Cranial magnetic resonance imaging of Wolfram (DIDMOAD) syndrome

Summary Wolfram syndrome is a rare neurodegenerative disorder characterized by diabetes insipidus, diabetes mellitus, optic atrophy and deafness (DIDMOAD). A wide spectrum of abnormalities of the central nervous system, urinary tract and endocrine glands is also observed. We report cranial MRI findi...

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Bibliographic Details
Published in:Australasian radiology 2005-04, Vol.49 (2), p.189-191
Main Authors: Pakdemirli, E, Karabulut, N, Bir, LS, Sermez, Y
Format: Article
Language:English
Subjects:
Adult
Brain Diseases - pathology
diabetes insipidus
diabetes mellitus
Diagnosis, Differential
DIDMOAD syndrome
Female
Humans
Magnetic Resonance Imaging
optic atrophy
Wolfram syndrome
Wolfram Syndrome - pathology
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Summary:Summary Wolfram syndrome is a rare neurodegenerative disorder characterized by diabetes insipidus, diabetes mellitus, optic atrophy and deafness (DIDMOAD). A wide spectrum of abnormalities of the central nervous system, urinary tract and endocrine glands is also observed. We report cranial MRI findings in a 32‐year‐old female patient with Wolfram syndrome. In addition to the classical features, including absence of the normal high signal of the neurohypophysis, atrophy of visual pathways, the brainstem, cerebellum and cerebral cortex, we observed bilateral hyperintensity on proton density‐ and T2‐ weighted images related to the optic radiations in the periventricular white matter of the temporal and parieto‐occipital lobes, which may reflect gliosis pathologically.
ISSN:0004-8461
1440-1673
DOI:10.1111/j.1440-1673.2005.01420.x