Published Online
on March 11, 2004

A more recent version of this article appeared on May 1, 2004

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Submitted on July 28, 2003
Revised on January 8, 2004
Accepted on January 13, 2004

Positron Emission Tomography Examination of Cerebral Blood Flow and Glucose Metabolism in Young CADASIL Patients

Susanna Tuominen MD; Qing Miao MSc; Timo Kurki MD, PhD; Seppo Tuisku MD; Minna Pöyhönen MD, PhD; Hannu Kalimo MD, PhD^*; Matti Viitanen MD, PhD; Hannu T. Sipilä MSc; Jörgen Bergman PhD; and Juha O. Rinne MD, PhD

From Department of Neurology (S.Tuominen), Turku PET Centre (S.Tuominen, H.T.S., J.B., J.O.R.), Department of Pathology (Q.M., H.K.), and Department of Radiology (T.K.), Turku University Hospital and University of Turku, Turku, Finland; Department of Neurology (S.Tuisku), Keski-Pohjanmaa Central Hospital, Kokkola, Finland; Department of Medical Genetics (M.P.), Family Federation of Finland, Helsinki, Finland; Department of Pathology (H.K.), University of Helsinki and Helsinki University Hospital, Helsinki, Finland; Department of Pathology (H.K.), Uppsala Academic Hospital and University of Uppsala, Uppsala, Sweden; Department of Geriatric Medicine (M.V.), University of Turku, Turku, Finland; Division of Geriatric Medicine (M.V.), Karolinska Institutet, Huddinge Hospital, Stockholm, Sweden.

^* To whom correspondence should be addressed. E-mail: hkalimo{at}utu.fi.

Background and Purpose--CADASIL causes repeated ischemic strokes leading to subcortical vascular dementia. The purpose of this study was to assess whether cerebral blood flow (CBF) and regional cerebral metabolic rates of glucose (rCMR_gluc) in CADASIL patients are affected in early adulthood.

Methods--CBF and rCMR_gluc were examined with positron emission tomography in correlation with magnetic resonance imaging (MRI) in 14 adult (19 to 41 years) CADASIL patients with the Notch3 R133C mutation. Seven patients had experienced transient ischemic attack and 3 had experienced 1 strokes.

Results--The mean CBF in the CADASIL patients was significantly lower in both frontal (P=0.019) and occipital (P=0.009) white matter (WM) than those in the controls. CBF decreased significantly with increased severity of the disease. The patients had lower mean rCMR_gluc values than the controls, although differences were not statistically significant. Sum scores of semiquantitative MRI rating scale (Scheltens) correlated significantly with WM CBF but not with rCMR_gluc.

Conclusions--In CADASIL, there is an early and significant decrease in the CBF of WM associated with simultaneous MRI changes. These are obviously caused by the arteriopathy in long penetrating arteries and indicate early tissue damage, also expressed as impaired rCMR_gluc in the WM.

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