ミオクロヌスてんかん（Lafora型）患者と家族における脳波調査

THE INVESTIGATION OF ELECTROENCEPHALCGRAM OF PATIENTS SUFFERING EROM MYOCLONUS EPILEPSY (LAFORA-TYPE) AND ITS FAMILY

　Lafora型ミオクロヌスてんかんの患者3名と脳波異常のみを示す1名を含む家族7人に対して継続的脳波検査を行なつた。

　脳波的特徴は，α波をほとんど欠く基礎律動の不規則な徐波化，高電圧徐波，多くの場合は左右相称同期性の棘徐波結合体で，末期になるほど棘波と多棘波要素が増加した。しかしこれら異常波は特有な病理組織学的所見に特異的ではなくて，単に臨床所見に呼応するもののようであつた。

　われわれが初めて見出した7歳3ヵ月の一見健康な男子における棘徐波結合体の出現は，すでに病理組織学的基盤が発生していることを了想せしめるが，かかる脳波所見は患者の早期発見と家族内非顕性患者の発見にも寄与するものと思われる。

The electroencephalographical investigations on a family of myoclonus epilepsy (Lafora-type) have been carried out. The family is consisted of 7 members : including 3 patients suffering from myoclonus epilepsy and one sustaining only the abnormal electroencepha-logram without clinical manifestations.

The common findings are the scarcity of the alpha activity, the disrhythmic slow activity, and the high voltage slow bursts, most of them being bilaterally symmetrical and synchronous spike and wave com-plexes. There was the increase in spike discharge and multiple spikes in the later stage of the illness. These abnormal findings are, however, not the characteristics of the histopathological change, they are, however, according to the clinical conditions.

The appearance of spike and wave complex in 7 year old who seemed to be in the excellent health entirely, suggests that the abnormality has a histo-pathological base, and suggests at the same time that electroencephalogram is able to take place in the early diagnosis or the elucidation of latent patients in the members of patient's family.