title: Advanced intimal hyperplasia without luminal narrowing of leptomeningeal arteries in CADASIL.
abstract: OBJECTIVE
  Leptomeningeal artery abnormalities in Cerebral Autosomal-Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) have not been extensively characterized. We quantified substructure and diameter of leptomeningeal arteries in CADASIL compared with age-matched controls and the very old; in addition, we characterized intimal thickening in CADASIL using immunohistochemistry.
  
  
  METHODS
  Frontal and temporal cortex of 6 genetically proven CADASIL brains (average age, 66 years), 6 controls without symptoms of cerebrovascular disease, and 6 very old brains (average age, 89 years) were examined for leptomeningeal artery intimal, medial, and adventitial thickness; inner diameter; and sclerotic index and for smooth muscle markers.
  
  
  RESULTS
  The intima of CADASIL arteries was thickened 5-fold compared with controls and the very aged (P<0.0001). Medial thickness was lower in CADASIL compared with controls and the very old (P<0.01). The adventitia was not significantly increased in CADASIL compared with age-matched controls. Arterial diameters were not smaller in CADASIL compared with controls. Sclerotic index was significantly increased in CADASIL compared with other groups (P<0.00001). Intimal cells in CADASIL expressed smooth muscle actin, S100A4, and vimentin but not desmin.
  
  
  CONCLUSIONS
  Principle changes of leptomeningeal arteries in CADASIL include intimal thickening and medial thinning, but not luminal narrowing. Smooth muscle-like cells participate in neointimal thickening of CADASIL arteries.
authors.affiliation: Department of Neurology, University of Michigan, 7629 Med Sci II 5622, 1137 Catherine St, Ann Arbor, MI 48109, USA. micwang@umich.edu
authors.forename: Hairong
authors.initials: H
authors.lastname: Dong
authors.forename: Haixia
authors.initials: H
authors.lastname: Ding
authors.forename: Kelly
authors.initials: K
authors.lastname: Young
authors.forename: Mila
authors.initials: M
authors.lastname: Blaivas
authors.forename: Paul J
authors.initials: PJ
authors.lastname: Christensen
authors.forename: Michael M
authors.initials: MM
authors.lastname: Wang
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001158:Arteries / Q000473:pathology*; D001921:Brain / Q000473:pathology*; D046589:CADASIL / Q000473:pathology*; D006801:Humans; D006965:Hyperplasia / Q000473:pathology; D008578:Meninges / Q000098:blood supply; D008875:Middle Aged; D017539:Tunica Intima / Q000473:pathology*