The natural history of seizures and neuropsychiatric symptoms in childhood epilepsy with centrotemporal spikes (CECTS).
The natural history of seizures and neuropsychiatric symptoms in childhood epilepsy with centrotemporal spikes (CECTS).
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DOI：
10.1016/j.yebeh.2019.07.038
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发表时间：
2020-02
期刊：
影响因子：
--
通讯作者：
Chu CJ
中科院分区：
文献类型：
--
作者：
Ross EE;Stoyell SM;Kramer MA;Berg AT;Chu CJ
儿童癫痫伴中央颞叶尖峰（CCECTS，正式名称为良性癫痫伴中央颞叶尖峰，BECTS）是一种常见的儿童癫痫综合征，其特征是精神、行为和认知异常以及自限性癫痫发作。尽管CECTS是最具特征的癫痫电临床综合征之一，但对神经精神结局的自然史知之甚少。我们报告了一项大型前瞻性入选CECTS儿童纵向队列的疾病过程中的精神病学、行为和认知概况。我们进一步描述了详细的癫痫发作过程，并测试了这些儿童中几种风险因素与神经精神和癫痫发作结果之间的关系。诊断为CECTS的患者作为社区研究的一部分被纳入，从诊断到疾病消退（16.0 +/ - 3.1年，N=60）。还招募了20名同胞作为对照。我们报告了发病前神经精神问题的自然史，发病后神经精神诊断，长期神经心理表现，癫痫病程，抗癫痫药物（ASM）治疗反应，以及无癫痫持续时间和缓解之间的关系。发病年龄和病前神经精神问题作为癫痫发作次数、癫痫持续时间、病后神经精神诊断和长期神经心理表现的预测因子进行了测试。ASM治疗时间、癫痫发作次数和癫痫持续时间作为病后神经精神诊断和长期神经心理表现的预测因子。患有CECTS的儿童在诊断前有高发生率的ADD/ADHD症状（18.3%）或学习困难（21.7%）。新的或持续的注意力缺陷多动障碍（20%）、情绪障碍（23.6%）、学习困难（14.5%）和行为障碍（7.3%）在CECTS诊断后很常见。在9年的随访中，正式神经心理测试的表现与人口统计和兄弟姐妹对照相当。超过三分之二的接受治疗的儿童在治疗期间至少经历过一次癫痫发作。大多数患儿（61.7%）在12个月无癫痫发作后进入缓解期。在所有儿童中，每一个月无癫痫发作，达到最终缓解的概率增加6-7% （p<1e-10）。发病前神经发育问题的存在预示着更长的癫痫持续时间（p=0.02），更高的癫痫发作次数（p=0.02），以及病后精神病学或神经发育诊断（p=0.002）。测试的特征都不能预测长期的神经心理表现。在CECTS中，儿童在病程中出现神经精神症状的风险很高，然而，长期的认知表现是有利的。大多数儿童在接受asm治疗时发生癫痫发作，这表明CECTS不像假设的那样具有药物反应性，或者治疗方法没有得到优化。在接受治疗和未接受治疗的儿童中，未来癫痫发作的风险可以从无癫痫发作的持续时间来估计。发病前神经精神问题的存在预示着CECTS患者更严重的病程。
Childhood epilepsy with centrotemporal spikes (CCECTS, formally benign epilepsy with centrotemporal spikes, BECTS) is a common childhood epilepsy syndrome characterized by psychiatric, behavioral, and cognitive abnormalities and self-limited seizures. Although CECTS is one of the most well-characterized electroclinical epilepsy syndromes, the natural history of neuropsychiatric outcomes is poorly understood. We report the psychiatric, behavioral, and cognitive profiles over the course of disease from a large, prospectively-enrolled, longitudinal cohort of children with CECTS. We further characterize the detailed seizure course and test the relationship between several proposed risk factors and neuropsychiatric and seizure outcomes in these children. Patients diagnosed with CECTS were enrolled as part of a community-based study and followed from diagnosis through disease resolution (16.0 +/− 3.1 yrs, N=60). 20 sibling controls were also recruited. We report the natural history of premorbid neuropsychiatric concerns, postmorbid neuropsychiatric diagnoses, long-term neuropsychological performance, seizure course, antiseizure medication (ASM) treatment response, and the relationship between duration seizure-free and remission. Age of onset and premorbid neuropsychiatric concerns were tested as predictors of seizure count, epilepsy duration, postmorbid neuropsychiatric diagnoses, and long-term neuropsychological performance. ASM treatment duration, seizure count, and epilepsy duration were tested as predictors of postmorbid neuropsychiatric diagnoses and long-term neuropsychological performance. Children with CECTS had a high incidence of ADD/ADHD symptoms (18.3%) or learning difficulties (21.7%) before diagnosis. New or persistent ADHD (20%), mood disorders (23.6%), learning difficulties (14.5%), and behavioral disorders (7.3%) were common after CECTS diagnosis. At 9-year follow-up, performance on formal neuropsychological testing was comparable to population statistics and sibling controls. More than two-thirds of treated children experienced at least one seizure during treatment. Most children (61.7%) had entered terminal resolution after 12 months seizure-free. Among all children, for each month seizure-free, there was a 6–7% increase in the probability of achieving terminal remission (p<1e-10). The presence of a premorbid neurodevelopmental concern predicted a longer epilepsy duration (p=0.02), higher seizure count (p=0.02), and a postmorbid psychiatric or neurodevelopmental diagnosis (p=0.002). None of the tested features predicted long-term neuropsychological performance. Children are at high risk of neuropsychiatric symptoms along the course of the disease in CECTS, however, long-term cognitive performance is favorable. The majority of children had a seizure while being treated with ASMs, suggesting that CECTS is not as pharmacoresponsive as assumed or that treatment approaches are not optimized. Among treated and untreated children, future seizure-risk can be estimated from duration seizure-free. The presence of a premorbid neuropsychiatric concern predicted a more severe disease course in CECTS.
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影响因子：
30.8
作者：
Carvill, Gemma L.;Regan, Brigid M.;Yendle, Simone C.;O'Roak, Brian J.;Lozovaya, Natalia;Bruneau, Nadine;Burnashev, Nail;Khan, Adiba;Cook, Joseph;Geraghty, Eileen;Sadleir, Lynette G.;Turner, Samantha J.;Tsai, Meng-Han;Webster, Richard;Ouvrier, Robert;Damiano, John A.;Berkovic, Samuel F.;Shendure, Jay;Hildebrand, Michael S.;Szepetowski, Pierre;Scheffer, Ingrid E.;Mefford, Heather C.
通讯作者：
Mefford, Heather C.
影响因子：
5.6
作者：
Besag, Frank M. C.
通讯作者：
Besag, Frank M. C.
影响因子：
2.2
作者：
Berg, AT;Lin, JX;Shinnar, S
通讯作者：
Shinnar, S
影响因子：
9.9
作者：
Camfield, Carol S.;Camfield, Peter R.
通讯作者：
Camfield, Peter R.
影响因子：
9.9
作者：
Bouma, PAD;Bovenkerk, AC;Brouwer, OF
通讯作者：
Brouwer, OF