title: Characteristics of the dementia in late-onset metachromatic leukodystrophy.
abstract: Patients with metachromatic leukodystrophy (MLD) of juvenile or adult onset present with behavioral abnormalities. In nine patients, diagnosed between ages 11 and 33 years, behavior and neuropsychological test results disclosed a pattern of dementia combining features associated with both frontal and white matter abnormalities. All the patients had been considered to have a psychiatric disorder prior to the diagnosis of MLD, even though none had any of the cardinal features of schizophrenia or other major psychosis. Early diagnosis of late-onset MLD is important to provide access to appropriate effective therapy.
authors.affiliation: Department of Neurology, University of Minnesota, Minneapolis 55455.
authors.forename: E G
authors.initials: EG
authors.lastname: Shapiro
authors.forename: L A
authors.initials: LA
authors.lastname: Lockman
authors.forename: D
authors.initials: D
authors.lastname: Knopman
authors.forename: W
authors.initials: W
authors.lastname: Krivit
mesh_terms: D000293:Adolescent; D000328:Adult; D001519:Behavior; D002648:Child; D003704:Dementia / Q000209:etiology* / Q000503:physiopathology / Q000523:psychology; D005260:Female; D006801:Humans; D007966:Leukodystrophy, Metachromatic / Q000175:diagnosis / Q000503:physiopathology / Q000523:psychology*; D008279:Magnetic Resonance Imaging; D008297:Male; D009420:Nervous System / Q000503:physiopathology; D009483:Neuropsychological Tests