Department of Neurology, Hospital de Pediatría "Prof Dr Juan P Garrahan", Buenos Aires, Argentina.
Servizio di Neuropsichiatria Infantile, Policlinico G.B. Rossi, Universita degli Studi di Verona, Italy.
Seizure. 2017 Oct;51:1-5. doi: 10.1016/j.seizure.2017.07.002. Epub 2017 Jul 11.
Myoclonic status in non-progressive encephalopathy (MSNPE) is characterized by the recurrence of long-lasting atypical status epilepticus associated with attention impairment and continuous polymorphous jerks, mixed with other complex abnormal movements, in infants suffering from a non-progressive encephalopathy. The ketogenic diet (KD) has been used as an alternative to antiepileptic drugs (AEDs) for patients with refractory epileptic encephalopathies.
In this study we assess the efficacy and tolerability of the KD in patients with MSNPE.
Between March 1, 1980 and August 31, 2013, 99 patients who met the diagnostic criteria of MSNPE were seen (58 patients in Verona and 41 patients in Buenos Aires). Six of these 99 patients were placed on the KD using the Hopkins protocol and followed for a minimum period of 24 months.
Twelve months after initiating the diet, three patients had a 75%-99% decrease in seizures, two had a 50%-74% decrease in seizures, and the remaining child had a less than 50% seizure reduction. In five patients with a seizure reduction of more than 50%, the myoclonic status epilepticus disappeared within 6 months after starting the diet. All patients had very good tolerability and no adverse events were identified. In most of the patients AEDs were reduced.
The KD is a promising therapy for MSNPE, with most of our patients showing a more than 50% seizure reduction. In patients that responded well to the diet cognitive performance and quality of life also improved.
本研究旨在评估生酮饮食（KD）在 MSNPE 患者中的疗效和耐受性。
1980 年 3 月 1 日至 2013 年 8 月 31 日期间，共观察到 99 例符合 MSNPE 诊断标准的患者（58 例来自维罗纳，41 例来自布宜诺斯艾利斯）。这 99 例患者中有 6 例采用霍普金斯方案进行 KD，随访时间至少 24 个月。
开始饮食 12 个月后，3 例患者癫痫发作减少 75%-99%，2 例减少 50%-74%，其余患儿减少不到 50%。在 5 例癫痫发作减少超过 50%的患者中，开始饮食后 6 个月内癫痫持续状态消失。所有患者均具有良好的耐受性，未发现不良反应。大多数患者减少了抗癫痫药物的使用。
KD 是 MSNPE 的一种有前途的治疗方法，我们的大多数患者癫痫发作减少超过 50%。对饮食反应良好的患者认知表现和生活质量也有所改善。
