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Other Names for this Disease
- Cerebral arteriopathy with subcortical infarcts and leukoencephalopathy
- Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy
- Dementia, hereditary multi-infarct type
- Familial vascular leukoencephalopathy
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Strokes are the main feature of the disease. The mean age at onset for stroke-like episodes is 46 years. These stroke-like episodes are often recurrent, leading to severe disability with inability to walk and urinary incontinence. Cognitive deficits, the second most frequent feature, are observed in over half of symptomatic individuals and may start as early as age 35 years. Most experience slowly progressive cognitive decline and approximately 75% of affected individuals develop dementia (severe deficits in executive functioning, verbal fluency, and memory). Migraines with aura occur in about 35% of individuals with CADASIL, with the first attack occurring at a mean age of 26 years. Thirty percent of individuals with CADASIL experience psychiatric disturbance, varying from personality changes to severe depression. Epilepsy is present in 10% of individuals with CADASIL and presents at middle age.
Last updated: 3/22/2011
- Lesnik Oberstein SAJ, Boom EMJ, Dichgans M. CADASIL. GeneReviews. July 23, 2009; http://www.ncbi.nlm.nih.gov/books/NBK1500/. Accessed 3/22/2011.