Pediatric Epilepsy Program, Department of Neurology, Massachusetts General Hospital, Boston, MA 02114, USA.
Epilepsy Res. 2013 May;104(3):269-74. doi: 10.1016/j.eplepsyres.2012.10.010. Epub 2012 Dec 3.
Clobazam (CLB) was recently approved by the FDA, but has not been evaluated in tuberous sclerosis complex (TSC). We retrospectively reviewed a cohort of patients with TSC and refractory epilepsy who started CLB over a 5-year period. Clinical characteristics and number of tubers on MRI were assessed. Duration of therapy, therapeutic response and adverse events were recorded. CLB was prescribed in 29 adults and children of whom 72% were cognitively impaired, with a median age at seizure onset of 5 months. Mean duration of CLB therapy was 17.3 months with a 12 and 24-month estimated retention rate of 82% and 68%, respectively. Twenty patients (69%) reported a good response (>50% seizure reduction) at the end of the titration, and six patients (21%) remained good responders after 12 months of CLB therapy. Adverse events occurred in 13 patients, predominantly somnolence and behavioral disorders. One quarter of the responders reported improvement in behavior. No predictive factor for a good response could be identified. CLB appears to be a well-tolerated and valuable option for treatment of refractory epilepsy in TSC.
氯巴占（CLB）最近获得了 FDA 的批准，但尚未在结节性硬化症复合征（TSC）中进行评估。我们回顾性分析了在 5 年内开始使用 CLB 的一组患有 TSC 和耐药性癫痫的患者。评估了临床特征和 MRI 上的结节数量。记录了治疗持续时间、治疗反应和不良反应。CLB 被开给了 29 名成年人和儿童，其中 72%存在认知障碍，癫痫发作的中位年龄为 5 个月。CLB 治疗的平均持续时间为 17.3 个月，12 个月和 24 个月的估计保留率分别为 82%和 68%。20 名患者（69%）在滴定结束时报告了良好的反应（>50%的癫痫发作减少），6 名患者（21%）在 CLB 治疗 12 个月后仍保持良好反应。13 名患者出现不良反应，主要为嗜睡和行为障碍。四分之一的反应者报告行为改善。未能确定良好反应的预测因素。CLB 似乎是治疗 TSC 耐药性癫痫的一种耐受良好且有价值的选择。
