高度の好塩基性心筋変性を伴つたミオクローヌス癲癇の一剖検例

An Autopsy Case of Myoclonic Epilepsy Associated with Basophilic Myocardial Degeneration of Highest Degree

まえがき

　ミオクローヌス癲癇（以下ME）の臨床観察例は決して少くないが，その剖検報告例は極めて寥々たるものである。我々が生前脳波を記録し，剖検に依つて診断を確定しえた一経験例は遺伝的負荷も見られた。定型的なME症例であり，その稀少性のゆえのみによつても報告に価するものであろう。その上この症例は最高度の心筋好塩基性変性を伴つていたが，此種の心臓変化とMEの合併という症例は，今迄に全然報告されていない。癲癇の発生機序の解明について神経細胞の膜電位が重要視されつつある現在，心筋細胞と神経細胞の中に同時に異常な沈着物が出現していたこの症例は癲癇研究にある指針を与えるのでないかとも考えられるので，ここに報告して大方の批判を仰ぎたいと思う。

Recently the authors have experienced a very interesting autopsy case of myoclonic epilepsy, on which they have reported here.

A girl aged 17yrs., two of whose cousins were epileptikers, suffered often from tonsillitis early in life and also from rheumatic fever at the age of 10yrs. The Wassermann's reaction was ne-gative. Since Jan., 1954 she had o often epi-leptic seizures and gradually became stupid. Since Oct. 23, 1954 she was hospitalized for the treatment of seizures and mental deterioration. At that time she had jerking of fingers of both sides with a frequency of 200 times per minute. Afterwards epileptic seizures of various types (grand mal et al.) appeared more frequently and the mental deterioration became more se-vere, until she died in status epilepticus on June, 6, 1955. The electroencephalogram revealed many myoclonic spikes, but the electrocardio-gram (Ⅱ derivation) showed nothing abnormal.

The postmortal histologic examination disclo-sed a conspicuous appearance of myoclonic co-rpuscles on the broad areas of grey matter of the brain, of which were involved most marked-ly: thalamus, substantia nigra, dentate nucleus, inferior olivary nucleus and nuclei of posterior column. The heart had rheumatic scars and ischemic infarctions, but the most noteworthy change was basophilic degeneration of the hig-hest degree (In the Papillary muscle of the left ventricle the count of degeneration corpuscles was 391.4 per qcm.)

Myoclonic corpuscles, corpora amylacea and myocardial corpuscles were examined tinctorially and histochemically.

Since a cardiocerebral lesion of this kind has never been noticed on the world literature, the authors commented on some possible intertion between myoclonic epilepsy and basophilic myo-cardial degeneration.