Long-term outcomes of childhood-onset epilepsy

描述（由申请人提供）：癫痫影响1-2%的人口。所有新病例中有一半发生在婴儿期至青春期早期。癫痫发作的结果多种多样。癫痫可以是一种终生难治性疾病，也可以在几次癫痫发作后消退。然而，许多人在达到持久缓解之前的头几年会经历反复的缓解-复发过程。与评估、治疗、管理、咨询、干预和潜在的预防相关的两个问题知之甚少：1）超过一半的手术治疗的难治性癫痫成年人患有儿童发作的癫痫，然而，前瞻性研究发现很少有儿童患有手术治疗的难治性癫痫。2)在其他神经系统正常的儿童期癫痫患者中，其中许多人处于缓解期和停药期;相对于非癫痫对照组，教育和社会结局受损。这两个观察结果的解释在现存文献中并不明显。首先，数据仅来自难治性患者的回顾性分析。从发病到成年手术的儿童数据很少。其次，报告长期社会和教育结果的研究仅描述了多年后的最终癫痫发作结果，因此忽略了演变超过10年的通常复杂的缓解-复发过程以及从早期存在的行为和精神共病，甚至早于癫痫发作。康涅狄格州研究是一项正在进行的前瞻性队列研究，招募了613名首次诊断为癫痫的儿童（最初为1个月至15岁）（1993年至1997年，中位随访约10年）。随着队列进入青春期后期/成年早期，我们将进行两方面的研究：1）海马疾病的存在和进展，其频率和确定最有可能发展为局灶性癫痫患者的因素; 2）癫痫发作结果与精神、行为、社会、教育和健康相关生活质量结果的相关性，以及评估精神和行为状况在多大程度上解释了社会和教育结果，而与癫痫发作变量无关。康涅狄格州的研究是唯一适合解决这些问题的后果，儿童发作癫痫在年轻的成年人。海马体积测定、体积变化和T2弛豫测定的检查将提供关于最常见的手术治疗癫痫的自然史、确定和早期检测的第一个前瞻性信息。

DESCRIPTION (provided by applicant): Epilepsy affects 1-2% of the population. Half of all new cases arise in infancy through early adolescence. Seizure outcomes are highly varied. Epilepsy can be a life-long intractable disorder or may resolve after only a few seizures. Many individuals, however, follow a repeated remitting-relapsing course for the first several years before attaining lasting remission. Two issues relevant to evaluation, treatment, management, counseling, intervention, and potentially prevention are poorly understood: 1) More than half the adults with intractable epilepsy who are treated surgically have epilepsy of childhood onset, yet, prospective studies find few children with surgically treatable intractable epilepsy. 2) In otherwise neurologically normal adults with childhood onset epilepsy, many of whom are in remission and off medication; educational and social outcomes are impaired relative to non-epilepsy controls. Explanations for both observations are not evident in the extant literature. Firstly, data come from retrospective analyses of refractory patients only. Few data exist from children followed from onset to surgery in adulthood. Secondly, studies reporting long-term social and educational outcomes characterize only final seizure outcomes after many years thus ignoring an often complex remitting-relapsing process that evolves over >10 years as well as behavioral and psychiatric co- morbidity present from an early age, even predating the seizures. The Connecticut study is an ongoing prospective cohort of 613 children (initially 1 mo - 15 yr) recruited when first diagnosed with epilepsy (1993- 7, median follow-up ~10 years). As the cohort matures into late adolescence/early adulthood, we will pursue two lines of investigation: 1) Presence and progression of hippocampal disease, its frequency and factors that identify patients with focal epilepsy most likely to develop it; 2) Correlation of seizure outcomes with psychiatric, behavioral, social, educational, and health-related quality of life outcomes, and an assessment of the extent to which psychiatric and behavioral conditions, independent of seizure variables, explain social and educational outcomes. The Connecticut study is uniquely suited to address these questions concerning the consequences of childhood onset epilepsy in young adults. The examination of hippocampal volumetry, changes in volume and T2 relaxometry will provide the first prospective information about the natural history, determinates, and early detection of the most common form of surgically treated epilepsy.