Holmes G L
Department of Neurology, Harvard Medical School, Children's Hospital, Boston, Massachusetts.
Epilepsia. 1993;34 Suppl 3:S49-61. doi: 10.1111/j.1528-1167.1993.tb06259.x.
Until relatively recently, genetic influences in partial seizures were thought to be of minimal importance. However, with further identification of childhood benign partial seizures it is becoming clear that inheritance plays a major role in the pathogenesis of these seizures. Diagnostic criteria proposed for benign partial seizures include absence of neurologic or intellectual deficits, family history of epilepsy, onset of seizures after age 2 years, stereotyped brief seizures, frequent nocturnal occurrence, spontaneous remission in adolescence, and electroencephalograms (EEGs) demonstrating spikes with a distinctive morphology and localization superimposed on normal background activity. The two most commonly described benign partial epilepsies of childhood are benign Rolandic epilepsy (BRE) and benign occipital epilepsy (BOE). Both disorders begin in childhood, are associated with characteristic EEG patterns, have seizures that are easily controlled with medication, often are familial, and have an excellent prognosis. The other benign partial seizure disorders in children that have been described are not as well studied as BOE and BRE, and the role of inheritance pattern, if any, is less clear.
直到最近，人们还认为遗传因素在部分性癫痫发作中作用极小。然而，随着儿童良性部分性癫痫的进一步明确，越来越清楚的是，遗传在这些癫痫发作的发病机制中起主要作用。针对良性部分性癫痫发作提出的诊断标准包括无神经或智力缺陷、癫痫家族史、2岁后发作、刻板短暂发作、频繁夜间发作、青春期自发缓解以及脑电图（EEG）显示在正常背景活动上叠加有形态和定位独特的棘波。儿童中最常描述的两种良性部分性癫痫是良性罗兰多癫痫（BRE）和良性枕叶癫痫（BOE）。这两种疾病均始于儿童期，与特征性脑电图模式相关，发作易通过药物控制，常具有家族性，且预后良好。已描述的儿童其他良性部分性癫痫发作疾病不如BOE和BRE研究充分，遗传模式（如果存在的话）的作用也不太明确。
