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Original Paper

Cognitive Alterations in Non-Demented CADASIL Patients

Taillia H.a · Chabriat H.a · Kurtz A.a · Verin M.d · Levy C.a,b · Vahedi K.a · Tournier-Lasserve E.b,c · Bousser M.G.a

Author affiliations

Services dea Neurologie et b Radiologie, Hôpital Saint-Antoine, c INSERM U25, Faculté de Médecine Necker, Paris, and d Service de Neurologie, CHU Rennes, France

Related Articles for ""

Cerebrovasc Dis 1998;8:97–101

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Article / Publication Details

First-Page Preview
Abstract of Original Paper

Published online: March 05, 1998
Issue release date: March – April

Number of Print Pages: 5
Number of Figures: 1
Number of Tables: 2

ISSN: 1015-9770 (Print)
eISSN: 1421-9786 (Online)

For additional information: http://www.karger.com/CED

Abstract

CADASIL is an inherited small-artery disease of the brain due to mutations of the Notch3 gene on chromosome 19. It is characterized by strokes, migraine with aura, and severe mood disturbances during mid adulthood and leads progressively to subcortical dementia. The precise onset of the cognitive decline in CADASIL remains unknown. We report here the cognitive evaluation of 8 non-demented symptomatic patients with CADASIL from 35 to 66 years of age. Altered performances were found in all subjects with the Wisconsin Card-Sorting Test (WCST), in 5/8 with the Trail-Making Test, and in 3/8 with copying of Rey’s figure. Altered performances with codes and similarities of the WAIS-R, the Wechsler Memory Scale, Raven’s Progressive Matrices, and the category and letter fluency task were observed less frequently (n ≤2). The score obtained with the WCST was not significantly correlated with the severity of the white-matter or basal ganglia signal abnormalities at MRI examination. Our data show that: (1) symptomatic CADASIL patients, although non-demented, can present with a subtle cognitive impairment; (2) tasks involving the frontal lobes are found most frequently altered, and (3) this subtle cognitive deficit can develop in the absence of major vascular events and does not appear to be correlated with the severity of brain lesions as seen at MRI examination.


Article / Publication Details

First-Page Preview
Abstract of Original Paper

Published online: March 05, 1998
Issue release date: March – April

Number of Print Pages: 5
Number of Figures: 1
Number of Tables: 2

ISSN: 1015-9770 (Print)
eISSN: 1421-9786 (Online)

For additional information: http://www.karger.com/CED


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