Coppola G, Plouin P, Chiron C, Robain O, Dulac O
Neuropediatric Department, Université René Descartes, Paris, France.
Epilepsia. 1995 Oct;36(10):1017-24. doi: 10.1111/j.1528-1157.1995.tb00961.x.
Fourteen infants of both sexes had a previously unreported epileptic condition characterized by nearly continuous multifocal seizures. The first seizures occurred at a mean age of 3 months, without antecedent risk factors. At 1 to 10 months, the seizures became very frequent. They were partial with variable clinical expression, and the EEG showed that the discharges randomly involved multiple independent sites, moving from one cortical area to another in consecutive seizures. Although their topography varied, the EEG ictal pattern of each seizure was very similar. It consisted of rhythmic alpha or theta activity which spread to involve an increasing area of the cortical surface. Patients regressed developmentally and became quadriplegic with severe axial hypotonia. Three patients died at age 7 months and at age 7 and 8 years, respectively. Seizures were controlled in only 2 patients, and only 3 children resumed psychomotor development. Extensive investigation failed to determine an etiology, and there was no familial recurrence. Neuropathological examination of the brain in two cases showed only severe hippocampal neuronal loss and accompanying gliosis.
14名男女婴儿患有一种先前未报告的癫痫病症，其特征为几乎持续的多灶性癫痫发作。首次癫痫发作的平均年龄为3个月，无前驱危险因素。在1至10个月时，癫痫发作变得非常频繁。发作属于部分性发作，临床表现各异，脑电图显示放电随机累及多个独立部位，在连续发作中从一个皮质区域转移到另一个区域。尽管其部位不同，但每次癫痫发作的脑电图发作模式非常相似。它由有节律的α或θ活动组成，这种活动扩散并累及越来越大的皮质表面区域。患者出现发育倒退，发展为四肢瘫痪并伴有严重的轴向肌张力减退。3名患者分别在7个月、7岁和8岁时死亡。仅2例患者的癫痫发作得到控制，只有3名儿童恢复了精神运动发育。广泛的调查未能确定病因，且无家族复发情况。对两例患者的大脑进行神经病理学检查，仅显示严重的海马神经元丢失及伴随的胶质增生。
