ミオクローヌスてんかんの1例

A CASE OF THE MYOCLONUS EPILEPSY

I．まえがき

　1891年Unverrichtによつてはじめて記載されたミオクローヌスてんかん（以下MEと略称する）は比較的まれな疾患とされながら諸外国ではすでに相当数の症例報告をみている。一方わが国では大正13年の中沢による臨床報告，大正15年湧井による剖検報告以来約40年を経過しながら，その報告例は比較的少ない。

　われわれは最近MEの1症例を剖検する機会を得たのでこれを報告し，近時ようやく活発になりつつあるわが国におけるME研究の一資料としたい。

Case Z.T., male. There were no specific finding in the familial or hereditary relations. At the age of eighteen, the epileptic convulsive seizure had appeared. Thereafter, the systemic mycclonus had developed, which had been enhanced by photic stimula-tion. At the age of twenty-seven, dementia, delu-sion, hallucination, dysarthria, gait disturbance, hypotonia of muscles and the positive Romberg's sign were observed. The labolatory examination revealed scarcely any finding in urine, blood and cerebrospinal fluid. At twenty eight, he died by the asphyxia arosed from the epileptic convulsive seizure.

The cerebral weight was 1660g. Microscopically, the pia showed the fibrous thickening. Neurons of the cerebral cortex were slightly disappeared, and the cytoarchitecture were well preserved. In any part of the central nervous system, no LAFORA-body were found. There were macroglial prolifera-tion. Partially, the perivascular infiltration of the small round cells were observed. In thalamus, striatum, substantia nigra and olive, the neuronal changes were slight.

In the cerebellum, the Purkinje-cells were slightly disappeared- And in the white matter of the cere-bellum, the glial nodules were scattered. The myelin sheath in the dentate nucleus were degenerated, and small amount of fat granule cells were seen.

We consider that these changes in the dentate nucleus are related to the myoclonus of this case, furthermore we emphasize the important role of the dentate nucleus in the symptoms of the myoclonus epilepsy.