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Diabetic Retinopathy and Optic Atrophy as Initial Sign of Wolfram Syndrome (WFS)

Schmidt J. C., Meyer C. H., Schüller C.,
Philipps-Universität Marburg, Medizinisches Zentrum für Augenheilkunde (Marburg)

Purpose: Wolfram syndrome (WFS) is an autosomal-recessive disorder on chromosome 4 (4p16.1).
Method: A 22 year old boy with juvenile diabetes mellitus and optic atrophy was ophthalmologically, internistically, audiometrically, neurologically, nephrologically and human gentcally examined.
Results: Visual acuity was 0.05 on both eyes. On funduscopy, there were no proliferations but an optic atrophy, which gave reduced potentials in the VEP. Visual fields were reduced to minimal island. Aside a beginning deafness on audiometry, a early polyneuropathy (PNP) in the legs and a light atrophy of the white brain substance, there was a predominance of the HLA-DR2 haplotype, which differs from the classic juvenile diabetes mellitus.
Conclusions: The presence of insulin-dependent diabetes mellitus and optic atrophy are the minimal criteria for WFS, which is also called "DIDMOAD" for diabetes insipidus, juvenile diabetes mellitus, optic atrophy and a few other neurologic symptoms including deafness, ataxia and periphery neuropathy. Young patients with diabetes mellitus and optic atrophy should have further evaluations to exclude hereditary diseases and detect early signs of an unknown systemic

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