Department of Neurology, Pediatric Epilepsy Program and Angelman Syndrome Clinic, Massachusetts General Hospital, Boston, Massachusetts, USA.
Epilepsia. 2012 Sep;53(9):1498-502. doi: 10.1111/j.1528-1167.2012.03537.x. Epub 2012 Jul 10.
The low glycemic index treatment (LGIT) is a high fat, limited carbohydrate diet used in the treatment of epilepsy. The purpose of this study was to assess the efficacy and tolerability of the LGIT for the treatment of refractory seizures in pediatric patients with Angelman syndrome.
A pediatric Angelman syndrome cohort with refractory epilepsy was treated with the LGIT and followed prospectively over 4 months. Parents recorded a daily seizure log for a minimum of 1 month prior to the start of treatment as well as throughout the LGIT trial. Electroencephalography (EEG) and neuropsychological assessments (Scales of Independent Behavior-Revised and the Vineland Adaptive Behavior Scales-2nd Edition were obtained for each subject at both baseline and 4-month follow-up time points. Clinical evaluations of subjects were completed by a neurologist and dietitian at the time of enrollment, as well as following both the first and fourth months of dietary therapy. At each time point, blood for laboratory chemistries was drawn and anthropometric measures were obtained.
Six children (mean age 3.3 years, range 1.1-4.8) with genetically confirmed Angelman syndrome initiated the LGIT, and completed the trial with no significant adverse events. Cohort averages for indices of seizure severity were as follows: age of 1.6 years at seizure onset, 3 lifetime antiepileptic drugs tried (range 1-6), and baseline seizure frequency of 10.1 events/week (range: 0.4-30.9). All subjects had a decrease in seizure frequency on the LGIT, with five of six exhibiting >80% seizure frequency reduction. All posttrial EEG studies showed improvement and three of four children with epileptiform activity on his or her baseline EEG had no discharges present on follow-up EEG. Developmental gains were noted by parents in all cases, although few of these neurocognitive gains were statistically significant on neuropsychological assessment.
This is the first prospective study assessing the LGIT for epilepsy. Our results indicate that this dietary therapy is highly effective in treating Angelman syndrome-related seizures. The diet was well tolerated by subjects as evidenced by five of six subjects remaining on the LGIT after completion of the trial. Beyond the prospective trial window, all five subjects who remained on the diet had >90% seizure reduction after 1 year of LGIT therapy. Despite the small sample size in this prospective study, the results indicate a potentially higher degree of efficacy of the LGIT for the Angelman syndrome population than that observed in the general epilepsy population. Although this study is too small to make definitive recommendations, these results suggest that the LGIT is a promising treatment option for Angelman syndrome-related epilepsy.
低血糖指数治疗（LGIT）是一种高脂肪、低碳水化合物的饮食，用于治疗癫痫。本研究的目的是评估 LGIT 治疗小儿安格曼综合征难治性癫痫的疗效和耐受性。
对一组患有难治性癫痫的小儿安格曼综合征患者进行 LGIT 治疗，并前瞻性随访 4 个月。父母在开始治疗前至少 1 个月记录每日癫痫发作日志，并在 LGIT 试验期间全程记录。每个受试者在基线和 4 个月随访时均进行脑电图（EEG）和神经心理评估（独立行为量表修订版和维兰德适应行为量表第二版）。在入组时以及在饮食治疗的第一个和第四个月后，由神经科医生和营养师对受试者进行临床评估。在每个时间点，抽取血液进行实验室化学检查，并进行人体测量。
6 名（平均年龄 3.3 岁，范围 1.1-4.8 岁）经基因证实患有安格曼综合征的儿童开始接受 LGIT 治疗，并完成了试验，无明显不良事件。癫痫严重程度指标的队列平均值如下：癫痫发作年龄为 1.6 岁，尝试过 3 种（范围 1-6 种）抗癫痫药物，基线时癫痫发作频率为 10.1 次/周（范围：0.4-30.9）。所有患者在 LGIT 治疗后癫痫发作频率均降低，6 例中有 5 例癫痫发作频率降低>80%。所有试验后 EEG 研究均显示改善，4 例基线 EEG 有癫痫样活动的儿童中有 3 例在随访 EEG 中无放电。父母在所有情况下都注意到了发育上的进步，尽管在神经心理学评估中，只有少数这些神经认知上的进步具有统计学意义。
这是第一项评估 LGIT 治疗癫痫的前瞻性研究。我们的结果表明，这种饮食疗法对治疗安格曼综合征相关癫痫非常有效。从 6 名患者中有 5 名在试验完成后仍继续接受 LGIT 治疗这一事实可以看出，该饮食疗法被患者很好地耐受。除了前瞻性试验窗口之外，在接受 LGIT 治疗 1 年后，仍继续接受饮食治疗的 5 名患者中有 5 名癫痫发作减少>90%。尽管本前瞻性研究的样本量较小，但结果表明 LGIT 对安格曼综合征患者的疗效可能比一般癫痫患者更高。尽管这项研究还太小，无法做出明确的建议，但这些结果表明 LGIT 是一种有前途的安格曼综合征相关癫痫治疗选择。
