肝レンズ核変性症候群（Wilson氏病）の1症例とそのB． A． L療法に就て

A Case of Hepatolenticular Degeneration (Wilson's Desease) and the Effect of B. A. L Treatment

　1912年にKinnear Wilson^1）は，進行性レンズ核変性と題する論文に，肝臓及び脳の両者に病変の存する13例の症例を報告した。

　此れは，屡々家族的に発生し，錐体外路症状を呈しつつ進行し，遂には死亡したものであるが，剖検により全例に著明な肝硬変像を認めている。

1. A case of seventeen years old male who developed hepatolenticular degeneration syndro-me was reported. This case showed extrapyra-midal syndrome such as rhythmic tremor and rigidity of limbs, and marked damage of the liver as revealed by both clinical findings and laboratory tests. The presence of the Kayser-Fleischer rings were recognized on the cornea of both eyes.

2. There were decrease of copper content of the serum (0.087mg/dl) increaese in excretion of copper in the urine (0.597mg/a day) and also increase in excretion of amino acid in the urine (333.2mg/a day).

3. The patient was given for 19 days a co-urse of BAL intramuscularly every twelve ho-urs of 120mg. By this treatment the extrapyra-midal syndrome gradually improved, however there was no improvement of the liver function. Excretion of the copper in the urine was in-creased by the injection of BAL.

4. The patient showed gynecomastia, hypo-gonadism and vascular spiders on the skin, which were considered to be due to the liver damage.

5. It was found out that copper particles were located in the liver and the cerebral basal ganglion (putamen), which may have something to do with the etiology of this illness. As we recognized that the increase in excretion of co-pper by BAL braught clinical improvement, it is considered that the abnormal metabolism of co-pper may leads to this sort of hepatolenticular degeneration.