RESUMO
"CADASIL" is a genetic microangiopathy with autosomal dominant inheritance. Its epidemiology and physiopathogenesis are poorly specified, but it is proven that this disease is due to a mutation of the NOTCH3 gene resulting in a loss of elasticity of the media of the affected vessels. The clinical expression is variable, dominated by migraine attacks with aura, ischemic vascular accidents and psychiatric disorders, in particular depression. MRI is essential for diagnosis even in the pre-symptomatic phase. It shows signal abnormalities in the basal ganglia and white matter, characteristic especially when located in the anterior part of the temporal lobes. The management of CADASIL is multidisciplinary, psychological for the most part without specificity of a particular treatment.
