OBJECTIVES: Determine the prevalence of pyridoxine dependent seizures in children less than 16 years of age attending a teaching hospital in south India with early onset (before 3 years) intractable epilepsy of unknown aetiology, using the criteria proposed by Baxter. METHOD: A cohort of 81 children, fulfilling the above criteria, was given 15 mg/kg/day of oral pyridoxine for 7 days. Non-responders were given a further 7-day trial of 30 mg/kg/day. Diagnosis of pyridoxine dependent seizures was made according to the criteria proposed by Baxter. RESULT: Six children (7.4%; four boys and two girls) were identified as definite cases and were continued on B6, without recurrence of seizures. Median age of seizure onset and diagnosis were 2.5 months and 2 year 9 months, respectively. No significant complications were observed with B6 therapy. CONCLUSION: Chance of identifying PDS is not low in a subpopulation of children with intractable early onset cryptogenic epilepsy using the criteria proposed by Baxter, which warrants a therapeutic trial with B6 in them.
OBJECTIVES: Determine the prevalence of pyridoxine dependent seizures in children less than 16 years of age attending a teaching hospital in south India with early onset (before 3 years) intractable epilepsy of unknown aetiology, using the criteria proposed by Baxter. METHOD: A cohort of 81 children, fulfilling the above criteria, was given 15 mg/kg/day of oral pyridoxine for 7 days. Non-responders were given a further 7-day trial of 30 mg/kg/day. Diagnosis of pyridoxine dependent seizures was made according to the criteria proposed by Baxter. RESULT: Six children (7.4%; four boys and two girls) were identified as definite cases and were continued on B6, without recurrence of seizures. Median age of seizure onset and diagnosis were 2.5 months and 2 year 9 months, respectively. No significant complications were observed with B6 therapy. CONCLUSION: Chance of identifying PDS is not low in a subpopulation of children with intractable early onset cryptogenic epilepsy using the criteria proposed by Baxter, which warrants a therapeutic trial with B6 in them.
Authors: Gunter Scharer; Chad Brocker; Vasilis Vasiliou; Geralyn Creadon-Swindell; Renata C Gallagher; Elaine Spector; Johan L K Van Hove Journal: J Inherit Metab Dis Date: 2010-09-03 Impact factor: 4.982
Authors: Remi Stevelink; Faith Pangilinan; Floor E Jansen; Kees P J Braun; Anne M Molloy; Lawrence C Brody; Bobby P C Koeleman Journal: Mol Genet Metab Rep Date: 2019-10-11