Cerebral 1H MR spectroscopy showing elevation of brain guanidino acetate in argininosuccinate lyase deficiency

MR spectroscopy in a patient with argininosuccinate lyase deficiency revealed elevated cerebral guanidinoacetate signals, indicating that the phenomenon of increased levels of this compound in brain tissue is not limited to creatine deficiencies. (c) 2005 Elsevier Inc. All rights reserved.