Vigevano F, Fusco L, Di Capua M, Ricci S, Sebastianelli R, Lucchini P
Section of Neurophysiology, Bambino Gesu Children's Hospital, Rome, Italy.
Eur J Pediatr. 1992 Aug;151(8):608-12. doi: 10.1007/BF01957732.
Five infants, three girls and two boys, first had convulsions between the ages of 4 and 6 months. Although the aetiology of the attacks was unknown, all the infants had a family history of similar convulsions occurring at the same age and having a benign outcome. The attacks, which always occurred in a cluster, were promptly controlled, in four cases with phenobarbital and in one case with valproate. Seizures were partial with secondary generalization and were characterized by head and eye deviation (not always the same side in each attack) diffuse hypertonia and then bilateral limb jerks. The interictal EEG was normal. The ictal EEG showed diffuse discharge with onset in the central-occipital region. Laboratory, radiological and neurological findings were normal. A history in at least one paternal relative (the father in four cases) of similar seizures, occurring at the same age suggested a genetic predisposition. No seizures or EEG anomalies were observed during the follow up.
五名婴儿，三名女孩和两名男孩，首次惊厥发作于4至6个月龄之间。尽管发作的病因不明，但所有婴儿都有家族病史，即在相同年龄出现类似惊厥且预后良好。发作总是成簇出现，在四例中用苯巴比妥迅速控制，一例用丙戊酸盐控制。癫痫发作是部分性发作继发全身性发作，其特征为头部和眼睛偏斜（每次发作并不总是同一侧）、弥漫性肌张力亢进，然后是双侧肢体抽搐。发作间期脑电图正常。发作期脑电图显示中央枕区开始的弥漫性放电。实验室、放射学和神经学检查结果均正常。至少一名父系亲属（四例中的父亲）有相同年龄出现类似发作的病史，提示存在遗传易感性。随访期间未观察到癫痫发作或脑电图异常。
