Pérez-Jiménez A, Colamaria V, Franco A, Grimau-Merino R, Darra F, Fontana E, Zullini E, Beltramello A, Dalla-Bernardina B
Servicio de Neuropsiquiatría Infantil, Hospital Policlínico, Universidad de Verona, Italia.
Rev Neurol. 1998 Jan;26(149):42-9.
Neuroimaging and experimental studies have related cytomegalovirus (CMV) to certain neuronal migration disorders.
To define the electroclinical picture of children with epilepsy associated with disorders of cortical development (DCD) and congenital CMV infection, we conducted a clinical, electroencephalographic and neuroradiological study of 10 children with this condition.
Eighty per cent of them had dismorphic traits, or malformations outside CNS. All showed other neuroradiological signs (cerebral calcification, white matter damage, porencephaly). Six patients with bihemispheric DCD (agyria-pachigyria, 2; 'poligyria', 1; schizencephaly, 1; bilateral opercular DCD, 2) showed: Tetraparesis, severe or profound mental deficiency, early onset epilepsy (mean age at onset: 11 months) with spasms, tonic seizures, partial seizures, and multifocal paroxysms or unusual diffuse sharp Alfa-Beta EEG activity. One child developed Epilepsia Partialis Continua. Children with bilateral opercular DCD evolved to a continuous spike and wave (SW) electrical status during wakefulness and sleep, linked to a worsening of psychomotor derangement. Four patients with unilateral hemispheric DCD (pachigyric or 'poligyric') showed: Congenital hemiparesis, mild intellectual deficiency, motor seizures (orofacial, hemiclonic, generalized) beginning in the third year of live, atypical absences with focal phenomena, frequent focal rhythmic SW discharges during wakefulness, and continuous SW status during sleep (CSWS).
A wide spectrum of DCD due to congenital CMV infection is documented. Characteristic electroclinical pictures related to the extent and topographical distribution of the DCD are recognized, which may lead to an appropriate diagnosis and prognosis.
神经影像学和实验研究已将巨细胞病毒(CMV)与某些神经元迁移障碍联系起来。
为明确患有与皮质发育障碍(DCD)及先天性CMV感染相关癫痫的儿童的电临床特征,我们对10例患有此病的儿童进行了临床、脑电图及神经放射学研究。
其中80%有畸形特征或中枢神经系统外畸形。所有患儿均有其他神经放射学征象(脑钙化、白质损害、脑穿通畸形)。6例双侧DCD患儿(无脑回-厚脑回,2例;“多脑回”,1例;脑裂畸形,1例;双侧岛盖部DCD,2例)表现为:四肢瘫、重度或极重度智力缺陷、早发性癫痫(平均发病年龄:11个月),伴有痉挛、强直发作、部分性发作及多灶性阵发或异常弥漫性锐慢波脑电图活动。1例患儿发展为持续性部分性癫痫。双侧岛盖部DCD患儿在清醒和睡眠时演变为持续性棘慢波(SW)电状态,与精神运动障碍加重相关。4例单侧半球DCD患儿(厚脑回或“多脑回”)表现为:先天性偏瘫、轻度智力缺陷、运动性发作(口面部、半侧阵挛性、全身性)始于生命第三年、伴有局灶现象的非典型失神、清醒时频繁的局灶性节律性SW放电及睡眠时持续性SW状态(CSWS)。
记录了先天性CMV感染所致的广泛谱系的DCD。识别出与DCD范围和部位分布相关的特征性电临床特征,这可能有助于做出恰当的诊断和预后判断。