Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
2
pubmed:dateCreated
2005-3-14
pubmed:abstractText
Wolfram syndrome is a rare diffuse neurodegenerative disorder also known as DIDMOAD due to its characteristics of diabetes insipidus, diabetes mellitus, optic nerve atrophy and deafness. It is also associated with a wide variety of abnormalities of the central nervous system, urinary tract and endocrine glands. It may be familial or sporadic. Imaging findings include absence of the short T1 nature of the pituitary posterior lobe, atrophy of the optic nerve, chiasma, and tracts. Atrophy of the brain stem and cerebellum has also been reported. We describe a 15-year-old boy and an 11-year-old girl with Wolfram syndrome who were siblings from a diabetes mellitus family. They received regular insulin control at our hospital and had symptoms of frequent urinary tract infection and diabetes insipidus. Magnetic resonance imaging revealed marked pons and cerebellar atrophy. Optic nerve and chiasma atrophy was also noted.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Feb
pubmed:issn
0929-6646
pubmed:author
pubmed:issnType
Print
pubmed:volume
104
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
129-32
pubmed:meshHeading
pubmed:year
2005
pubmed:articleTitle
Imaging characteristics of familial Wolfram syndrome.
pubmed:affiliation
Department of Radiology, Chung-Shan Medical University Hospital, Taichung, Taiwan.
pubmed:publicationType
Journal Article, Case Reports