A 15 year old boy has bilateral optic atrophy, diabetes mellitus and diabetes insipidus. The diagnosis is-: (A) Kjer syndrome, (B) Behr syndrome, (C) Wolfram syndrome, (D) None
Answer is C. The Wolfram syndrome is a hereditary optic atrophy. It presents between the ages of 5-21 years. This is also referred to as "DIDMOAD" i.e. Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy and Deafness.