• Logo
  • SBMUJournals

DIDMOAD (Wolfram) Syndrome

Masoud Nashibi, Ardeshir Tajbakhsh, Solmaz Mahdavipour Vahdati, Farhad Safari, Kamran Mottaghi




Wolfram syndrome was first described by physician D J Wolfram and Wagener in 1938. This autosomal recessive syndrome is also referred to as DIDMOAD syndrome which stands for Diabetes Insipidus, Insulin Dependent Diabetes Mellitus, Optic Atrophy and Deafness


Anesthesia, Cochlear Implantation, Wolfram Syndrome, Wolframin protein


Eller P, Foger B, Gander R, Sauper T, Lechleitner M, Finkenstedt G, et al. Wolfram syndrome: a clinical and molecular genetic analysis. Journal of medical genetics. 2001;38(11):E37.

Hilson JB, Merchant SN, Adams JC, Joseph JT. Wolfram syndrome: a clinicopathologic correlation. Acta neuropathologica. 2009;118(3):415-28.

Barrett TG, Bundey SE. Wolfram (DIDMOAD) syndrome. Journal of medical genetics. 1997;34(10):838-41.

d'Annunzio G, Minuto N, D'Amato E, de Toni T, Lombardo F, Pasquali L, et al. Wolfram syndrome (diabetes insipidus, diabetes, optic atrophy, and deafness): clinical and genetic study. Diabetes care. 2008;31(9):1743-5.

Matsunaga K, Tanabe K, Inoue H, Okuya S, Ohta Y, Akiyama M, et al. Wolfram syndrome in the Japanese population; molecular analysis of WFS1 gene and characterization of clinical features. PloS one. 2014;9(9):e106906.

Kanki T, Klionsky DJ. Mitochondrial abnormalities drive cell death in Wolfram syndrome 2. Cell research. 2009;19(8):922-3.

Chen YF, Kao CH, Chen YT, Wang CH, Wu CY, Tsai CY, et al. Cisd2 deficiency drives premature aging and causes mitochondria-mediated defects in mice. Genes & development. 2009;23(10):1183-94.

El-Shanti H, Lidral AC, Jarrah N, Druhan L, Ajlouni K. Homozygosity mapping identifies an additional locus for Wolfram syndrome on chromosome 4q. American journal of human genetics. 2000;66(4):1229-36.

Fabbri LP, Nucera M, Grippo A, Menicucci A, De Feo ML, Becchi C, et al. Wolfram syndrome. How much could knowledge challenge the fate? A case report. Medical science monitor : international medical journal of experimental and clinical research. 2005;11(7):CS40-4.

Masoud Nashibi, Ardeshir Tajbakhsh, Farhad Safari, Kamran Mottaghi. Anesthetic Consideration of Niemann- Pick Disease Type C. Journal of Cellular & Molecular Anesthesia [Case Report]. 2016;1(2):73-7.

DOI: https://doi.org/10.22037/jcma.v1i3.12545


  • There are currently no refbacks.