Psychiatric findings in Wolfram syndrome homozygotes.
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.
Swift
R G
RG
Biological Sciences Research Center, University of North Carolina, Chapel Hill 27599.
Sadler
D B
DB
Swift
M
M
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