The authors demonstrate on the case of a 6-month-old boy the typical signs of Canavan’s disease during MR imaging and in the 1H MR spectrum of the brain, where the pathognomic finding is an absolute elevation of the N-acetyl aspartate concentration. This explains also the integration of the two MR methods in leukodystrophies not classified by laboratory examinations.

        Key words: MR imaging - MR spectroscopy - Canavan’s disease - leukodystrophy - N-acetyl aspartate