Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL)
CADASIL is a genetic form of subcortical vascular dementia. It is a disease of small arteries, particularly in the brain, and is associated with damage to the brain's white matter due to loss of blood supply. The disease starts during mid-adulthood and is characterized by recurrent stoke-like events (transient or permanent), attacks of migraine with aura, severe mood disorders (usually depression), and dementia. MRI scans of the brain show extensive areas of diseased white matter. CADASIL is an autosomal dominant syndrome with 100% penetrance. This means that a child of an afflicted parent has a 50% chance of having the genetic abnormality and that if the genetic abnormality is present, there is a 100% chance of developing the disorder. Because CADASIL is the first genetic form of vascular dementia and depression with an identified gene, future studies may aid us in better understanding of cerebral arteriosclerosis (hardening of the arteries in the brain), a common cause of disability in the elderly.
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