What does Wolfram syndrome affect?

What does Wolfram syndrome affect?

Wolfram syndrome , which is also known by the acronym DIDMOAD, is an inherited condition characterized by diabetes insipidus (DI), childhood-onset diabetes mellitus (DM), a gradual loss of vision caused by optic atrophy (OA), and deafness (D).

Is Wolfram syndrome inherited?

Wolfram syndrome is caused by mutations in the WFS1 (most common) or WFS2 (CISD2) gene that are inherited in an autosomal recessive pattern in most affected individuals, although dominant forms exist.

What is dominant atrophy?

DEFINITION OF THE DISEASE: Dominant Optic Atrophy (DOA) is a neuro-ophthalmic condition characterized by a bilateral degeneration of the optic nerves, causing insidious visual loss, typically starting during the first decade of life.

What kind of disease does Wolfram syndrome cause?

Another name for the syndrome is DIDMOAD, which refers to diabetes insipidus, diabetes mellitus, optic atrophy, and deafness. Most cases of Wolfram syndrome are caused by changes (mutations) in the WFS-1 gene.

How old do you have to be to have Wolfram syndrome?

All the features that give Wolfram syndrome the acronym DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness) are observed in 65% of people, while others only have some of the associated health problems. Most affected people develop diabetes mellitus and optic atrophy before age 15 years.

How is diabetes insipidus treated with Wolfram syndrome?

Treatment of Wolfram syndrome is symptomatic and supportive. It requires a multidisciplinary effort to manage the various aspects of this condition. When diabetes mellitus is present, the patient will need insulin treatment. Diabetes insipidus can be difficult to diagnose and may need to be treated with intranasal or oral dDAVP.

Is the optic atrophy in Wolfram syndrome the same?

The optic atrophy in LHOA and the optic atrophy in Wolfram syndrome may look the same and have the same symptoms. LHOA can start in one eye or both, but both eyes are usually affected within six months. In most people, vision loss is permanent.