title: Diabetes mellitus and optic atrophy in two siblings: a report on a new association and a review of the literature.
abstract: Two siblings with diabetes mellitus and optic atrophy (Wolfram syndrome) are described. As often noted, they also had atonic urinary bladders. Only one of the siblings had some impairment of hearing. Other findings not previously reported that appeared in each subject were esophageal dysphagia and vertigo. An autopsy in one revealed brain stem hypoplasia and thinning and flattening of the optic nerves with atrophy of the lateral geniculate bodies.
mesh_terms: D000328:Adult; D001933:Brain Stem / Q000473:pathology; D003680:Deglutition Disorders / Q000235:genetics; D003920:Diabetes Mellitus / Q000235:genetics* / Q000473:pathology; D005260:Female; D006311:Hearing Disorders / Q000235:genetics; D006801:Humans; D008297:Male; D009896:Optic Atrophy / Q000235:genetics*; D009900:Optic Nerve / Q000473:pathology; D013577:Syndrome; D001745:Urinary Bladder Diseases / Q000235:genetics; D014717:Vertigo / Q000235:genetics