PT  - JOURNAL ARTICLE
AU  - M.K. Liem
AU  - S.A.J. Lesnik Oberstein
AU  - J. Haan
AU  - R.v.d. Boom
AU  - M.D. Ferrari
AU  - M.A.v. Buchem
AU  - J.v.d. Grond
TI  - Cerebrovascular Reactivity Is a Main Determinant of White Matter Hyperintensity Progression in CADASIL
AID  - 10.3174/ajnr.A1533
DP  - 2009 Jun 01
TA  - American Journal of Neuroradiology
PG  - 1244--1247
VI  - 30
IP  - 6
4099  - http://www.ajnr.org/content/30/6/1244.short
4100  - http://www.ajnr.org/content/30/6/1244.full
SO  - Am. J. Neuroradiol.2009 Jun 01; 30
AB  - 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.