Wolfram Syndrome (DIDMOAD)

Definition

Wolfram syndrome is a rare autosomal recessive neurodegenerative disease characterized by Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy and Hearing Loss (DIDMOAD).

Symptoms

  • Childhood-onset diabetes
  • Optic atrophy (vision loss)
  • Diabetes insipidus
  • Sensorineural hearing loss

Reasons

Mutation in the WFS1 gene.

Diagnosis

Clinical criteria, genetic testing.

Treatment

Symptomatic treatment (insulin, desmopressin), supportive care.

Which Polyclinic Should You Apply To?

Endocrinology Polyclinic or Neurology Polyclinic

What to Do in an Emergency?

In case of diabetic ketoacidosis or severe dehydration, emergency services should be consulted.

This page is for general information purposes only.