DIDMOAD syndrome

Classification according to ICD-10
E10.7	Primarily insulin-dependent diabetes mellitus [type 1 diabetes] - with multiple complications
H48.0 *	Optic Atrophy in Diseases Classified Elsewhere
ICD-10 online (WHO version 2019)

The DIDMOAD syndrome (or Wolfram syndrome ) is a mitochondrial and pronounced form of diabetes mellitus .

clinic

DIDMOAD is an English acronym and stands for the description of the characteristics typical for this syndrome:

• Diabetes insipidus
• Diabetes mellitus
• optical atrophy ( optic nerve - atrophy )
• deafness ( numbness )

The patients also develop diabetic neuropathy . The disease begins in the first decade of life and the course is progressive. Around a quarter of patients also develop epilepsy in the course of the disease .

genetics

Wolfram syndrome is an autosomal recessive inherited disease and is caused by mutations in the wolframine gene (WFS1) on the short arm of chromosome 4 (4p16.1).

Differential diagnostics

The Rosenberg-Chutorian syndrome , Rogers syndrome and Hagemoser-Weinstein-Bresnick syndrome are to be distinguished .

Individual evidence

1. ↑ Hamid Emminger, Thomas Kia (ed.): Exaplan. Volume 2, 7th edition. Urban & Fischer, Munich 2011, ISBN 978-3-437-42464-9 , p. 1720.
2. ↑ A. Chaussenot, S. Bannwarth, C. Rouzier et al: Neurologic features and genotype-phenotype correlation in Wolfram syndrome . In: Ann Neurol . tape 69 , 2011, p. 501-508 .

literature

• TG Barrett, SE Bundey: Wolfram (DIDMOAD) syndrome. In: J Med Genet. 34 (10), Oct 1997, pp. 838-841. Review. PMID 9350817

Web links

DIDMOAD syndrome. In: Orphanet (Rare Disease Database).

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !