Advanced

AJNR - American Journal of Neuroradiology

Published ahead of print on March 6, 2009
doi: 10.3174/ajnr.A1533

This Article Figures Only Full Text Full Text (PDF) All Versions of this Article: ajnr.A1533v1 ajnr.A1533v2 30/6/1244    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Liem, M.K. Articles by Grond, J.v.d. Search for Related Content PubMed PubMed Citation Articles by Liem, M.K. Articles by Grond, J.v.d.

American Journal of Neuroradiology 30:1244-1247, June-July 2009
© 2009 American Society of Neuroradiology

BRAIN

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

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

^a Departments of Radiology, Leiden University Medical Center, Leiden, the Netherlands
^b Clinical Genetics, Leiden University Medical Center, Leiden, the Netherlands
^c Neurology, Leiden University Medical Center, Leiden, the Netherlands
^d Department of Neurology, 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.

This article has been cited by other articles:

				A. Stam, J Haan, A. van den Maagdenberg, M. Ferrari, and G. Terwindt Migraine and Genetic and Acquired Vasculopathies Cephalalgia, September 1, 2009; 29(9): 1006 - 1017. [Abstract] [Full Text] [PDF]