Verrotti A, Greco R, Chiarelli F, Domizio S, Sabatino G, Morgese G
Department of Medicine, Pediatrics Section, University of Chieti, Italy.
J Child Neurol. 1999 Nov;14(11):746-9. doi: 10.1177/088307389901401112.
We studied six children (four girls and two boys) suffering from cryptogenic myoclonic absence seizures with early onset. The age at onset of the seizures ranged between 6 and 27.8 months (mean age +/- SD: 18.5+/-12.4 months). The neurologic evaluation was normal in all patients at the first hospital admission. After the diagnosis, we followed up all children for at least 5 years. At the end of follow-up, two of these patients (a girl and a boy) showed severe mental retardation, a high number (from one to three per day) of seizures, and persistent pathologic electroencephalograms. The other patients showed normal electroencephalograms: all of them were seizure free and without mental retardation. The two patients with mental retardation have been treated with polytherapy. In all other children we used valproate alone successfully. Our data suggest that myoclonic absence seizures with early onset can have a good long-term prognosis. Valproate is a useful anticonvulsant drug in these patients. Mental retardation is present only in patients with poor seizure control.
我们研究了6名患有早发性隐源性肌阵挛失神癫痫的儿童（4名女孩和2名男孩）。癫痫发作的起始年龄在6至27.8个月之间（平均年龄±标准差：18.5±12.4个月）。所有患者首次入院时神经学评估均正常。确诊后，我们对所有儿童进行了至少5年的随访。随访结束时，其中两名患者（一名女孩和一名男孩）出现严重智力发育迟缓、癫痫发作频繁（每天1至3次）以及持续的病理性脑电图。其他患者脑电图正常：他们均无癫痫发作且无智力发育迟缓。两名智力发育迟缓的患者接受了联合治疗。在所有其他儿童中，我们单独使用丙戊酸盐取得了成功。我们的数据表明，早发性肌阵挛失神癫痫可能有良好的长期预后。丙戊酸盐在这些患者中是一种有用的抗惊厥药物。智力发育迟缓仅出现在癫痫控制不佳的患者中。
