Servei de Neurologia, Hospital de Sant Boi, Sant Boi de Llobregat, Barcelona, Spain.
Neurologia. 2011 May;26(4):193-9. doi: 10.1016/j.nrl.2010.09.005. Epub 2010 Nov 13.
Juvenile absence epilepsy (JAE) is a generalized form of epilepsy, characterized by absence seizures (AS) initiated in adolescence, with a typical EEG showing generalized spike-wave discharges. Apart from absences, other seizure types may be observed such as myoclonia and generalized tonic-clonic seizures (GTCS). Its long-term prognosis is uncertain.
We retrospectively selected all patients who met the 1989 ILAE diagnostic criteria for JAE. We analysed clinical variables, pharmacological treatment, and seizure remission with medical treatment and seizure relapse after stopping medical treatment.
We identified 21 patients, 17 women and 4 men, 86% of whom had suffered GTCS and 14% myoclonias. Mean age at AS onset was 17 years old (range 10-44), 4 patients debuted with AS in adulthood. Mean follow up duration was 25 years (range 10-43). Ninety per cent of the patients were treated with valproate and 62% needed polytherapy. Currently 43% have achieved seizure freedom under medical treatment. All attempts to stop treatment failed, in some cases after long periods of seizure remission.
Less than fifty per cent of patients with JAE achieve remission, antiepileptic treatment is mandatory during all life, despite having long periods of remission.
青少年失神癫痫（JAE）是一种全身性癫痫，以青少年期起病的失神发作（AS）为特征，典型脑电图表现为全面性棘慢波放电。除了失神发作外，还可能观察到其他发作类型，如肌阵挛和全面性强直-阵挛发作（GTCS）。其长期预后不确定。
我们回顾性选择了所有符合 1989 年 ILAE 诊断标准的 JAE 患者。我们分析了临床变量、药物治疗以及药物治疗后缓解和停止治疗后复发的情况。
我们共确定了 21 例患者，其中女性 17 例，男性 4 例，86%的患者有 GTCS，14%的患者有肌阵挛。AS 发作的平均年龄为 17 岁（范围 10-44 岁），4 例患者在成年期出现 AS 发作。平均随访时间为 25 年（范围 10-43 年）。90%的患者接受丙戊酸钠治疗，62%的患者需要联合治疗。目前，43%的患者在药物治疗下实现了无发作。所有停止治疗的尝试均失败，在某些情况下，经过长时间的无发作缓解后仍复发。
青少年失神癫痫患者中不到 50%实现缓解，抗癫痫治疗在整个生命周期中都是必要的，尽管有长时间的缓解期。
