Grocott Olivia R, Herrington Katherine S, Pfeifer Heidi H, Thiele Elizabeth A, Thibert Ronald L
Angelman Syndrome Clinic and Center for Dietary Therapy of Epilepsy, Massachusetts General Hospital, Boston, MA, United States.
Angelman Syndrome Clinic and Center for Dietary Therapy of Epilepsy, Massachusetts General Hospital, Boston, MA, United States.
Epilepsy Behav. 2017 Mar;68:45-50. doi: 10.1016/j.yebeh.2016.12.018. Epub 2017 Jan 19.
The low glycemic index treatment, a dietary therapy that focuses on glycemic index and reduced carbohydrate intake, has been successful in reducing seizure frequency in the general epilepsy population. Epilepsy is a common feature of Angelman syndrome and seizures are often refractory to multiple medications, especially in those with maternal deletions. Dietary therapy has become a more frequently used option for treating epilepsy, often in combination with other antiepileptic drugs, due to its efficacy and favorable side effect profile. This study aimed to assess the effectiveness of the low glycemic index treatment for seizure control in Angelman syndrome. Through a retrospective medical record review of 23 subjects who utilized the low glycemic index treatment at the Clinic and Center for Dietary Therapy of Epilepsy at the Massachusetts General Hospital, we found that the high level of seizure control and favorable side effect profile make the low glycemic index treatment a viable treatment for seizures in Angelman syndrome. The majority of subjects in our cohort experienced some level of seizure reduction after initiating the diet, 5 (22%) maintained complete seizure freedom, 10 (43%) maintained seizure freedom except in the setting of illness or non-convulsive status epilepticus, 7 (30%) had a decrease in seizure frequency, and only 1 (4%) did not have enough information to determine seizure control post-initiation. The low glycemic index treatment monotherapy was successful for some subjects in our cohort but most subjects used an antiepileptic drug concurrently. Some subjects were able to maintain the same level of seizure control on a liberalized version of the low glycemic index treatment which included a larger amount of low glycemic carbohydrates. No correlation between the level of carbohydrate restriction and level of seizure control was found. Few subjects experienced side effects and those that did found them to be mild and easily treated. The efficacy of the low glycemic index treatment and its favorable side effect profile make it an excellent alternative or supplement to antiepileptic drug therapy for the treatment of seizures in Angelman syndrome.
低血糖指数疗法是一种注重血糖指数并减少碳水化合物摄入量的饮食疗法，已成功降低了普通癫痫人群的癫痫发作频率。癫痫是天使综合征的常见特征，癫痫发作往往对多种药物难治，尤其是那些有母系缺失的患者。由于其疗效和良好的副作用，饮食疗法已成为治疗癫痫更常用的选择，通常与其他抗癫痫药物联合使用。本研究旨在评估低血糖指数疗法对天使综合征癫痫控制的有效性。通过对在马萨诸塞州总医院癫痫饮食治疗诊所和中心采用低血糖指数疗法的23名受试者进行回顾性病历审查，我们发现高水平的癫痫控制和良好的副作用使低血糖指数疗法成为天使综合征癫痫发作的可行治疗方法。我们队列中的大多数受试者在开始饮食后癫痫发作有一定程度的减少，5名（22%）维持完全无癫痫发作，10名（43%）除生病或非惊厥性癫痫持续状态外维持无癫痫发作，7名（30%）癫痫发作频率降低，只有1名（4%）没有足够信息来确定开始治疗后癫痫的控制情况。低血糖指数疗法单一疗法对我们队列中的一些受试者是成功的，但大多数受试者同时使用抗癫痫药物。一些受试者能够在低血糖指数疗法的宽松版本（包括大量低血糖碳水化合物）下维持相同水平的癫痫控制。未发现碳水化合物限制水平与癫痫控制水平之间存在相关性。很少有受试者出现副作用，即使出现也发现很轻微且易于治疗。低血糖指数疗法的疗效及其良好的副作用使其成为天使综合征癫痫治疗中抗癫痫药物治疗的极佳替代或补充方法。
