Abstract

Bouziane Soukaina*, Hassimi Ouail, Bennis Ahmed, Chraibi Fouad, Abdellaoui Meriem and
Benatiya Andaloussi Idris 

ABSTRACT

Wolfram syndrome is a rare autosomal recessive neurodegenerative disorder characterized by the onset of insulin-dependent diabetes mellitus in childhood associated with bilateral optic atrophy that often results in blindness. Diabetes insipidus, deafness, psychiatric disorders, anosmia, urinary tract abnormalities, nystagmus, ataxia, and myoclonus are less common. We report a case of Wolfram syndrome, diagnosed in a 12-year-old boy with a history of insulin- dependent diabetes. The work-up was motivated by the occurrence of a bilateral and progressive visual acuity decrease related to optic atrophy and peripheral visual field constriction.