Articles
Diabetes in Wolfram Syndrome: Recognition, Genetics, and Clinical pearls for clinicians
Wolfram syndrome is a rare, autosomal recessive neurodegenerative disorder in which early-onset insulin-dependent diabetes mellitus is a hallmark feature. Recognizing the diabetes–optic atrophy combination and understanding WFS1-related biology helps guide evaluation, genetic testing, and anticipatory care for affected patients.
Overview
Wolfram syndrome (often abbreviated DIDMOAD for Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness) is a rare inherited condition in which insulin-requiring diabetes mellitus commonly appears in childhood and is typically followed by other neuroendocrine and neurological problems [Source 1, Source 3].
Typical clinical trajectory