Linkage and association between a CD4 gene polymorphism and IDDM in Danish IDDM patients. The Danish IDDM Epidemiology and Genetics Group, and The Danish Study Group of Diabetes in Childhood

PURPOSE: Our purpose was to determine the neuroradiologic findings of Marinesco-Sj?gren syndrome on plain skull radiographs, CT, and MR images. METHODS: Eight patients with proved Marinesco-Sj?gren syndrome (age range, 4 to 56 years) had a total of nine CT scans, seven MR imaging studies, and two plain radiographic examinations of the skull. The findings were reviewed retrospectively, with particular attention to the size of the posterior fossa and cerebellum. RESULTS: All patients had hypoplastic cerebellar hemispheres and a hypoplastic vermis in a small posterior fossa. One patient had a midline posterior fossa cyst and another had agenesis of the corpus callosum. CONCLUSION: Hypoplasia of the cerebellar hemispheres and the vermis and a small posterior fossa are the most prominent neuroradiologic findings in Marinesco-Sj?gren syndrome.