AJDRAJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 2009;30:1244.

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American Journal of Neuroradiology
DOI 10.3174/ajnr.A1533

BRAIN

Cerebrovascular Reactivity Is a Main Determinant of White Matter Hyperintensity Progression in CADASIL

M.K. Liem, S.A.J. Lesnik Oberstein, J. Haan, R.v.d. Boom, M.D. Ferrari, M.A.v. Buchem and J.v.d. Grond

From the Departments of Radiology (M.K.L., 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, Leiden, the Netherlands; and Department of Neurology (J.H.), Rijnland Hospital, Leiderdorp, the Netherlands.

Please address correspondence to Dr. M.K. Liem, Department of Radiology, Leiden University Medical Center, C2S, Albinusdreef 2, 2333 ZA Leiden, the Netherlands; e-mail: m.k.liem{at}lumc.nl

BACKGROUND AND PURPOSE: Basal total cerebral blood flow (TCBF) and cerebrovascular reactivity (CVR) are assumed to play an important role in the pathophysiology of small-vessel disease. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a unique monogenetic model to study the pathophysiology of arterial small-vessel disease. The aim of this study was to investigate the role of TCBF and CVR in the progression of MR imaging abnormalities in CADASIL.

MATERIALS AND METHODS: Basal TCBF was measured in 25 NOTCH3 mutation carriers and 13 control subjects at baseline. CVR after administration of acetazolamide was measured in 14 NOTCH3 mutation carriers and 9 control subjects. Increase in white matter hyperintensities (WMHs), lacunar infarcts, and microbleeds on MR imaging was measured 7 years later.

RESULTS: Lower CVR at baseline was associated with larger increase of WMHs (P = .001) but not with a larger increase of lacunar infarcts or microbleeds. TCBF at baseline was not associated with an increase of MR imaging abnormalities.

CONCLUSIONS: Decreased CVR is a potential predictor of disease progression as indicated by increasing WMHs in CADASIL.






Copyright © 2009 by the American Society of Neuroradiology. Print ISSN: 0195-6108 Online ISSN: 1936-959X