Positron emission tomography examination of cerebral blood flow and glucose metabolism in young CADASIL patients.
OBJECTIVE
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 > or =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.
Tuominen
Susanna
S
Department of Neurology, Turku University Hospital and University of Turku, Turku, Finland.
Miao
Qing
Q
Kurki
Timo
T
Tuisku
Seppo
S
Pöyhönen
Minna
M
Kalimo
Hannu
H
Viitanen
Matti
M
Sipilä
Hannu T
HT
Bergman
Jörgen
J
Rinne
Juha O
JO
D000328:Adult; D000367:Age Factors; D001921:Brain / Q000000981:diagnostic imaging* / Q000378:metabolism*; D002560:Cerebrovascular Circulation; D015161:Dementia, Multi-Infarct / Q000000981:diagnostic imaging* / Q000235:genetics / Q000378:metabolism*; D015140:Dementia, Vascular / Q000000981:diagnostic imaging / Q000235:genetics / Q000378:metabolism; D005947:Glucose / Q000378:metabolism*; D006801:Humans; D008279:Magnetic Resonance Imaging; D011518:Proto-Oncogene Proteins / Q000235:genetics; D000071656:Receptor, Notch3; D011956:Receptors, Cell Surface; D051880:Receptors, Notch; D014055:Tomography, Emission-Computed
C499374:NOTCH3 protein, human; D011518:Proto-Oncogene Proteins; D000071656:Receptor, Notch3; D011956:Receptors, Cell Surface; D051880:Receptors, Notch; D005947:Glucose