Takao Muro

Studies on hepatocerebral disease: III. Hepatolenticular degeneration in Japan, with studies on copper metabolism

Age in Years Fig. 1. Age and sex of patients in Japan with Wilson’s disease in accord with American and European studies, that a disturbance of copper metabolism also plays a major role in the pathogenesis of Wilson’s disease in Japan. Similar but rare cases with clinicopathologic findings suggestive of Wilson’s disease but without an abnormality of copper metabolism have also been seen. Wilson’s disease used to be considered a very rare disease in Japan. However, much interest was aroused in this disease recently, and 62 cases have been reported in the past ten years. The inference is well grounded that many more unreported cases must be present in this countrv. The distribution of the patients showed no pnrticular grouping, and the cases were found in almost all sections of Japan. The heredity of 43 families was investigated in regard to this condition. Familial occurrence of the disease or blood relationship between father and mother of the patient was demonstrated in 15 families, or 35%. In the latter cases, most marriages were between cousins.

Studies on the neurohumoral control of the human body and its clinical applications: Histochemical study on cholinesterase of the human hypothalamus

1. The distribution of ChE in human hypothalamus was investigated withKoelles histochemical method. 2. Ganglionic cells of the paraventricular and supraoptic nuclei have strong ChE activity. Cells of the mamillo-infundibular nucleus and of the tuber nucleus have also strong ChE activity, though somewhat weaker than those of the former nuclei. 3. The small autonomic cells of the ventromedian and dorsomedian nuclei have markedly weaker ChE activity than those of the aforementioned nuclei. 4. ChE activity is found in protoplasm and nucleolus of ganglionic cells. However, nucleus of ganglionic cells shows no ChE activity. 5. Glial cells also have usually rather strong ChE activity. 6. The activity of ChE in the ground substance of various portions of hypothalamus is variable. 7. ChE activity of the corpus mamillare is as strong as those of putamen and caudate nucleus. ChE is present not only in ganglionic cells but also abundantly in the ground substance in the corpus mamillare.