脑淀粉样血管病赵元立英文.pptxVIP

Cerebral Amyloid Angiopathy脑淀粉样血管病;What is CAA?;Prada et al., J. Neurosci., 2007 ;Background;Cerebral Amyloid angiopathyTwo-photon projection of a z-series about 150 um deep into the brain of a living 20-mo-old transgenic mouse expressing a mutant human amyloid precursor protein. This animal had amyloid deposits surrounding some cerebral vessels. Brian J. Bacskai, Massachusetts General Hospital, USA;Epidemiology;Sex and Age;Diagnosis;Transaxial T2-weighted gradient-echo MR images show innumerable microhemorrhages predominantly at cerebral gray–white matter junction. Microhemorrhages are not present in basal ganglia, pons, or cerebellum. Large focal hemorrhages are present in bilateral parietal lobe Marisa Kastoff Blitstein AJR 2007; 189:720-725 ;Guideline for diagnosis;Bhomraj Thanvi Age and Ageing 2006 35(6):565-571 ;Special type of CAA;Why bleeding;Seth Love, Frontiers in Bioscience 14, 4778-4792, January, 2009 ;The cause of amyloid deposits in blood vessels in the brain in sporadic CAA is not known In hereditary CAA, genetic defects, typically on chromosome 21, allow accumulation of amyloid, a protein made up of units called beta-pleated sheet fibrils. The fibrils tend to clump together, so that the amyloid cannot be dissolved and builds up in the brain blood vessel walls. One form of amyloid fibril subunit proteins is the amyloid beta protein. ;Steven Greenberg Geriatrics and aging, 2008 11(5): 15-17 ;Systemic theory amyloid beta protein in blood deposited in blood vessels in the brain breakdown blood-brain barrier amyloid beta protein deposited in brain substance forms neuritic plaque Second theory amyloid fibrils produced by perivascular microglia Third theory both nerve cells and glia produce amyloid precursor protein, increases with aging;病理机制;病理机制;Amyloid Family: Aβ ACys ATTR AGel PrPSc ABri ADan;病理特点;脑淀粉样血管病;HE VS Congo Red;Pathology;由皮层向皮层下过度的区域中受累血管的分布情况;高倍镜下典型的嗜刚果红染色的血管壁，呈现“双环”状 标本中可见不同程度受累的血管;由低倍到高倍示Aβ(+)的脑血管，集中分布在皮层及皮层下区域;grading;Mortal