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青少年起病的全面性遗传性癫痫的长期预后。

Long-term outcome in adolescent-onset generalized genetic epilepsies.

机构信息

Department of Neurology, Epilepsy-Center Berlin-Brandenburg, Charité - University Medicine Berlin, Berlin, Germany.

出版信息

Epilepsia. 2017 Jul;58(7):1244-1250. doi: 10.1111/epi.13761. Epub 2017 May 2.

Abstract

OBJECTIVE

Until now, it has been unclear if the three subsyndromes of adolescent-onset generalized genetic epilepsy (GGE) differ in long-term prognosis. Therefore, this study aimed to compare long-term seizure outcome in juvenile absence epilepsy (JAE), juvenile myoclonic epilepsy (JME), and epilepsy with generalized tonic-clonic seizures alone (EGTCS).

METHODS

This retrospective study is based on the archive of an institutional tertiary care outpatient clinic for adult patients with epilepsy. Charts of 870 epilepsy outpatients were reviewed among whom 176 had adolescent-onset GGE (53 JAE, 66 JME, 57 EGTCS). Median patient age at investigation was 60 years; median follow-up time was 42.5 years. If possible, GGE patients were additionally interviewed on psychosocial and clinical variables.

RESULTS

Age at first seizure was significantly higher in EGTCS patients (median 18 years) than in patients with JAE or JME (14 years each; p ≤ 0.001). Long-term seizure outcome hardly differed between the three subsyndromes. At the end of follow-up, 60% of all patients were in 5-year terminal seizure remission, and in 14%, epilepsy even had resolved (>10 years without seizures, >5 years without pharmacotherapy). Twenty percent of patients had persistent seizures during the last year of follow-up. Across all patients, 23% reported a psychiatric comorbidity, 87% had married, and 57% had achieved university entrance qualification.

SIGNIFICANCE

Long-term outcome was shown to be highly similar across all subsyndromes of adolescent-onset GGE. Even in a selection of difficult-to-treat epilepsy patients still attending an adult epilepsy clinic, most become seizure-free. To confirm these findings, prospective studies are needed.

摘要

目的

目前尚不清楚青少年起病的全面性遗传癫痫(GGE)的三个亚综合征在长期预后方面是否存在差异。因此,本研究旨在比较青少年失神癫痫(JAE)、青少年肌阵挛癫痫(JME)和单纯全面性强直-阵挛发作癫痫(EGTCS)的长期癫痫发作结局。

方法

这是一项回顾性研究,基于一个成人癫痫门诊的档案。在 870 名癫痫门诊患者中,有 176 名患有青少年起病的 GGE(53 例 JAE、66 例 JME、57 例 EGTCS)。研究时患者的中位年龄为 60 岁;中位随访时间为 42.5 年。如果可能,还会对 GGE 患者进行额外的心理社会和临床变量访谈。

结果

EGTCS 患者的首次癫痫发作年龄明显高于 JAE 或 JME 患者(分别为 18 岁和 14 岁;p≤0.001)。三种亚综合征之间的长期癫痫发作结局几乎没有差异。在随访结束时,所有患者中有 60%处于 5 年无癫痫发作期,14%的患者癫痫已经缓解(>10 年无癫痫发作,>5 年无药物治疗)。20%的患者在随访的最后一年仍有持续性癫痫发作。在所有患者中,23%报告有精神共病,87%已婚,57%达到了大学入学资格。

意义

研究结果表明,青少年起病的 GGE 的所有亚综合征的长期预后高度相似。即使在接受成人癫痫门诊治疗的一组难治性癫痫患者中,大多数患者也能无癫痫发作。为了证实这些发现,还需要进行前瞻性研究。

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