title: Psychiatric findings in Wolfram syndrome homozygotes.
abstract: Diabetes mellitus and bilateral optic atrophy are the defining characteristics of the autosomal recessive Wolfram syndrome. Diabetes insipidus, neurogenic bladder, deafness, and other neurological manifestations are frequent. A review was made of the medical records of 68 Wolfram syndrome patients, aged between 8 and 43 years, identified by casefinding throughout the USA. 41 of the patients (60%) had episodes of severe depression, psychosis, or organic brain syndrome, as well as impulsive verbal and physical aggression. These symptoms were very severe in 17 patients (25%), of whom 12 required admission to a psychiatric hospital and 11 attempted suicide. We conclude that the Wolfram syndrome gene predisposes homozygotes to psychiatric illness.
authors.affiliation: Biological Sciences Research Center, University of North Carolina, Chapel Hill 27599.
authors.forename: R G
authors.initials: RG
authors.lastname: Swift
authors.forename: D B
authors.initials: DB
authors.lastname: Sadler
authors.forename: M
authors.initials: M
authors.lastname: Swift
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003866:Depressive Disorder / Q000188:drug therapy / Q000209:etiology; D004198:Disease Susceptibility; D005260:Female; D006720:Homozygote; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008499:Medical Records; D001523:Mental Disorders / Q000188:drug therapy / Q000209:etiology*; D008875:Middle Aged; D019965:Neurocognitive Disorders / Q000188:drug therapy / Q000209:etiology; D011618:Psychotic Disorders / Q000188:drug therapy / Q000209:etiology; D012189:Retrospective Studies; D013406:Suicide, Attempted / Q000523:psychology; D011795:Surveys and Questionnaires; D014929:Wolfram Syndrome / Q000150:complications* / Q000235:genetics