1.
Maghreb Medical. 2008; 28 (389): 141-142
in French
| IMEMR
| ID: emr-134664
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