What is Wolfram Syndrome?
A syndrome is the name given to a condition where features occur in a consistent pattern, and where the cause is not understood. Wolfram syndrome is named after a Dr Wolfram, who in 1938 described 4 brothers and sisters from the same family with diabetes mellitus and optic atrophy. He worked in the Mayo Clinic in North America, and since then over 200 patients have been described in the world medical literature. It is also called DIDMOAD syndrome, after 4 common features (Diabetes Mellitus, Diabetes Insipidus, Optic Atrophy, and Deafness). How common is it? Wolfram syndrome affects about 1 in 770,000 of the total UK population, or 1 in 500,000 children, so it is very rare. A consultant paediatrician may only see one affected child in a professional lifetime. What may happen in the condition? Diabetes mellitus is the name given when the body cannot convert glucose or sugar to energy, because the pancreas is not making enough insulin hormone. Symptoms include thirst and frequent passing
