GLUTARIC ACIDURIA TYPE-I - A SERIOUS PITFALL IF DIAGNOSED TOO LATE

We report the MR imaging findings in two children with glutaric acidur ia type I (GA I). It is important to consider this disorder in the dif ferential diagnosis in a child presenting with an unclear hydrocephalu s or atrophy. The imaging findings consist of basal ganglia changes, f rontotemporal atrophy, and retarded myelination. A definite diagnosis with an urine test and a dietary treatment can avoid encephalopathy wi th irreversible changes.