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Because endothelial cell abnormalities are found in white matter hyperintensities and cavernous malformations, the authors set out to determine if an increased number of white matter lesions was present in 191 patients with familial cerebral cavernous malformations all carrying the same gene defect. Results were compared with those obtained via logistic regression analysis in healthy controls and patients with sporadic cavernous malformations. White matter lesions were found in 15% of patients with the familial disease, 2% of healthy controls, and 2.5% of those with sporadic malformations. In patients with the familial disease, only age was associated with white matter lesions.