BACKGROUND: Cerebral autosomal dominant arteriopathy with subcortical infarcts 
and leukoencephalopathy (CADASIL) is increasingly recognized as an inherited 
arterial disease leading to a step-wise decline and eventually to dementia. 
CADASIL is caused by mutations in NOTCH3 epidermal growth factor-like repeat 
that maps to chromosome 19. CADASIL cases have been identified in most countries 
of Western and Central Europe, the Americas, Japan, Australia, the Caribbean, 
South America, Tanzania, Turkey, South Africa and Southeast Asia, but not in 
Arabs.
METHODS: We studied three families from Saudi Arabia (Family A), Kuwait (Family 
B) and Yemen (Family C) with 19 individuals affected by CADASIL.
RESULTS: The mean age of onset was 31 +/- 6 and the clinical presentation 
included stroke in 68%, subcortical dementia in 17% and asymptomatic 
leukoariosis detected by MRI in 15%. Migraine and depression were frequently 
associated, 38% and 68% respectively. The mean age of death was 56 +/- 11. All 
NOTCH3 exons were screened for mutations, which revealed the presence of 
previously reported mutations c.406C>T (p.Arg110>Cys) in two families (family 
A&B) and c.475C>T (p.Arg133>Cys) mutation in family C.
CONCLUSION: CADASIL occurs in Arabs, with clinical phenotype and genotype 
similar to that in other ethnic groups.