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Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy: Progression of MR Abnormalities in Prospective 7-year Follow-up Study¹

¹ From the Departments of Radiology (M.K.L., I.L.v.d.N., R.v.d.B., M.A.v.B., J.v.d.G.), Clinical Genetics (S.A.J.L.O.), and Neurology (J.H., M.D.F.), Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, the Netherlands; and Department of Neurology, Rijnland Hospital, Leiderdorp, the Netherlands (J.H.). Received February 22, 2008; revision requested April 3; revision received April 28; accepted May 21; final version accepted June 3. Address correspondence to M.K.L. (e-mail: m.k.liem{at}lumc.nl).

Purpose: To prospectively investigate the patterns and rates of progression of magnetic resonance (MR) imaging abnormalities in a well-documented cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) cohort 7 years after baseline and to identify the prognostic factors that determine the rates and patterns of this progression.

Materials and Methods: The local ethics committee approved the study, and informed consent was obtained from all participants. From 12 unrelated families, 25 patients who were NOTCH3 mutation carriers and 13 who were non–mutation carriers were examined clinically and with standardized MR imaging at baseline and after 7 years. The progression of white matter hyperintensities (WMHs), lacunar infarcts, microbleeding, and brain volume loss was measured semiquantitatively. Correlation testing and group comparison testing were performed to identify the risk factors associated with increased progression of CADASIL-related MR abnormalities.

Results: Compared with the non–mutation carriers, the mutation carriers showed significant increases in numbers of lacunar infarct (P < .01), WMH (P < .01), and microbleed (P < .05) lesions but no increased loss of brain volume. The distributions of new WMHs and new lacunar infarcts at follow-up were similar to the distributions of these abnormalities at baseline. High WMH (P < .05), lacunar infarct (P < .01), and microbleed (P < .01) lesion loads at baseline—but not cardiovascular risk factors—were associated with faster progression of these abnormalities.

Conclusion: Patients with CADASIL who have a high MR abnormality lesion load at baseline are at risk for faster progression of MR abnormalities.

© RSNA, 2008