Cusmai R, Ricci S, Pinard J M, Plouin P, Fariello G, Dulac O
Service de Neuropédiatrie, Hopital Saint Vincent de Paul, Unité INSERM U29, Paris, France.
Epilepsia. 1993 Jul-Aug;34(4):738-42. doi: 10.1111/j.1528-1157.1993.tb00455.x.
Clinical, neuroradiological data and outcome of epilepsy of 32 patients with symptomatic infantile spasms caused by perinatal insults are reported. Neuroradiological investigations showed focal as well as diffuse cerebral lesions. Fifteen children had a porencephalic lesion, 12 had periventricular leukomalacia and five had diffuse bilateral cerebral atrophy, associated with status marmoratus in one child and bilateral ulegyria in another. Eight patients (25%) had epilepsy following West syndrome. In the group of 15 children with porencephalic cysts, the lesion was always unilateral, but topography and extension varied. Eight children had circumscribed cysts involving the rolandic or parieto-occipital regions, and the outcome of epilepsy was favorable. Seven patients had large lesions involving the frontal lobe, associated with unilateral hemispheric atrophy in four cases. These four children had an unfavorable epilepsy outcome. In the 12 patients with periventricular leukomalacia, all premature, eleven had no seizure relapse after the spasms; one of these children had epilepsy. In the five full-term patients with bilateral and diffuse cerebral lesions, three children had epilepsy at the last observation. In children with leukomalacia and in patients with localized porencephalic lesions the outcome of epilepsy appears to be better than in patients with diffuse cerebral lesions or in children with extensive porencephalic cysts, particularly those involving the frontal lobe.
报告了32例由围产期损伤引起的症状性婴儿痉挛症患者的临床、神经放射学数据及癫痫结局。神经放射学检查显示局灶性和弥漫性脑损伤。15名儿童有脑穿通畸形病变,12名有脑室周围白质软化,5名有双侧弥漫性脑萎缩,其中1名儿童伴有大理石样状态,另1名伴有双侧脑回肥厚。8例患者(25%)在韦斯特综合征后发生癫痫。在15名有脑穿通畸形囊肿的儿童组中,病变总是单侧的,但部位和范围各不相同。8名儿童有局限于中央沟或顶枕区的囊肿,癫痫结局良好。7例患者有累及额叶的大病变,4例伴有单侧半球萎缩。这4名儿童癫痫结局不佳。在12例脑室周围白质软化的患者中,均为早产儿,11例在痉挛后无癫痫复发;其中1名儿童有癫痫。在5名有双侧弥漫性脑病变的足月儿患者中,3名儿童在最后一次观察时有癫痫。在有白质软化的儿童和有局限性脑穿通畸形病变的患者中,癫痫结局似乎比有弥漫性脑病变的患者或有广泛脑穿通畸形囊肿的儿童更好,尤其是那些累及额叶的患者。