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Diabetes Mellitus and Optic Atrophy in Two Siblings: A Report on a New Association and a Review of the Literature

  1. Romesh Khardori,
  2. John W Stephens,
  3. Otto C Page and
  4. Robert S Dow
  1. Divisions of Endocrinology and Metabolism, Department of Medicine, Good Samaritan Hospital and Medical Center, Oregon Health Sciences University Portland, Oregon
  2. Neurology, Department of Medicine, Good Samaritan Hospital and Medical Center, Oregon Health Sciences University Portland, Oregon

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.

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