Chandra Sadanandavalli Retnaswami, Issac Thomas Gregor, Deepak Sai, Teja Ravi, Kuruthukulangara Seby
Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.
Department of Clinical Neurosciences, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.
J Pediatr Neurosci. 2016 Jul-Sep;11(3):188-192. doi: 10.4103/1817-1745.193361.
Epilepsy accounts for 1% of the global disease burden and about 8-10 million epilepsy patients live in India. About 30-40% of these patients become drug-resistant and land up with palliative or disease-modifying surgeries. This is a situation causing great concern in view of the psychosocial and economic burden on the patient and the family apart from severe cognitive and motor consequences, especially in children. Therefore, it is mandatory to have an insight into the wide spectrum of causes with reference to refractoriness to antiepileptic medications in children with epilepsy.
Children admitted under our team with refractory epilepsy as per the International League Against Epilepsy (ILAE) criteria in the last 3 years were included in the study.
Refractory epilepsy constituted 13.3% of inpatients in the pediatric group. Males dominated with 68.9% of these patients. Nearly 34.4% of these patients were found to suffer from various neurometabolic diseases. Almost 3.5% were due to pyridoxine-dependent convulsions. This group of patients showed an excellent response to dietary manipulation, disease-modifying treatment for the metabolic disorder, and supportive small-dose anticonvulsants. During follow-up, they showed very good response with reference to global development and seizure control.
Pyridoxine-dependent convulsions are relatively rare forming about 3.5% of refractory epilepsies in this series. With initiation of appropriate therapy, results with reference to seizure control as well as neurodevelopment became evident within 2 weeks, and at 1-year follow-up, complete independence for majority of the needed activities is achieved with minimum cost, almost zero side effects, and absolute elimination of the need for palliative surgery.
癫痫占全球疾病负担的1%，印度约有800万至1000万癫痫患者。这些患者中约30%-40%会产生耐药性，最终接受姑息性或改善病情的手术。鉴于这给患者及其家庭带来的社会心理和经济负担，以及严重的认知和运动后果，尤其是对儿童而言，这种情况令人极为担忧。因此，必须深入了解癫痫患儿对抗癫痫药物难治的广泛病因。
纳入过去3年中按照国际抗癫痫联盟（ILAE）标准由我们团队收治的难治性癫痫患儿。
难治性癫痫占儿科住院患者的13.3%。男性占主导，占这些患者的68.9%。近34.4%的患者患有各种神经代谢疾病。约3.5%是由于维生素B6依赖型惊厥。这组患者对饮食调整、针对代谢紊乱的病情改善治疗以及小剂量支持性抗惊厥药物反应良好。在随访期间，他们在整体发育和癫痫控制方面表现出非常好的反应。
维生素B6依赖型惊厥相对罕见，在本系列难治性癫痫中约占3.5%。开始适当治疗后，癫痫控制以及神经发育方面的效果在2周内就很明显，在1年随访时，大多数所需活动可实现完全独立，成本最低，几乎没有副作用，并且完全无需进行姑息性手术。
