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American Journal of Neuroradiology, Vol 19, Issue 2 281-283, Copyright © 1998 by American Society of Neuroradiology

ARTICLES

Neuroradiologic findings in Marinesco-Sjogren syndrome

BA Georgy, RD Snow, BG Brogdon and W Wertelecki
Department of Radiology, University of South Alabama College of Medicine, Mobile, USA.

PURPOSE: Our purpose was to determine the neuroradiologic findings of Marinesco-Sjogren syndrome on plain skull radiographs, CT, and MR images. METHODS: Eight patients with proved Marinesco-Sjogren 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-Sjogren syndrome.


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Copyright © 2009 by the American Society of Neuroradiology. Print ISSN: 0195-6108 Online ISSN: 1936-959X