Synonyms
Agnogenic medial arteriopathy; Chronic familial vascular encephalopathy; Familial Binswanger’s disease; Familial disorder with subcortical ischemic strokes, dementia, and leukoencephalopathy; Hereditary multi-infarct dementia
Short Description or Definition
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a rare (prevalence 4.6 per 100,000) autosomal dominant, adult-onset inherited cerebral vascular disease, characterized by migraines, recurrent transient ischemic attacks, and strokes leading to cognitive decline and dementia.
Current Knowledge
Clinical manifestation: The typical age of onset is 30–50 years of age. Although there is considerable variability among patients, the initial clinical manifestation often begins with migraines with aura and slowly progresses to transient ischemic attacks (TIA) and recurrent strokes. The migraine attacks are often reported to be particularly long lasting or severe and may even...
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References and Readings
Kalaria, R. N., Viitanen, M., Kalimo, H., Dichgans, M., & Tabira, T. (2004). The pathogenesis of CADASIL: An update. Journal of Neurological Sciences, 226, 35–39.
Kalimo, H., Ruchoux, M. M., Viitanen, M., & Kalaria, R. N. (2002). CADASIL: A common form of hereditary arteriopathy causing brain infarcts and dementia. Brain Pathology, 12(3), 371–384.
Moreno, F. C., Raxvi, S. S., Davidson, R., & Muir, K. W. (2014). Changing clinical patterns and increasing prevalence in CADASIL. Acta Neurologica Scandinavica, 130(3), 197–203.
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Eastvold, A., Suchy, Y. (2018). CADASIL. In: Kreutzer, J.S., DeLuca, J., Caplan, B. (eds) Encyclopedia of Clinical Neuropsychology. Springer, Cham. https://doi.org/10.1007/978-3-319-57111-9_500
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DOI: https://doi.org/10.1007/978-3-319-57111-9_500
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Publisher Name: Springer, Cham
Print ISBN: 978-3-319-57110-2
Online ISBN: 978-3-319-57111-9
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