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左乙拉西坦治疗眼睑肌阵挛伴失神发作(Jeavons综合征)的一项试点试验。

A pilot trial of levetiracetam in eyelid myoclonia with absences (Jeavons syndrome).

作者信息

Striano Pasquale, Sofia Vito, Capovilla Giuseppe, Rubboli Guido, Di Bonaventura Carlo, Coppola Antonietta, Vitale Giuseppina, Fontanillas Luis, Giallonardo Anna Teresa, Biondi Roberto, Romeo Antonino, Viri Maurizio, Zara Federico, Striano Salvatore

机构信息

Epilepsy Center, Department of Neurological Sciences, Federico II University, Napoli, Italy.

出版信息

Epilepsia. 2008 Mar;49(3):425-30. doi: 10.1111/j.1528-1167.2007.01524.x. Epub 2008 Jan 29.

Abstract

OBJECTIVE

Eyelid myoclonia with absences (EMA) or Jeavons syndrome characterized by eyelid myoclonia (EM) (with or without absences), eye closure-induced EEG paroxysms, and photosensitivity. We conducted an open-label trial of levetiracetam in EMA.

PATIENTS AND METHODS

Patients were recruited in different Italian Epilepsy Centres. Levetiracetam was administrated at starting dose of 10 mg/kg/day up to 50-60 mg/kg/day in two doses. Treatment period included a 5-6 week up-titration phase and a 12-week evaluation phase. The number of days with EM (i.e., days with seizures, DwS) and number of generalized tonic-clonic seizures (GTCS) were evaluated. Analysis of intent-to-treat population was performed using Fisher's and Wilcoxon tests.

RESULTS

Thirty-five patients (23 F) with a mean age of 19 +/- 6 years were recruited. Twenty-seven had previously undergone one to five adequate trials of antiepileptic drugs. The median number of DwS/month was 12 +/- 8.2. Twenty-one patients experienced GTCS (median number/month: 1 +/- 0.2). Thirty-four subjects completed the trial. Levetiracetam was well tolerated (mean dose: 1985 mg/day). Responders were 28/35 (80%) patients, nine taking levetiracetam as monotherapy. Six patients were seizure-free, 15 had > or =75% and seven >50% seizure reduction. GTCS remitted in 14 out of 21 (66.6%) patients. The number/month of DwS (median: 12 vs 5; p = 0.0001) and of GTCS (median: 1 vs 0; p = 0.0001) decreased compared to baseline period. Disappearance or clear reduction in paroxysmal abnormalities at eye closure occurred in 20 of the responders and photoparoxysmal response in 19. Mean follow-up was 23.9 +/- 18.5 months.

CONCLUSION

Levetiracetam is effective and well tolerated in EMA. Placebo-controlled studies should confirm these findings.

摘要

目的

眼睑肌阵挛伴失神发作(EMA)或Jeavons综合征,其特征为眼睑肌阵挛(EM)(伴或不伴失神发作)、闭眼诱发的脑电图阵发性异常及光敏感性。我们开展了一项左乙拉西坦治疗EMA的开放标签试验。

患者与方法

患者在意大利不同的癫痫中心招募。左乙拉西坦起始剂量为10mg/kg/天,分两次给药,最大剂量可达50 - 60mg/kg/天。治疗期包括一个5 - 6周的剂量递增阶段和一个12周的评估阶段。评估肌阵挛发作天数(即癫痫发作天数,DwS)和全面强直阵挛发作(GTCS)次数。采用Fisher检验和Wilcoxon检验对意向性治疗人群进行分析。

结果

招募了35例患者(23例女性),平均年龄19±6岁。27例患者此前接受过1至5次充分的抗癫痫药物试验。每月DwS中位数为12±8.2。21例患者出现GTCS(每月中位数:1±0.2)。34例受试者完成试验。左乙拉西坦耐受性良好(平均剂量:1985mg/天)。28/35(80%)例患者有反应,9例将左乙拉西坦作为单药治疗。6例患者无癫痫发作,15例发作减少≥75%,7例发作减少>50%。21例患者中有14例(66.6%)GTCS缓解。与基线期相比,每月DwS(中位数:12对5;p = 0.0001)和GTCS(中位数:1对0;p = 0.0001)次数减少。20例有反应者闭眼时阵发性异常消失或明显减轻;19例光阵发性反应减轻。平均随访23.9±18.5个月。

结论

左乙拉西坦治疗EMA有效且耐受性良好。安慰剂对照研究应证实这些发现。

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