Clinical autosomal dominating arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)
Mendel, T.; Członkowska, A.
Neurologia i Neurochirurgia Polska 33(5): 1117-1128
1999
ISSN/ISBN: 0028-3843 PMID: 10672562 Document Number: 499191
CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is a diffuse disease of small arteries, predominating in the brain. It starts during mid-adulthood and is characterized by recurrent ischaemic events (transient or permanent), attacks of migraine with aura, severe mood disorders, subcortical dementia and at MRI white periventricular leukoencephalopathy. CADASIL is an autosomal dominant disease. The gene Notch3 on which the mutation was detected is located on chromosome 19. There is so far no specific treatment and death occurs after a mean of twenty years.