TY - JOUR T1 - Wolfram Syndrome Presenting Marked Brain MR Imaging Abnormalities with Few Neurologic Abnormalities JF - American Journal of Neuroradiology JO - Am. J. Neuroradiol. SP - 305 LP - 306 VL - 28 IS - 2 AU - S. Ito AU - R. Sakakibara AU - T. Hattori Y1 - 2007/02/01 UR - http://www.ajnr.org/content/28/2/305.abstract N2 - 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. ER -