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American Journal of Neuroradiology 28:305-306, February 2007
© 2007 American Society of Neuroradiology

Case Report
BRAIN

Wolfram Syndrome Presenting Marked Brain MR Imaging Abnormalities with Few Neurologic Abnormalities

S. Ito, R. Sakakibara and T. Hattori

From the Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan

Please address correspondence to Shoichi Ito, MD, Department of Neurology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba, 260-8670, Japan; e-mail: sito{at}faculty.chiba-u.jp

SUMMARY: Wolfram syndrome is a rare autosomal recessive disorder featuring diabetes insipidus, diabetes mellitus, optic atrophy, and deafness; DIDMOAD is a commonly accepted anonym for this disorder. We describe a 35-year-old man with Wolfram syndrome, who had marked atrophy of the brain stem, middle cerebellar peduncle, and cerebellum. Despite these MR imaging findings involving the pontocerebellar tract, the patient had no neurologic abnormalities suggesting dysfunction of the brain stem or cerebellum. Patients with Wolfram syndrome may have discrepancies between neurologic and radiologic findings.






Copyright © 2008 by the American Society of Neuroradiology. Print ISSN: 0195-6108 Online ISSN: 1936-959X