RESUMEN
We report on two patients with pyridoxine-dependent seizures who illustrate the varied clinical presentation and management problems of this syndrome. Subjects and Interventions: The first patient [a Saudi girl who showed the typical presentation] was born meconium-stained and had seizures in the first day of life that were finally controlled in the third day, 2 minutes after intravenous administration of 50 mg pyridoxine. Before initiating pyridoxine, a burst-suppression pattern dominated her EEG. The second [a Sudanese girl] had an atypical presentation. Onset was relatively late with irritability, hyperacusis and vomiting at the age of 3 days, followed by subtle seizures that could easily be controlled with conventional anticonvulsants. The diagnosis was established at 3 1/2 months following recurrent attacks of afebrile multifocal myoclonic jerks. Parenteral injection of 100 mg pyridoxine [given under EEG] resulted in cessation of electroclinical seizures. Both patients exhibited lethargy, hypotonia and/or apnoea immediately after pyridoxine administration. Main Outcome Measures and. After 7 years follow-up the first patient had only mild delay in language development. The second child had normal development at 14 months. The two patients represent the first cases reported from Saudi Arabia and the Middle East