title: Pyridoxine-dependent convulsions among children with refractory seizures: A 3-year follow-up study.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.
authors.forename: Sadanandavalli Retnaswami
authors.initials: SR
authors.lastname: Chandra
authors.affiliation: Department of Clinical Neurosciences, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.
authors.forename: Thomas Gregor
authors.initials: TG
authors.lastname: Issac
authors.affiliation: Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.
authors.forename: Sai
authors.initials: S
authors.lastname: Deepak
authors.affiliation: Department of Clinical Neurosciences, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.
authors.forename: Ravi
authors.initials: R
authors.lastname: Teja
authors.affiliation: Department of Psychiatry, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.
authors.forename: Seby
authors.initials: S
authors.lastname: Kuruthukulangara
keywords: Neurometabolic disorders; pyridoxine-dependent seizures; refractory seizures