title: A kindred affected by cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). A 2-year neuropsychological follow-up.
abstract: We report a 2-year prospective neuropsychological study of five asymptomatic subjects with magnetic resonance imaging (MRI) abnormalities from an Italian kindred affected by cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). These subjects completed tests for attention capacities, processing speed, abstract thinking, short-term memory, learning and constructional praxis. Seven normal subjects matched for age and education, belonging to the same pedigree and not having MRI hyperintensities were examined as controls. The results did not show significant differences between asymptomatic subjects and normal controls. Cognitive performance of asymptomatic subjects did not deteriorate during a 2-year follow-up. Our findings suggest that, at this stage of the disease process, the presence of diffuse leukoencephalopathy does not imply subtle cognitive defects.
authors.affiliation: Fondazione Maugeri, Centro Medico di Telese, Telese Terme (BN), Italy.
authors.forename: L
authors.initials: L
authors.lastname: Trojano
authors.forename: M
authors.initials: M
authors.lastname: Ragno
authors.forename: A
authors.initials: A
authors.lastname: Manca
authors.forename: G
authors.initials: G
authors.lastname: Caruso
mesh_terms: D000328:Adult; D000368:Aged; D002539:Cerebral Arterial Diseases / Q000175:diagnosis / Q000503:physiopathology*; D003071:Cognition; D015161:Dementia, Multi-Infarct / Q000175:diagnosis / Q000503:physiopathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007968:Leukoencephalopathy, Progressive Multifocal / Q000175:diagnosis / Q000503:physiopathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008568:Memory; D008875:Middle Aged; D009483:Neuropsychological Tests; D010375:Pedigree; D011446:Prospective Studies