Abstract
MacDermont et al1 (Pediatrics 65:624, 1980) have described the uncomplicated efficacy of valproate in treating the seizures of two patients with nonketotic hyperglycinemia even though valproate elevates urinary and serum glycine concentrations in individuals with convulsive disorders.2,3 Recent observations of increased urinary propionate excretion as a metabolic consequence of valproate therapy in two patients with seizures seem to contraindicate its use in children with disorders of propionate metabolism.4 However, we have successfully and safely used valproate to control seizures in a patient with propionyl coenzyme A carboxylase (PCC) deficiency, a disorder characterized by both hyperglycinemia and propionic acidemia.
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