To the Editor:Wolfram syndrome (WS,MIM 222300) is a rare autosomal recessive disorder caused by mutations in WFS1 or CISD2 (WFS2).Its prevalence is estimated to be one in 160,000 to 770,000.[1] Patients usually present with insulin-dependent diabetes mellitus (DM) at around 6 years old and then with optic atrophy (OA) at around 11 years old.Other symptoms,such as diabetes insipidus,sensorineural deafness,urinary tract abnormalities,and neuropsychiatric disorders,occur at older ages and affect a fraction of individuals.The expected age at death is approximately 30 to 40 years,mostly caused by respiratory failure.WS is inherited in an autosomal recessive manner,while some pathogenic mutations in the WFS1 gene are dominant,are associated with hearing impairment,OA,DM,and psychiatric problems,and cause Wolfram-like syndrome.[2]In the present report,we describe a case of WFS1-related spectrum disorder onset with non-insulin-dependent DM and OA in a Chinese patient.This patient had compound heterozygous mutations in the WFS1 gene,and a novel nonsense mutation,c.2217C＞A,was found.