[Visual impairment in juvenile diabetes mellitus due to optic atrophy: Wolfram's syndrome].

Ned Tijdschr Geneeskd

HagaZiekenhuis, locatie Juliana Kinderziekenhuis, Den Haag, The Netherlands.

Published: June 2010

Wolfram's syndrome is a rare neurodegenerative disorder, which usually first manifests itself around the age of 6 years. The diagnosis can be made based on the characteristics incorporated in the 'DIDMOAD' acronym: diabetes insipidus, diabetes mellitus, optic atrophy and deafness. We present 2 boys, diagnosed with diabetes mellitus at the age of 5 and 4 years respectively. Both children developed optic atrophy over the years. These 2 cases illustrate that alongside diabetic retinopathy, possible syndromes, such as Wolfram's syndrome, should also be considered in children with diabetes mellitus and visual impairment.

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