title: Positron emission tomography examination of cerebral blood flow and glucose metabolism in young CADASIL patients.
abstract: 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.
authors.affiliation: Department of Neurology, Turku University Hospital and University of Turku, Turku, Finland.
authors.forename: Susanna
authors.initials: S
authors.lastname: Tuominen
authors.forename: Qing
authors.initials: Q
authors.lastname: Miao
authors.forename: Timo
authors.initials: T
authors.lastname: Kurki
authors.forename: Seppo
authors.initials: S
authors.lastname: Tuisku
authors.forename: Minna
authors.initials: M
authors.lastname: Pöyhönen
authors.forename: Hannu
authors.initials: H
authors.lastname: Kalimo
authors.forename: Matti
authors.initials: M
authors.lastname: Viitanen
authors.forename: Hannu T
authors.initials: HT
authors.lastname: Sipilä
authors.forename: Jörgen
authors.initials: J
authors.lastname: Bergman
authors.forename: Juha O
authors.initials: JO
authors.lastname: Rinne
mesh_terms: 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
chemical_list: C499374:NOTCH3 protein, human; D011518:Proto-Oncogene Proteins; D000071656:Receptor, Notch3; D011956:Receptors, Cell Surface; D051880:Receptors, Notch; D005947:Glucose