Muranaka H, Takebe Y
Department of Pediatrics, Hirosaki University School of Medicine, Aomori.
No To Hattatsu. 1994 Sep;26(5):428-33.
A case of unusual early myoclonic encephalopathy was reported. He showed massive and/or fragmentary myoclonic seizures soon after birth until 20 months before death. These seizures were resistant to various kinds of anti-epileptic drugs. The EEG pattern of suppression-burst was continuously seen in both waking and sleep states from 2 months to 19 months of age. He was supported with mechanical ventilator because of the central respiratory dysfunction since 4 days after birth until death. This case was electroclinically considered as that of early myoclonic encephalopathy. The central respiratory dysfunction has not been reported in early myoclonic encephalopathy.
报告了一例罕见的早期肌阵挛性脑病病例。他在出生后不久直至死亡前20个月出现大量和/或片段性肌阵挛发作。这些发作对各种抗癫痫药物均耐药。从2个月至19个月大，在清醒和睡眠状态下均持续出现抑制-爆发脑电图模式。自出生后4天直至死亡，由于中枢性呼吸功能障碍，他一直依靠机械通气维持生命。该病例在临床电生理方面被诊断为早期肌阵挛性脑病。早期肌阵挛性脑病中尚未有中枢性呼吸功能障碍的报道。
