CADASIL--病例分享及文献复习.pptVIP免费

CADASILCADASIL病例报道及文献复习病例报道及文献复习中山市中医院神经内科中山市中医院神经内科王本国王寅杨楠王本国王寅杨楠CADASILCADASILCerebralAutosomalDominantArteriopathywithSubcorticalInfarcts&Leuko-encephalopathyInheritedsmallvesseldiseaseInheritedsmallvesseldiseasecausingstrokeandcausingstrokeandsubcorticalsubcorticalvasculardementiavasculardementiathatstartsthatstartsinearlyadulthoodandinearlyadulthoodandprogressesovertime.progressesovertime.Thisisanonatherosclerotic,Thisisanonatherosclerotic,nonamyloidangiopathynonamyloidangiopathyinvolvingsmallarteriesandinvolvingsmallarteriesandcapillariesofthebrainandcapillariesofthebrainandotherorgans.otherorgans.CausedbyCausedbymissensemutationsmissensemutationsintheintheNotch3Notch3geneongeneonchromosome19p13.chromosome19p13.CaseStudiesCaseStudiesMostreportedcasesfromEuropeMostreportedcasesfromEurope105peoplefrom33affectedfamilies105peoplefrom33affectedfamiliesVascularriskfactorsareuncommonVascularriskfactorsareuncommonMeanageofinitialsymptomonset36Meanageofinitialsymptomonset36++1212yearsyearsMigrainein40%(28Migrainein40%(28++11yrs)11yrs)Stroke/TIA43%(41Stroke/TIA43%(41++9yrs)9yrs)Depression8%Depression8%Cognitivedecline6%Cognitivedecline6%Seizure3%Seizure3%杨务辉，男性，44岁，ID:0223534主诉：反复头晕头痛、四肢乏力7天余2011-10-70:12由急诊拟“脑梗塞？”收入院。病例资料病例资料现病史：7天前无明显诱因出现头晕头痛，头晕呈晕沉感，头痛为左侧颞顶部放电样疼痛，每次持续数秒，伴四肢乏力，疲倦喜卧，无意识丧失、视物黑曚、发热恶寒、咳嗽咯痰、腹痛腹泻等，经休息后仍反复发作。门诊CT：双侧半卵圆中心-放射冠及基底节区多发稍低密度灶，脑梗塞与其他脑白质病变鉴别，建议颅脑MRI进一步检查。病例资料病例资料既往史：无高血压、糖尿病、冠心病等病史。间断头痛个人史：出生生长于中山，生活环境可，无到过疫区。吸烟，1包/天，饮酒3-4量/天，10年余。无其他不良嗜好，无工毒物、粉尘、放射性物质接触史，无冶游史。婚育史：适龄婚育，已育，子女及配偶均体健。家族史：其弟患可疑脑梗塞病史。病例资料病例资料神清，反应...