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Wolfram Syndrome:Evidence of a Diffuse Neurodegenerative Disease by Magnetic Resonance Imaging
Neurol 42:1220-1224, 1992,, Rando,T.A.,et al, 1992
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Article Abstract
Wolfram syndrome is an autosomal recessive disorder beginning in childhood that consists of four cardinal features:optic atrophy,diabetes mellitus, diabetes insipidus,and neurosensory hearing loss.Aside from these features,the clinical picture is highly variable and may include other neurologic abnormalities such as ataxia,nystagmus,mental retardation,and seizures.We present two unrelated patients with Wolfram syndrome,both of whom had the four cardinal features and several other neurologic abnormalities.MRIs showed widespread atrophic changes throughout the brain,some of which correlated with the major neurologic features of the syndrome.
 
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ataxia
bladder dysfunction
brainstem,atrophy
cerebellar atrophy,primary
cerebral cortical atrophy
degenerative diseases of CNS
diabetes insipidus
diabetes mellitus
dysarthria
dystonia
hearing loss
hydrocephalus
hyposmia
mental retardation
MRI
MRI,abnormal
nystagmus
optic atrophy
pigmentary retinopathy
psychiatric problems in neurologic disorders
retinopathy
seizure
sensorineural hearing loss
Wolfram syndrome

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