[Wolfram syndrome about 2 family cases]

ABSTRACT

Wolfram syndrome or DIDMOAD syndrome is an hereditary optic atrophy occurring in insulin-dependent diabetics. It can be associated with other endocrinal, ORL, neurologic or urologic disorders, We report 2 cases brother and sister presenting 2 principal signs of this syndrome optic atrophy and insulin-dependent diabetes. The ophthalmologic exam shows a visual acuity at stade of blindness. The genetic inquiry shows a parental consanguinity and 2 other supposed dead cases affected in the family