C455R notch3 mutation in a Colombian CADASIL kindred with early onset of stroke.

Arboleda-Velasquez, J F; Lopera, F; Lopez, E; Frosch, M P; Sepulveda-Falla, D; Gutierrez, J E; Vargas, S; Medina, M et al. · Neurology · 2002

case_report · Level V

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Abstract

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is caused by mutations in the notch3 epidermal growth factor-like repeats. A Colombian kindred carries a novel C455R mutation located in the predicted ligand-binding domain. Stroke occurred in the patients at an unusually early age (median age: 31 years) in comparison to the more frequent onset in the fourth decade of life in other CADASIL populations, including a second Colombian kindred with an R1031C mutation.

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