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Titolo:
CEREBRAL AUTOSOMAL-DOMINANT ARTERIOPATHY WITH SUBCORTICAL INFARCTS AND LEUKOENCEPHALOPATHY - STUDY OF 2 AMERICAN FAMILIES WITH PREDOMINANT DEMENTIA
Autore:
HEDERA P; FRIEDLAND RP;
Indirizzi:
UNIV HOSP CLEVELAND,DEPT NEUROL,LAB NEUROGERIATR,10990 EUCLID AVE CLEVELAND OH 44106 UNIV HOSP CLEVELAND,DEPT NEUROL,LAB NEUROGERIATR CLEVELAND OH 44106 CASE WESTERN RESERVE UNIV CLEVELAND OH 44106
Titolo Testata:
Journal of the neurological sciences
fascicolo: 1, volume: 146, anno: 1997,
pagine: 27 - 33
SICI:
0022-510X(1997)146:1<27:CAAWSI>2.0.ZU;2-8
Fonte:
ISI
Lingua:
ENG
Soggetto:
BEHAVIORAL-CHANGES; ALZHEIMER-DISEASE; CADASIL; STROKE; LESIONS; ENCEPHALOPATHY; CHROMOSOME-19; TOMOGRAPHY;
Keywords:
STROKE; HEREDITARY; DEMENTIA; CADASIL; ARTERIOPATHY;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
31
Recensione:
Indirizzi per estratti:
Citazione:
P. Hedera e R.P. Friedland, "CEREBRAL AUTOSOMAL-DOMINANT ARTERIOPATHY WITH SUBCORTICAL INFARCTS AND LEUKOENCEPHALOPATHY - STUDY OF 2 AMERICAN FAMILIES WITH PREDOMINANT DEMENTIA", Journal of the neurological sciences, 146(1), 1997, pp. 27-33

Abstract

Few European families have been reported with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). We describe four patients from two independent American families. All four cases underwent comprehensive clinical, neuropsychological and pathological examination. Pathological data were correlated with clinical features. Dementia was a prominent and constant feature in all subjects. The families differed in phenotypical presentation and we analyzed possible pathological substrates that may account for the differences. Autopsy showed multiple ischemic infarcts in the white matter, abnormal vasculature with thickening and degeneration of the vessel wall. The clinical course in the first family was characterized by early dementia without stroke-like episodes; however, autopsy demonstrated strokes in the basal ganglia and thalamus. The members of the second family developed dementia later and had history of several clinically evident strokes. Pathological examination showed only widespread degeneration of the white matter. Our study of two American families with CADASIL suggests that involvement of the basal ganglia and thalamusis important for early development of dementia and clinically can present as a gradual dementia, resembling a neurodegenerative process. Selective damage of the white matter and central gray matter provides further insight to the pathogenesis of vascular dementia. (C) 1997 Elsevier Science B.V.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 24/11/20 alle ore 14:37:22