Part of type C of Niemann-Pick disease is affection of the central nervous system, manifested during MR images and in the 1H MR spectrum of the brain by signs of atrophy and demyelinization with an increased lipid signal. The authors summarize these findings in their own observation and discuss some differences as compared with those known from the literature.

        Key words: MR imaging - MR spectroscopy - congenital metabolic diseases of the CNS - M. Niemann- Pick, type C