Cranial magnetic resonance imaging of Wolfram (DIDMOAD) syndrome

E Pakdemirli; N Karabulut; L S Bir; Y Sermez

doi:10.1111/j.1440-1673.2005.01420.x

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Journal article

Cranial magnetic resonance imaging of Wolfram (DIDMOAD) syndrome

E Pakdemirli, N Karabulut, L S Bir and Y Sermez

Australasian radiology, Vol.49(2), pp.189-191

04/2005

DOI: 10.1111/j.1440-1673.2005.01420.x

PMID: 15845065

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Abstract

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.

Magnetic Resonance Imaging

Adult

Brain Diseases - pathology

Diagnosis, Differential

Female

Humans

Wolfram Syndrome - pathology

Details

Title: Subtitle: Cranial magnetic resonance imaging of Wolfram (DIDMOAD) syndrome
Creators: E Pakdemirli - Pamukkale University
N Karabulut
L S Bir - Pamukkale University
Y Sermez - Pamukkale University
Resource Type: Journal article
Publication Details: Australasian radiology, Vol.49(2), pp.189-191
DOI: 10.1111/j.1440-1673.2005.01420.x
PMID: 15845065
NLM abbreviation: Australas Radiol
ISSN: 0004-8461
eISSN: 1440-1673
Language: English
Date published: 04/2005
Academic Unit: Radiology
Record Identifier: 9984697636002771

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