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Epilepsy syndromes in cerebral palsy: varied, evolving and mostly self-limited
Brain ( IF 14.5 ) Pub Date : 2022-07-24 , DOI: 10.1093/brain/awac274
Monica S Cooper 1, 2, 3 , Mark T Mackay 1, 2, 3 , Charuta Dagia 1, 2 , Michael C Fahey 4 , Katherine B Howell 1, 2, 3 , Dinah Reddihough 1, 2, 3 , Susan Reid 1, 2, 3 , A Simon Harvey 1, 2, 3
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Seizures occur in approximately one third of children with cerebral palsy. This study aimed to determine epilepsy syndromes in children with seizures and cerebral palsy due to vascular injury, anticipating that this would inform treatment and prognosis. We studied a population-based cohort of children with cerebral palsy due to prenatal or perinatal vascular injuries, born 1999-2006. Each child’s MRI was reviewed to characterise patterns of grey and white matter injury. Children with syndromic or likely genetic causes of cerebral palsy were excluded, given their inherent association with epilepsy and our aim to study a homogeneous cohort of classical cerebral palsy. Chart review, parent interview and EEGs were used to determine epilepsy syndromes and seizure outcomes. Of 256 children, 93 (36%) had one or more febrile or afebrile seizures beyond the neonatal period and 87 (34%) had epilepsy. Children with seizures were more likely to have had neonatal seizures, have spastic quadriplegic cerebral palsy, and function within Gross Motor Function Classification System level IV or V. Fifty-six (60%) children with seizures had electroclinical features of a self-limited focal epilepsy of childhood (SeLFE); we diagnosed these children with a SeLFE-variant given the current ILAE classification precludes a diagnosis of SeLFE in children with a brain lesion. Other epilepsy syndromes were focal epilepsy - not otherwise specified in 28, infantile spasms syndrome in 11, Lennox-Gastaut syndrome in three, genetic generalised epilepsies in two, and febrile seizures in nine. No epilepsy syndrome could be assigned in seven children with no EEG. Twenty-one changed syndrome classification during childhood. SeLFE-variant usually manifested with a mix of autonomic and brachio-facial motor features, and occipital and/or centro-temporal spikes on EEG. Of those with SeLFE-variant, 42/56 (75%) had not had a seizure for >2 years. Favourable seizure outcomes were also seen in some children with infantile spasms syndrome and focal epilepsy not otherwise specified. Of the 93 children with seizures, at last follow-up (mean age 15 years), 61/91 (67%) had not had a seizure in >2 years. Children with cerebral palsy and seizures can be assigned specific epilepsy syndrome diagnoses typically reserved for normally developing children, those syndromes commonly being age-dependent and self-limited. Compared to typically developing children with epilepsy, SeLFE-variant occurs much more commonly in children with cerebral palsy and epilepsy. These findings have important implications for treatment and prognosis of epilepsy in cerebral palsy, and research into pathogenesis of SeLFE.

中文翻译:

脑瘫的癫痫综合征:变化多端、不断发展且大多为自限性

大约三分之一的脑瘫儿童会出现癫痫发作。本研究旨在确定因血管损伤导致癫痫发作和脑瘫的儿童的癫痫综合征,预计这将为治疗和预后提供信息。我们研究了 1999 年至 2006 年出生的因产前或围产期血管损伤导致脑瘫儿童的人群队列。审查了每个孩子的 MRI 以表征灰质和白质损伤的模式。考虑到他们与癫痫的内在关联以及我们的目标是研究经典脑瘫的同质队列,患有脑瘫综合征或可能遗传原因的儿童被排除在外。图表审查、家长访谈和脑电图用于确定癫痫综合征和癫痫发作结果。在 256 名儿童中,93 人 (36%) 在新生儿期后出现过一次或多次发热或无热性惊厥,87 人 (34%) 患有癫痫。癫痫发作的儿童更可能有新生儿癫痫发作、痉挛性四肢瘫痪脑瘫以及在粗大运动功能分类系统 IV 级或 V 级内的功能。56 名 (60%) 癫痫发作儿童具有自限性局灶性电临床特征儿童癫痫症 (SeLFE);鉴于目前的 ILAE 分类排除了脑部病变儿童的 SeLFE 诊断,我们诊断出这些儿童患有 SeLFE 变异。其他癫痫综合征包括局灶性癫痫 - 未另行说明的 28 例、婴儿痉挛综合征 11 例、Lennox-Gastaut 综合征 3 例、遗传性全身性癫痫 2 例、热性惊厥 9 例。七名没有脑电图的儿童不能确定为癫痫综合征。21 人在童年时期改变了证候分类。SeLFE 变异通常表现为混合的自主神经和臂面部运动特征,以及脑电图上的枕骨和/或中央颞区尖峰。在具有 SeLFE 变体的那些人中,42/56 (75%) 没有癫痫发作超过 2 年。在一些患有婴儿痉挛综合征和局灶性癫痫的儿童中也观察到了良好的癫痫发作结果。在最后一次随访(平均年龄 15 岁)的 93 名癫痫发作儿童中,61/91(67%)在>2 年内没有发作过。患有脑瘫和癫痫发作的儿童可以被指定为特定的癫痫综合征诊断,通常是为正常发育的儿童保留的,这些综合征通常是年龄依赖性和自限性的。与正常发育的癫痫儿童相比,SeLFE 变异更常见于患有脑瘫和癫痫的儿童。这些发现对脑瘫癫痫的治疗和预后以及SeLFE的发病机制研究具有重要意义。
更新日期:2022-07-24
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