Prognosis of juvenile myoclonic epilepsy 45 years after onset
Prognosis of juvenile myoclonic epilepsy 45 years after onset
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DOI：
10.1212/01.wnl.0000437303.36064.f8
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发表时间：
2013-12-10
期刊：
影响因子：
9.9
通讯作者：
Janz, Dieter
中科院分区：
文献类型：
--
作者：
Senf, Philine;Schmitz, Bettina;Janz, Dieter
目的：青少年肌阵挛癫痫(JME)是最常见的特发性全身性癫痫亚综合征，约占青少年和成人癫痫病例的3%~11%。然而，对这种临床实体的长期医学演变知之甚少。这项研究的目的是分析临床上明确的JME患者癫痫发作演变的长期结果和癫痫发作结果的预测因素。方法：在这项回顾性队列研究中，我们分析了66名JME患者的癫痫结果，这些患者在柏林Charite大学神经科接受治疗，并由一位资深癫痫专家初步诊断。结果：经过平均44.6年(20-69年)的随访，591%的患者在最后一次接触前至少5年没有癫痫发作。在无癫痫发作的患者中，28例(71.8%)仍在服用抗癫痫药物，11例(28.2%)至少在最近5年内停药。我们确定JME发作时出现额外的失神发作是一个独立的预测不良结局的指标。结论：相当大比例的JME患者在其疾病的后期没有癫痫发作和停用抗癫痫药物治疗。有JME和额外失神发作的患者可能代表不同的JME亚型，结果更差。
Objectives: Juvenile myoclonic epilepsy (JME) is the most common idiopathic generalized epilepsy subsyndrome, contributing to approximately 3% to 11% of adolescent and adult cases of epilepsy. However, little is known about the long-term medical evolution of this clinical entity. The aim of this study was to analyze long-term outcome in a clinically well-defined series of patients with JME for seizure evolution and predictors of seizure outcome.Methods: In this retrospective cohort study, we analyzed seizure outcome in 66 patients who had JME, were treated at the Department of Neurology, Charite-Universitatsmedizin Berlin, and were initially diagnosed by a single senior epileptologist.Results: After a mean follow-up time of 44.6 years (20-69 years), 59.1% of patients remained free of seizures for at least 5 years before the last contact. Among the seizure-free patients, 28 (71.8%) were still taking antiepileptic drugs and 11 (28.2%) were off medication for at least the last 5 years. We identified manifestation of additional absence seizures at onset of JME as an independent predictor of an unfavorable outcome regarding seizure freedom.Conclusions: A significant proportion of patients with JME were seizure-free and off antiepileptic drug therapy in the later course of their disorder. Patients with JME and additional absence seizures might represent a different JME subtype with a worse outcome.