Definition:
Wolfram syndrome otherwise known as the DIDMOAD which is the acronym for the Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness, generally a genetic disorder classified as a neurodegenerative disease which affects the brain and the central nervous system. It was first describe in 1938 by Dr. Don J. Wolfram, M.D. (Wolfram, D. J.; Wagener, H. P: Diabetes mellitus and simple optic atrophy among siblings: report of four cases. Mayo Clin. Proc. 13: 715-718, 1938).
Diagnosis:
Diagnosis includes the analization of theWFS1 gene and the examination of the family history of the patient.
Treatment:
There is no known treatment for the Wolfram Syndrome but treatment may focus more on the symptoms as they manifests.
Symptoms and Signs:
Its symptoms includes the occurrence of Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness.
Causes:
It is said to be caused by the malfunction of the mitochondria and of the myelination which is similar to multiple sclerosis. It is an autosomal recessive or dominant or mitochondrial inherited medical condition. Usually it is caused by the mutation in the gene that is said to be responsible for the production of wolframin, a protein, that results in the loss of its function.
