title: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy: decrease in regional cerebral blood volume in hyperintense subcortical lesions inversely correlates with disability and cognitive performance.
abstract: OBJECTIVE
  Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an arteriopathic syndrome related to a genetic defect on chromosome 19. Characteristic changes in CADASIL can be observed onT2-weighted MR images in the subcortical white matter. The purpose of this study was to measure changes of regional cerebral blood volume (rCBV) with dynamic contrast-enhanced MR imaging and to correlate the changes to disability and cognitive performance.
  
  
  METHODS
  We obtained rCBV measurements of 24 individuals with proven CADASIL on a 1.5-T MR imaging unit. A susceptibility-weighted MR imaging sequence was used for bolus tracking. Principles of the indicator dilution theory were applied to estimate values of absolute rCBV (mL/100 g). Disability was determined by using the Rankin scale, and overall cognitive performance was assessed by using the Mini-Mental State Examination.
  
  
  RESULTS
  The mean rCBV in the subcortical white matter that was hyperintense on the T2-weighted images (2.7 +/- 0.8 mL/100 g) was significantly lower than the rCBV in the white matter that appeared normal on the T2-weighted images (4.4 +/- 1.3 mL/100 g) (P <.05). The mean rCBV in the gray matter was within the normal range (8.3 +/- 1.7 mL/100 g). Both cognitive impairment and disability negatively correlated with rCBV in the subcortical white matter that was hyperintense (P <.05) but not with rCBV in the normal appearing white matter. rCBV did not correlate with age.
  
  
  CONCLUSIONS
  rCBV measured in the hyperintense subcortical white matter in individuals with CADASIL was decreased and inversely correlated with disability and cognitive impairment.
authors.affiliation: Institute of Clinical Radiology, Klinikum Grossadern, Ludwig-Maximilians University, Marchioninstrasse 15, D-81377 Munich, Germany.
authors.forename: R
authors.initials: R
authors.lastname: Bruening
authors.forename: M
authors.initials: M
authors.lastname: Dichgans
authors.forename: C
authors.initials: C
authors.lastname: Berchtenbreiter
authors.forename: T
authors.initials: T
authors.lastname: Yousry
authors.forename: K C
authors.initials: KC
authors.lastname: Seelos
authors.forename: R H
authors.initials: RH
authors.lastname: Wu
authors.forename: M
authors.initials: M
authors.lastname: Mayer
authors.forename: G
authors.initials: G
authors.lastname: Brix
authors.forename: M
authors.initials: M
authors.lastname: Reiser
mesh_terms: D000328:Adult; D000368:Aged; D001810:Blood Volume / Q000502:physiology; D001921:Brain / Q000098:blood supply; D002539:Cerebral Arterial Diseases / Q000175:diagnosis / Q000235:genetics*; D002544:Cerebral Infarction / Q000175:diagnosis / Q000235:genetics*; D002869:Chromosome Aberrations / Q000235:genetics*; D025063:Chromosome Disorders; D003704:Dementia / Q000175:diagnosis / Q000235:genetics*; D015140:Dementia, Vascular / Q000175:diagnosis / Q000235:genetics*; D004185:Disability Evaluation; D004292:Dominance, Cerebral / Q000502:physiology; D005260:Female; D005799:Genes, Dominant / Q000235:genetics*; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008297:Male; D008609:Mental Status Schedule; D008875:Middle Aged; D009460:Neurologic Examination; D012039:Regional Blood Flow / Q000502:physiology; D013577:Syndrome