肝脳疾患の本態に関する研究（第5報）

Pathogenesis of Hepatocerebral disease

Ⅰ．緒言

　或る実験仮説に立脚して研究を進めて行く場合，屡々吾々の予測と反した実験成績を得，其の判断に苦しむ事が多い。吾六は過去数年間肝臓と脳の機能的関連を認める総ての疾患を肝脳疾患と名付け，其の本態，分類及び相互関係に就いて臨床的，生化学的，組織化学的並びに病理学的に考察し発表を重ねて来た。^{1），2），3），4），5）），6），7）}其の間これ等疾患群の独自性，相関性に就き其の都度得られた実験成績から，色々の考察を加え発表してきたが，今迄の実験成績及び其の後に得られた成績を合せ，現在吾々の持つて居る本症候群に関する考え方を報告し，合せて今後の研究への態度を明らかにし度い。肝脳疾患とは次に示す様に臨床的には多くの疾患を含み一つの症候群と考えるのが妥当である。

　1）Wilson民病（進行性肝レンズ核変性症）。

During several years the authors have been studying about the pathogenesis of hepatocer-ebral disease including Wilson's disease.

As the authors had an opportunity to observe on 8 cases of Wilson's disease and 7 cases of special type of hepatocerebral degeneration (Inose) diagnosed by pathological and clinical findings from the biochemical, histochemical andpathological standpoints, the authors will explain our opinion on the pathogenesis of Wi-lson's disease and interrelation of etiology am-ong Wilson's disease, special type of hepatoce-rebral degeneration and various liver diseases.

Concerning to copper metabolism in Wilson's disease, moreover, the authours reseached for the problem "How copper plays a part in fetus liver and brain."

The conculsions are as followings ;

1). The abnormal accumulatioon and specific distribution of copper in brain and liver are pathognomonic findings for diagnosis of Wi- lson's disease in addition to the morpholog- ical changes such as pseudo-cirrhosis of liverand progressive and regressive changes of glial cells (Alzheimer I and II type of glial cell.).

However, one case which was diagnosed as Wilson's disease through pathological study revealed no accumulatuion of copper in brain and liver. The etiology of this case is tho-ught to differ from the other cases revealed copper accumulation. So the authors named conveniently this case as copper (-) Wilson's disease.

The other hand authors found atypical case of Wilson's disease diagnosed by copper acc-umulation and specific morphological changes, but its clinical course was almost resemble to that of special type of hepatocerebral degeneration.

2). The entity of Wilson's disease is thought to be the defficiency of caeruloplasmin in blood plasma, but abnormal accumulation of copper may primarily cause the development of specific morphological changes in liver and brain.

3). A special type of hepatocerebral degen-eration is thought to be a prolonged form of hepatic coma which appeares in general liver diseases.

4). Copper accumulation in fetus liver es-pecially after 5 month of pregnancy is thought to be secondary phenomenon obliged to the patten of fetus plasma protein (diminution of α₂ globulin) and the high level of serum copper.