化学的研究^2）が活溌に行われ，臨床経験も増しつゝあり，一般にウイルソン仮性硬化症群と同様，「肝変化なくして本症なし」と云う概念が確立された観がある。併しその本態に就いては，依然として不明のまゝであり，今後更に症例を増して研究を進めるべき段階にある。

A man, 23 years old. Suddenly he lost con-sciousness, but did'nt fall into coma. He was excited by hallucinations in dazed condition. Imfortant clinical symptoms were following : periodical coma, organic dementia and emotinal irritability. Neurological symptoms were signs of pyramidal and extrapyramidal tracts (im-perfect left hemiplegia, handtremor, slow be-haviour etc.). Other somatic symptoms were functional obstruction of liver, emaciation, gingival hemorrhage, ascites chylosus but no Kayser-Fleischer's corneal-ring. After 11 months he died on the last coma.

The histological characteristics were the following four matter : 1. the cortex was de-generated diffusely. 2. an appearance of Alz-heimer's atypical large glia cells (only second type), which were impregnated as the progres-sive change of astrocytes. 3. proof of red en-closures into nucleus of Alzheimer's cells by glycogen-staining. 4. cirrhosis of liver. 1949, Dr. Inose reported the same clinical cases and concluded that above mentioned histological four characteristics were different from that of Wilson-pseudosclerosis.

I concluded that particular change of meta-bolism which was derived from cirrhosis of liver, influenced the blood-brain-barrier.