RT Journal Article SR Electronic T1 The reflection of histology in MR imaging of Pelizaeus-Merzbacher disease. JF American Journal of Neuroradiology JO Am. J. Neuroradiol. FD American Society of Neuroradiology SP 99 OP 103 VO 10 IS 1 A1 M S van der Knaap A1 J Valk YR 1989 UL http://www.ajnr.org/content/10/1/99.abstract AB Pelizaeus-Merzbacher disease is a rare disease of infants and young children in which there is an arrest of myelination of the CNS. The cause of the disorder is unknown. Three cases are reported, representing the connatal and classical forms of the disease. The MR images obtained of these children are compared with histologic findings as reported in the literature. In the connatal form a complete absence of myelin in the brain is demonstrated. In the classical form an arrest of myelination in an early stage of development is shown in combination with progressive abnormalities of the unmyelinated white matter and possibly a slow degradation of some myelin. MR imaging appears to be a unique diagnostic method for assessing the degree of hypomyelination, the core of Pelizaeus-Merzbacher disease, and for contributing to the understanding of the underlying pathology of this disorder.