Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy (CADASIL) Study

This is an observational study to understand better the risk factors and progression of a leading cause of vascular cognitive impairment and dementia (VCID). 500 participants will be enrolled and can expect to be on study for up to 5 years.

Inclusion Criteria for CADASIL Participants:Age at least 18 years oldPositive NOTCH 3 genetic testingWilling to commit to three in-person visits (baseline and 18-month follow-up and 36-month follow-up) as well as remote visits by phone, email, mail or internet.No unstable comorbidities (i.e., receiving stable treatment) that could affect neurological/psychiatric functionAll medications will be allowed although the protocol will mandate documentation of medications and our analyses will particularly assess potential impact of medications on outcomes (i.e., sedation of abnormal movements).Able to undergo an MRI scan and blood draw at each visitA companion who knows the participant well (>= 3 hours/month of contact) and can provide additional information (either remotely or in-person).Inclusion Criteria for Healthy Controls (HC)Will meet same criteria as CADASIL participants exceptNegative NOTCH3 genetic testingExclusion Criteria:Evidence of unstable (not stable or treated for at least 3 months) medical or psychiatric illness (including substance abuse)History of severe learning disability, mental retardation, or other central nervous system (CNS) disease or event not attributable to CADASIL (e.g., seizures, additional neurological diagnoses)History of serious alcohol or drug abuse within the past year