Camfield Carol S, Camfield Peter R
Department of Pediatrics, IWK Health Centre, Halifax, Nova Scotia, Canada.
Neurology. 2009 Sep 29;73(13):1041-5. doi: 10.1212/WNL.0b013e3181b9c86f.
To document the long-term evolution of juvenile myoclonic epilepsy (JME) in a population-based cohort.
All patients developing JME by 16 years of age in Nova Scotia between 1977 and 1985 were contacted in 2006-2008.
Twenty-four patients (17 women) had JME, 3.5% of all childhood-onset epilepsy. Age at first seizure was 10.4 +/- 4.3 years. We contacted 23 of 24 (96%) at a mean age of 36 +/- 4.8 years. All were initially treated with antiepileptic drugs (AEDs). At the end of a 25.8 +/- 2.4-year follow-up, 11 (48%) had discontinued treatment: 6 were seizure-free (without AEDs for 5-23 years), 3 had myoclonic seizures only (without AEDs for >18 years), and 2 continued with rare seizures. Convulsive status epilepticus occurred in 8 (36%) and 3 had intractable epilepsy. About 70% reported good satisfaction with their health, work, friendships, and social life (Likert scales). Despite 87% high school graduation, 31% were unemployed. Sixteen live with a partner, 7 alone. Nine received antidepressant medications. Ten women had > or =1 pregnancy and 4 men fathered a child. Eleven pregnancies (80%) were unplanned, outside of a stable relationship. At least 1 major unfavorable social outcome was noted in 76%.
Our sample size is modest but the long follow-up and population-based sampling is unique. All seizure types in juvenile myoclonic epilepsy (JME) resolved in 17% and for 13%, only myoclonus persisted. Therefore, one-third of people with JME have troublesome seizures vanish and antiepileptic drug treatment is no longer needed. Depression, social isolation, unemployment, and social impulsiveness complicate the lives of many patients.
记录基于人群队列中青少年肌阵挛性癫痫（JME）的长期演变情况。
2006 - 2008年联系了1977年至1985年间在新斯科舍省16岁前发病的所有JME患者。
24例患者（17名女性）患有JME，占所有儿童期起病癫痫的3.5%。首次发作年龄为10.4±4.3岁。我们在平均年龄36±4.8岁时联系到了24例中的23例（96%）。所有患者最初均接受抗癫痫药物（AEDs）治疗。在25.8±2.4年的随访结束时，11例（48%）停止治疗：6例无癫痫发作（停用AEDs 5 - 23年），3例仅出现肌阵挛发作（停用AEDs超过18年），2例仍有罕见发作。8例（36%）发生惊厥性癫痫持续状态，3例患有难治性癫痫。约70%的患者对其健康、工作、友谊和社交生活表示高度满意（李克特量表）。尽管高中毕业率为87%，但31%的人失业。16人与伴侣同住，7人独自生活。9人接受抗抑郁药物治疗。10名女性有≥1次怀孕，4名男性育有子女。11次怀孕（80%）是计划外的，且并非处于稳定关系中。76%的患者至少出现1项重大不良社会结局。
我们的样本量适中，但长期随访和基于人群的抽样具有独特性。青少年肌阵挛性癫痫（JME）的所有发作类型在17%的患者中得到缓解，13%的患者仅肌阵挛持续存在。因此，三分之一的JME患者烦人的癫痫发作消失，不再需要抗癫痫药物治疗。抑郁、社会孤立、失业和社会冲动使许多患者的生活变得复杂。
