Martin H J, Deroubaix-Tella P, Thelliez P
Rev Electroencephalogr Neurophysiol Clin. 1981 Dec;11(3-4):397-403. doi: 10.1016/s0370-4475(81)80077-9.
In a neonate who had seizures in the second day of life, the EEG showed - during waking and sleep alike - periodic bursts of asymmetrical high voltage on a low amplitude background tracing. From the third week head jerks occurred, followed by repetitive spasms and then adversive tonic seizures. A severe psychomotor retardation was noted. A CT examination demonstrated a right hemispheric hypertrophy. The pathological examination revealed astrocytic gliosis of the cortex associated with a decrease in the oxidative enzyme activity of neurons, and characterized electron microscopically, by the presence of spheroid bodies in the neuropile. This case was different from those published by Aicardi and Goutières (encéphalopathie myoclonique néo-natale) because there was no erratic myoclonus. It is more similar to the 'early infantile epileptic encephalopathy with suppression bursts' described by Ohtahara. This electro-clinical condition should be characterized as the earliest type of secondary generalized epilepsy.
在一名出生第二天出现惊厥的新生儿中,脑电图显示——无论在清醒还是睡眠状态下——在低振幅背景描记图上有不对称的高电压周期性爆发。从第三周开始出现头部抽搐,随后是重复性痉挛,然后是强直性扭转性发作。观察到严重的精神运动发育迟缓。CT检查显示右半球肥大。病理检查显示皮质星形细胞胶质增生,伴有神经元氧化酶活性降低,电镜下特征为神经毡中有球形小体。该病例与Aicardi和Goutières报道的(新生儿肌阵挛性脑病)不同,因为没有不规则肌阵挛。它更类似于大原描述的“具有爆发抑制的早期婴儿癫痫性脑病”。这种电临床情况应被视为继发性全身性癫痫的最早类型。