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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 20:378-380 (3 1999)
© 1999 American Society of Neuroradiology

ARTICLE

MR Imaging of Dejerine-Sottas Disease

Daniel D. Maki^a, David M. Yousem^,a, Celeste Corcoran^a and Steven L. Galetta^a

^a From the Departments of Radiology (D.D.M., D.M.Y.) and Neurology (S.L.G.), University of Pennsylvania Medical Center, Philadelphia; and Narragansett Bay Pediatrics, Narragansett, RI (C.C.).

Summary: We report the MR findings in two patients with clinically and histologically proved Dejerine-Sottas disease. One patient had spinal involvement with multiple thickened and clumped nerve roots of the cauda equina; the second had multiple enlarged and enhancing cranial nerves. Although these findings are not specific for Dejerine-Sottas disease, they are suggestive of the diagnosis, which is further corroborated with history and confirmed with sural nerve biopsy and laboratory studies.

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