Coppola Giangennaro, Klepper Jörg, Ammendola Eduardo, Fiorillo Monica, della Corte Rita, Capano Guglielmo, Pascotto Antonio
Clinic of Child Neuropsychiatry, Second University of Naples, Naples, Italy.
Eur J Paediatr Neurol. 2006 May;10(3):148-51. doi: 10.1016/j.ejpn.2006.03.001. Epub 2006 Apr 24.
Tuberous sclerosis complex (OMIM 191100) is a multiorgan disease commonly associated with epilepsy refractory to anticonvulsants. Individual reports indicate that seizures in children with tuberous sclerosis might benefit from a ketogenic diet. We studied the effects of the diet introduced at 3.5 years of age in three boys with tuberous sclerosis and refractory partial seizures.
On admission a classical LCT ketogenic diet was initiated and patients were followed for 12 months. Antiepileptic drugs were maintained unless adverse effects required reduction.
Two patients became seizure-free within 2 months on the diet. In the third patient drop attacks decreased significantly. On follow-up the diet was well accepted and without adverse effects.
The ketogenic diet should be considered as a treatment option for children with tuberous sclerosis and partial seizures refractory to anticonvulsants. Our data support the need for further studies in larger cohorts to confirm the effectiveness of the ketogenic diet in this entity.
结节性硬化症（OMIM 191100）是一种多器官疾病，常与对抗惊厥药难治的癫痫相关。个别报告表明，结节性硬化症患儿的癫痫发作可能从生酮饮食中获益。我们研究了在3.5岁时开始采用该饮食对三名患有结节性硬化症和难治性部分性癫痫发作的男孩的影响。
入院时开始采用经典的长链甘油三酯生酮饮食，并对患者进行12个月的随访。除非出现不良反应需要减量，否则维持抗癫痫药物治疗。
两名患者在饮食治疗2个月内癫痫发作消失。第三名患者跌倒发作显著减少。随访时，该饮食耐受性良好且无不良反应。
对于患有结节性硬化症且对抗惊厥药难治的部分性癫痫发作患儿，应考虑将生酮饮食作为一种治疗选择。我们的数据支持需要在更大的队列中进行进一步研究，以证实生酮饮食在该疾病中的有效性。
