TY - CHAP M1 - Book, Section TI - Wolfram Syndrome A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas Y1 - 2019 N1 - T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - This is a congenital medical condition characterized by diabetes mellitus (DM) and insipidus (DI) with optic nerve atrophy, mental retardation, and deafness. It is also called DIDMOAD, an acronym that stands for Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness. This disorder affects mostly the central nervous system, especially the brain stem. The onset of the disease is during childhood. The first symptom is typically DM, which usually appear at the age of six. Optic atrophy is the second manifestation and the median age is 11 years old. The first retinal signs are loss of color and peripheral vision. This medical condition leads to blindness within 8 years from the initial symptoms. Life expectancy is approximately 30 years with a mortality rate about 65% before the age of 35 years. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accessanesthesiology.mhmedical.com/content.aspx?aid=1164088364 ER -