Treatment of late-onset nonketotic hyperglycinaemia: Effectiveness of imipramine and benzoate

We report a patient with late-onset nonketotic hyperglycinaemia managed wit h a sequential approach to drug therapy in placebo-controlled therapeutic t rials. Partial response to low-protein diet and sodium benzoate and dramati c response to imipramine are demonstrated, with parental scores on the Deve lopmental Behavioural Checklist falling from the 86th centile before treatm ent to normal with combined benzoate and imipramine therapy.