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Pyridoxine-dependent convulsions among children with refractory seizures: A 3-year follow-up study
Sadanandavalli Retnaswami Chandra1, Thomas Gregor Issac2, Sai Deepak1
1Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.
Insights
Pyridoxine-dependent convulsions, a rare cause of refractory childhood epilepsy, respond well to targeted treatment. Early intervention leads to significant seizure control and improved neurodevelopment with minimal cost and side effects.
Area of Science:
- Pediatric Neurology
- Epileptology
- Metabolic Disorders
Background:
- Epilepsy affects 1% of the global burden, with millions in India. A significant portion (30-40%) develop drug-resistant epilepsy, imposing substantial psychosocial and economic burdens.
- Refractory epilepsy in children presents severe cognitive and motor challenges, necessitating a deeper understanding of its diverse causes.
- Identifying the spectrum of causes for antiepileptic drug refractoriness in pediatric epilepsy is crucial for effective management.
Purpose of the Study:
- To investigate the spectrum of causes for refractory epilepsy in children.
- To identify specific conditions contributing to drug-resistant epilepsy in the pediatric population.
- To evaluate treatment responses and outcomes for children with refractory epilepsy, particularly focusing on treatable metabolic causes.
Main Methods:
- A retrospective study included children admitted with refractory epilepsy, adhering to International League Against Epilepsy (ILAE) criteria, over a 3-year period.
- Patient data were analyzed to determine the prevalence of different epilepsy etiologies.
- Treatment response and neurodevelopmental outcomes were assessed following appropriate interventions.
Main Results:
- Refractory epilepsy accounted for 13.3% of pediatric inpatients, with males being predominant (68.9%).
- Neurometabolic diseases were identified in 34.4% of cases, with pyridoxine-dependent convulsions (PDC) representing 3.5% of refractory epilepsies.
- Patients with PDC demonstrated excellent response to dietary modifications, metabolic disorder treatment, and low-dose anticonvulsants, showing rapid seizure control and global development improvements.
Conclusions:
- Pyridoxine-dependent convulsions are a rare but significant cause of refractory epilepsy in children, responding dramatically to specific treatment.
- Early initiation of appropriate therapy for PDC leads to rapid seizure control and neurodevelopmental progress within weeks, with sustained benefits at 1-year follow-up.
- Treatment for PDC is cost-effective, has minimal side effects, and can prevent the need for palliative surgery, offering a high chance of functional independence.
Introduction:
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.
Patients And 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.
Conclusion:
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.
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