Wilson仮性硬化症群の一症例に就いて

A Case of Wilson's Disease (Hepatolenticular Degeneration

I．緒言

　1883年にWestphai^26）が多発性硬化症に似た症状を呈し，然も剖検上，中枢神経系に於いて何等硬化病巣を発見出来ない神経病の一群を認め，仮りに仮性硬化症Pseudoskleroseなる名称を与えたのを初めとして，1912年にWilson^27）が進行性レンズ核変性Progressive lenticular degenerationなる名のもとに，臨床的に仮性硬化症と酷似する，孤立的又は家族性疾患にして，解剖学的に常に肝硬変を伴い，中枢神経系に於いては両側レンズ核に限られた軟化の見られる一群の疾患例を報告し，所謂Wilson氏病なる名称の濫觴を成したのである。その後，Spielmeyer （1920）^19）の詳細なる病理組織学的研究により，両者は実は同一の病的過程であつて，本質的差違が存しないと云う結論に達し，現今ではウイルソン-仮性硬化症群Wilson-Pseudosklerose-Gruppe又は肝脳変性Degeneratio hepatolenticularis （1921,Hall）^4）と一括して呼ばれる様になつたが，如何にして肝と脳にかかる病的変化が生ずるかと云う問題は，未だ充分に解明せられてはいない。

　我々は最近，臨床的に特有なる錐体外路性運動障碍を呈し，病理解剖学的に両側被殻の変性と肝硬変が見出された一症例を経験し，肝脳変性に就いて考察する機会を得たのでここに報告する。

Recently, we observed a case of Wilson's disease which showed clinically the extrapyra-midal syndrome and histo-pathologically the degeneration of bilateral putamen and cirrhosis of the liver.

A case of 42 year old doctor who showed clinically extrapyramidal syndrome such as masklike face, tremor of the left hand, flection of the head and accentuation of the muscle tone in the biginning and simultaneously exhi-bited dysarthria, dysgraphia dysphagia and de-mentia and then progressed in contracture of four extremities in the end.

Histopathologically, the most characteristic feature is the degeneration of ventral part of bilateral putamen, that is, the yellow coloration and malacia of putamen. We observed total falling of the medullary sheath and the degene-ration and disappearance of the nerve cells and on the other hand found the proliferation of blood-vessels and microglia in this submalactic tissue and recognized the enormous deposition of neutral fat in the central part of this pa-thological tissue. Moreover, we recognized the numerous indications of Alzheimer's glia cell of the First and the Second type which is cha-racteristic of this disease, among the degene-rated nerve cells. The liver was accompanied with the proliferation of the connective tissue and pseudo-gall-duct and the degeneration of the liver cells.

The above clinical and histopathological view is closely combined with that of the cases of disease which was reported by Wilson.