Optic atrophy in Wolfram syndrome.
The clinical and electrophysiologic findings in 11 cases of Wolfram syndrome are presented. These findings suggest that optic atrophy in Wolfram syndrome is not secondary to retinal pathology, but probably represents part of a generalized degeneration of neural structures. The relationship of diabetes mellitus to this process of neural degeneration remains unclear.
D000293:Adolescent; D000328:Adult; D003623:Dark Adaptation; D004585:Electrooculography; D004596:Electroretinography; D005074:Evoked Potentials, Visual; D005260:Female; D006801:Humans; D008297:Male; D009896:Optic Atrophy / Q000235:genetics* / Q000473:pathology; D009900:Optic Nerve / Q000473:pathology; D010375:Pedigree; D014794:Visual Fields; D014929:Wolfram Syndrome / Q000235:genetics* / Q000473:pathology