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Neurodegeneration and Diabetes:UK Nationwide Study of Wolfram (DIDMOAD) Syndrome
Lancet 1458-1463, Barrett,T.G.,et al, 1995
See this aricle in Pubmed

Article Abstract
Wolfram syndrome is the association of diabetes mellitus and optic atrophy,and is sometimes called DIDMOAD(diabetes insipidus,diabetes mellitus,optic atrophy,and deafness).Incomplete characterisation of this autosomal recessive syndrome has relied on case-reports,and there is confusion with mitochondrial genome disorders.We therefore undertook a UK nationwide cross-sectional case-finding study to describe the natural history,complications,prevalence,and inheritance of the syndrome.The natural history of Wolfram syndrome suggests that most patients will eventually develop most complications of this progressive, neurodegenerative disorder.Family studies indicate autosomal recessive inheritance with a carrier frequency of one in 354,an absence of a maternal mitochondrial tRNA Leu(3243)mutation.Juvenile-onset diabetes mellitus and optic atrophy are the best available diagnostic criteria for Wolfram syndrome,the differential diagnosis of which includes other causes of neurodegeneration.
 
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ataxia
ataxia,cerebellar
ataxic gait
brainstem,atrophy
deafness
degenerative diseases of CNS
diabetes insipidus
diabetes mellitus
familial
genetic neurologic disorders
mortality
MRI
MRI,abnormal
myoclonus
optic atrophy
respiratory failure
review article
sensorineural hearing loss
startle myoclonus
visual acuity,decreased
visual loss
Wolfram syndrome

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