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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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| Published in: | Australasian radiology 2005-04, Vol.49 (2), p.189-191 |
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| container_end_page | 191 |
| container_issue | 2 |
| container_start_page | 189 |
| container_title | Australasian radiology |
| container_volume | 49 |
| creator | Pakdemirli, E Karabulut, N Bir, LS Sermez, Y |
| description | 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. |
| doi_str_mv | 10.1111/j.1440-1673.2005.01420.x |
| format | article |
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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.</description><identifier>ISSN: 0004-8461</identifier><identifier>EISSN: 1440-1673</identifier><identifier>DOI: 10.1111/j.1440-1673.2005.01420.x</identifier><identifier>PMID: 15845065</identifier><language>eng</language><publisher>Oxford, UK: Blackwell Science Pty</publisher><subject>Adult ; Brain Diseases - pathology ; diabetes insipidus ; diabetes mellitus ; Diagnosis, Differential ; DIDMOAD syndrome ; Female ; Humans ; Magnetic Resonance Imaging ; optic atrophy ; Wolfram syndrome ; Wolfram Syndrome - pathology</subject><ispartof>Australasian radiology, 2005-04, Vol.49 (2), p.189-191</ispartof><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c2840-7ba5e835cd87a60c0e737e96aeb792584c967e230cb4710ca07b1db9387b4cff3</citedby><cites>FETCH-LOGICAL-c2840-7ba5e835cd87a60c0e737e96aeb792584c967e230cb4710ca07b1db9387b4cff3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/15845065$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Pakdemirli, E</creatorcontrib><creatorcontrib>Karabulut, N</creatorcontrib><creatorcontrib>Bir, LS</creatorcontrib><creatorcontrib>Sermez, Y</creatorcontrib><title>Cranial magnetic resonance imaging of Wolfram (DIDMOAD) syndrome</title><title>Australasian radiology</title><addtitle>Australas Radiol</addtitle><description>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.</description><subject>Adult</subject><subject>Brain Diseases - pathology</subject><subject>diabetes insipidus</subject><subject>diabetes mellitus</subject><subject>Diagnosis, Differential</subject><subject>DIDMOAD syndrome</subject><subject>Female</subject><subject>Humans</subject><subject>Magnetic Resonance Imaging</subject><subject>optic atrophy</subject><subject>Wolfram syndrome</subject><subject>Wolfram Syndrome - pathology</subject><issn>0004-8461</issn><issn>1440-1673</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2005</creationdate><recordtype>article</recordtype><recordid>eNqNkMlOwzAQhi0EoqXwCsgnBIeEcezYyQmqlqWoVSUE4mg5jlOlylLsVrRvj0MruDKXGc38s30IYQIh8Xa7DAljEBAuaBgBxCEQFkG4PUL938Ix6gMACxLGSQ-dObcEIJTE_BT1SJywGHjcR_cjq5pSVbhWi8asS42tcW2jGm1w6XNls8BtgT_aqrCqxtfjyXg2H45vsNs1uW1rc45OClU5c3HwA_T--PA2eg6m86fJaDgNdJT4i0SmYpPQWOeJUBw0GEGFSbkymUgjf45OuTARBZ0xQUArEBnJs5QmImO6KOgAXe3nrmz7uTFuLevSaVNVqjHtxkkuBI8iJrww2Qu1bZ2zppAr6z-xO0lAdvTkUnaQZAdJdvTkDz259a2Xhx2brDb5X-MBlxfc7QVfZWV2_x4sX2aT13kX028ovXyi</recordid><startdate>200504</startdate><enddate>200504</enddate><creator>Pakdemirli, E</creator><creator>Karabulut, N</creator><creator>Bir, LS</creator><creator>Sermez, Y</creator><general>Blackwell Science Pty</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>200504</creationdate><title>Cranial magnetic resonance imaging of Wolfram (DIDMOAD) syndrome</title><author>Pakdemirli, E ; Karabulut, N ; Bir, LS ; Sermez, Y</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c2840-7ba5e835cd87a60c0e737e96aeb792584c967e230cb4710ca07b1db9387b4cff3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2005</creationdate><topic>Adult</topic><topic>Brain Diseases - pathology</topic><topic>diabetes insipidus</topic><topic>diabetes mellitus</topic><topic>Diagnosis, Differential</topic><topic>DIDMOAD syndrome</topic><topic>Female</topic><topic>Humans</topic><topic>Magnetic Resonance Imaging</topic><topic>optic atrophy</topic><topic>Wolfram syndrome</topic><topic>Wolfram Syndrome - pathology</topic><toplevel>online_resources</toplevel><creatorcontrib>Pakdemirli, E</creatorcontrib><creatorcontrib>Karabulut, N</creatorcontrib><creatorcontrib>Bir, LS</creatorcontrib><creatorcontrib>Sermez, Y</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Australasian radiology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Pakdemirli, E</au><au>Karabulut, N</au><au>Bir, LS</au><au>Sermez, Y</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Cranial magnetic resonance imaging of Wolfram (DIDMOAD) syndrome</atitle><jtitle>Australasian radiology</jtitle><addtitle>Australas Radiol</addtitle><date>2005-04</date><risdate>2005</risdate><volume>49</volume><issue>2</issue><spage>189</spage><epage>191</epage><pages>189-191</pages><issn>0004-8461</issn><eissn>1440-1673</eissn><abstract>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.</abstract><cop>Oxford, UK</cop><pub>Blackwell Science Pty</pub><pmid>15845065</pmid><doi>10.1111/j.1440-1673.2005.01420.x</doi><tpages>3</tpages></addata></record> |
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| ispartof | Australasian radiology, 2005-04, Vol.49 (2), p.189-191 |
| issn | 0004-8461 1440-1673 |
| language | eng |
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| source | Wiley |
| 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 |
| title | Cranial magnetic resonance imaging of Wolfram (DIDMOAD) syndrome |
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