RECURRENT EPISODES OF BIZARRE BEHAVIOR IN A BOY WITH ORNITHINE TRANSCARBAMYLASE DEFICIENCY - DIAGNOSTIC FAILURE OF PROTEIN LOADING AND ALLOPURINOL CHALLENGE TESTS

Recurrent episodes of bizarre behavior were the only clinical symptoms that finally led to the diagnosis of ornithine transcarbamylase defic iency in an 8-year-old boy. The suspected diagnosis could not be confi rmed with the use of current challenge tests. The response to a high-p rotein diet for 24 hours appeared to be a helpful diagnostic aid.