Encyclopedia of Clinical Neuropsychology

2018 Edition
| Editors: Jeffrey S. Kreutzer, John DeLuca, Bruce Caplan


  • Angela EastvoldEmail author
  • Yana Suchy
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-57111-9_500


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

  1. 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.CrossRefGoogle Scholar
  2. 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.PubMedCrossRefPubMedCentralGoogle Scholar
  3. 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.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of NeurologyThe University of UtahSalt Lake CityUSA
  2. 2.Department of PsychologyThe University of UtahSalt Lake CityUSA