A Sayouti, R Benhaddou, R Khoumiri, L Gaboune, H Guelzim, N Benfdil, A Moutaoukil
Wolfram syndrome is a rare autosomal recessive neurodegenerative disease; it is characterized by the appearance of diabetes mellitus in childhood associated with bilateral optic atrophy that often leads to blindness. Insipid diabetes, deafness, psychiatric disorders, anosmia, anomalies of the urinary tract, nystagmus, ataxia, and myoclonias are less frequent. We report two cases of Wolfram syndrome, diagnosed in a 12-year-old girl and a 13-year-old boy. In each case, there was a history of diabetes mellitus; they consulted for a progressive loss of vision...
June 2007: Journal Français D'ophtalmologie