The review by Vlisides and Moore did not mention a recently identified group of genetic disorders posing a significant risk for stroke, the most common of which is CADASIL (Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy).2,3  CADASIL is caused by an autosomal dominant defect in the NOTCH3 gene, causing abnormal, fragile vascular smooth muscle and resulting in early stroke and dementia in a genetic pattern similar to Huntington disease: 1 per 10,000 prevalence with 50% of offspring affected in mid-adulthood. Although a large hospital will likely encounter several CADASIL patients each year, the disease is rarely diagnosed or reported.

CADASIL is readily identified by the history, and so I have added two questions to my preoperative questionnaire: “Do you have blood relatives with stroke and dementia before the age of 60?”...

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