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MRI correlates of cognitive decline in CADASIL

A 7-year follow-up study

From the Departments of Radiology (M.K.L., I.L.v.d.N., M.A.v.B., J.v.d.G.), Clinical Genetics (S.A.J.L.O.), Neurology (J.H., M.D.F.), and Neuropsychology (H.A.M.M.), Leiden University Medical Center; the Department of Neurology (J.H.), Rijnland Hospital, Leiderdorp; and the Department of Psychology (H.A.M.M.), Leiden University, The Netherlands.

Address correspondence and reprint requests to Dr Liem, Department of Radiology, Leiden University Medical Center, C2S, Albinusdreef 2, 2333 ZA Leiden, the Netherlands m.k.liem{at}lumc.nl

Background: Cognitive decline is one of the clinical hallmarks of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), a cerebrovascular disease caused by NOTCH3 mutations. In this 7-year follow-up study, we aimed to determine whether there are associations between the different radiologic hallmarks in CADASIL and decline in specific cognitive domains.

Methods: Twenty-five NOTCH3 mutation carriers and 13 controls had standardized neuropsychological testing and MRI examinations at baseline and after a follow-up of 7 years. To identify longitudinal associations between MRI abnormalities and cognitive decline, correlation analysis was used.

Results: At follow-up, mutation carriers showed a decline in global cognitive function (CAMCOG, p < 0.01) and in the cognitive domains language, memory, and executive function, compared to controls. Cognitive decline, especially executive dysfunction, was associated with increase in lacunar infarcts, microbleeds, and ventricular volume. In contrast, WMHs and brain atrophy were not associated with cognitive decline.

Conclusion: Increase in lacunar infarcts, microbleeds, and ventricular volume, but not white matter lesions or atrophy, are associated with cognitive decline in the process of CADASIL in younger-aged, mildly affected patients with CADASIL.

Abbreviations: ANOVA = analysis of variance; CADASIL = cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy; CAMCOG = Cambridge Cognitive Examination; DSM-IV = Diagnostic and Statistical Manual of Mental Disorders, 4th edition; MC = mutation carrier; MMSE = Mini-Mental State Examination; nonMC = non-mutation carrier; WMH = white matter hyperintensity; WMS = Wechsler Memory Scale.

Supplemental data at www.neurology.org

Disclosure: The authors report no disclosures.

Received May 13, 2008. Accepted in final form October 2, 2008.

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