Cognition, mood and behavior in CADASIL.

CADASIL is the most common familial cerebral small vessel disease (cSVD). Stereotyped mutations of the NOTCH3 gene are responsible for this archetypal ischemic cSVD that can lead, at the very end stage, to severe dementia. Variable cognitive alterations, mood, or behavior disturbances are frequently observed during the course of the disease. In this review, these clinical manifestations, their occurrence, severity and duration are analyzed in relation to the disease progression. Also, the potential relationships with cerebral lesions and treatment options are discussed.