The review presents data on etiology, clinical presentation, results of additional research methods, differential diagnosis and possible pathogenetic mechanisms of neurodegeneration syndrome CADASIL. Despite the fact that research for the causes and mechanisms of development of this pathology is increasing every year, still one can not drill its molecular links, which means that specific therapy remains elusive. Therefore, it is necessary further study of molecular polymorphism of neurodegeneration, models development and identifying new approaches to the reconstruction and analysis of this pathology.
