Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) as a model of small vessel disease: update on clinical, diagnostic, and management aspects.
Published version
Peer-reviewed
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Repository DOI
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Authors
Di Donato, Ilaria
Bianchi, Silvia
De Stefano, Nicola
Dichgans, Martin
Dotti, Maria Teresa
Abstract
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common and best known monogenic small vessel disease. Here, we review the clinical, neuroimaging, neuropathological, genetic, and therapeutic aspects based on the most relevant articles published between 1994 and 2016 and on the personal experience of the authors, all directly involved in CADASIL research and care. We conclude with some suggestions that may help in the clinical practice and management of these patients.
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Keywords
CADASIL, Genetics, NOTCH 3, Small vessel disease, Vascular dementia, CADASIL, Cerebral Small Vessel Diseases, Humans
Journal Title
BMC Med
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Journal ISSN
1741-7015
1741-7015
1741-7015
Volume Title
Publisher
Springer Science and Business Media LLC