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CADASIL
Cerebral autosomal dominant arteriopathy with subcortical infarcts
and leukoencephalopathy (CADASIL) is an inherited small vessel disease
causing stroke and dementia. This condition causes repeated ischemic
attacks and is related to mutations in the NOTCH3 gene. This condition
affects small blood vessels, mainly in the brain. An abnormality
in the muscle cells surrounding these blood vessels (vascular smooth
muscle cells) gradually destroys the blood vessel cells. The resulting
blood vessel damage can lead to migraines, emotional and mental
disorders, stroke-like episodes, dementia, and other impairments
of normal brain function. Patients with CADASIL are also at increased
risk of heart attack (myocardial infarction) because of damaged
blood vessels in the heart. Most patients with CADASIL do not have
the common risk factors for stroke and heart attack, such as high
blood pressure and high cholesterol, although in some cases these
features may also be present. DNA tests are available.
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