Metabolic disorders constitute an important cause ofneurologic disease including infantile seizures.Objective: To identify clinical and biochemicalfeatures suggestive of an underlying metabolicetiology.Methods: 40 patients with convulsive disorderswere evaluated by selective metabolic screeningand basic metabolic investigations.Results: 19 patients had metabolic derangements.Biochemical genetic diagnosis could be formulatedin only 15 patients: UCD (5 patients), Organicacidurias (5 patients), aminoacidopathies (2patients), Disorders of gluconeogenesis (2 patients),and congenital lactic acidosis (1 patient).