Licchetta Laura, Bisulli Francesca, Vignatelli Luca, Zenesini Corrado, Di Vito Lidia, Mostacci Barbara, Rinaldi Claudia, Trippi Irene, Naldi Ilaria, Plazzi Giuseppe, Provini Federica, Tinuper Paolo
From IRCCS Istituto delle Scienze Neurologiche di Bologna (L.L., F.B., L.V., C.Z., B.M., G.P., F.P., P.T.) and Department of Biomedical and Neuromotor Sciences (L.L., F.B., L.D.V., C.R., I.T., I.N., G.P., F.P., P.T.), University of Bologna, Italy.
Neurology. 2017 Jan 3;88(1):70-77. doi: 10.1212/WNL.0000000000003459. Epub 2016 Nov 23.
To assess the long-term outcome of sleep-related hypermotor epilepsy (SHE).
We retrospectively reconstructed a representative cohort of patients diagnosed with SHE according to international diagnostic criteria, sleep-related seizures ≥75% and follow-up ≥5 years. Terminal remission (TR) was defined as a period of ≥5 consecutive years of seizure freedom at the last follow-up. We used Kaplan-Meier estimates to calculate the cumulative time-dependent probability of TR and to generate survival curves. Univariate and multivariate Cox regression analyses were performed.
We included 139 patients with a 16-year median follow-up (2,414 person-years). The mean age at onset was 13 ± 10 years. SHE was sporadic in 86% of cases and familial in 14%; 16% of patients had underlying brain abnormalities. Forty-five percent of patients had at least 1 seizure in wakefulness lifetime and 55% had seizures only in sleep (typical SHE). At the last assessment, 31 patients achieved TR (TR group, 22.3%), while 108 (NTR group, 77.7%) still had seizures or had been in remission for <5 years. The cumulative TR rate was 20.4%, 23.5%, and 28.4% by 10, 20, and 30 years from inclusion. At univariate analysis, any underlying brain disorder (any combination of intellectual disability, perinatal insult, pathologic neurologic examination, and brain structural abnormalities) and seizures in wakefulness were more frequent among the NTR group (p = 0.028; p = 0.043). Absence of any underlying brain disorder (hazard ratio 4.21, 95% confidence interval 1.26-14.05, p = 0.020) and typical SHE (hazard ratio 2.76, 95% confidence interval 1.31-5.85, p = 0.008) were associated with TR.
Our data show a poor prognosis of SHE after a long-term follow-up. Its outcome is primarily a function of the underlying etiology.
评估睡眠相关性运动过多型癫痫（SHE）的长期预后。
我们根据国际诊断标准，回顾性重建了一个具有代表性的被诊断为SHE的患者队列，睡眠相关性癫痫发作≥75%且随访≥5年。终末期缓解（TR）定义为在最后一次随访时连续≥5年无癫痫发作。我们使用Kaplan-Meier估计来计算TR的累积时间依赖性概率并生成生存曲线。进行单因素和多因素Cox回归分析。
我们纳入了139例患者，中位随访时间为16年（2414人年）。发病时的平均年龄为13±10岁。86%的SHE病例为散发性，14%为家族性；16%的患者有潜在的脑部异常。45%的患者在清醒期至少有1次癫痫发作，55%的患者仅在睡眠中发作（典型SHE）。在最后一次评估时，31例患者实现了TR（TR组，22.3%），而108例（非TR组，77.7%）仍有癫痫发作或缓解时间<5年。从纳入起10年、20年和30年时的累积TR率分别为20.4%、23.5%和28.4%。在单因素分析中，非TR组中任何潜在的脑部疾病（智力残疾、围产期损伤、病理性神经系统检查和脑结构异常的任何组合）和清醒期癫痫发作更为常见（p = 0.028；p = 0.043）。无任何潜在脑部疾病（风险比4.21，95%置信区间1.26 - 14.05，p = 0.020）和典型SHE（风险比2.76，95%置信区间1.31 - 5.85，p = 0.008）与TR相关。
我们的数据显示，长期随访后SHE的预后较差。其预后主要取决于潜在病因。
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