PT  - JOURNAL ARTICLE
AU  - Ito, S.
AU  - Sakakibara, R.
AU  - Hattori, T.
TI  - Wolfram Syndrome Presenting Marked Brain MR Imaging Abnormalities with Few Neurologic Abnormalities
DP  - 2007 Feb 01
TA  - American Journal of Neuroradiology
PG  - 305--306
VI  - 28
IP  - 2
4099  - http://www.ajnr.org/content/28/2/305.short
4100  - http://www.ajnr.org/content/28/2/305.full
SO  - Am. J. Neuroradiol.2007 Feb 01; 28
AB  - 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.