疯牛病和CJD北京友谊医院神经内科北京友谊医院神经内科神经病毒室神经病毒室王得新王得新内容：疯牛病（牛海绵样脑病）－－为什么对疯牛病（牛海绵样脑病）－－为什么对动物和人类构成威胁？动物和人类构成威胁？关于朊病毒关于朊病毒((Prion)Prion)的研究的研究Creutzfeldt-JakobCreutzfeldt-Jakob病的影像学诊断病的影像学诊断可传播性海绵样脑病的药物治疗进展可传播性海绵样脑病的药物治疗进展疯牛病（牛海绵样脑病）－－为什么对动物和人类构成威胁？TransmissibleSpongiformEncephalopathies(TSEs)AnimalHumanScrapie—sheep/goatKuruTME—minkCJD—sporadicCWD—maledeer/elkfamilyiatrogenicvariantBSE—cattleGSSexoticungulatesFFIFSE可传播性海绵样脑病的特征长潜伏期－－恒定的致死性疾病长潜伏期－－恒定的致死性疾病病因性病原（朊病毒）－－不能被现有病因性病原（朊病毒）－－不能被现有消毒灭菌手段灭活消毒灭菌手段灭活血清学试验不能证实这种感染血清学试验不能证实这种感染在潜伏期内感染可以在组织中存在（扁在潜伏期内感染可以在组织中存在（扁桃腺、脾脏、淋巴组织）桃腺、脾脏、淋巴组织）英国BSE流行情况BSE在欧盟国家发生情况0501001502002503003504004505001987198819891990199119921993199419951996199719981999200020010500010000150002000025000300003500040000countrieswithcasesbefore2000Francecountrieswithfirstcasesin2000SwitzerlandUKFIRSTCASESOFBSEUnitedKingdomIrelandSwitzerlandPortugalFranceNetherlandsBelgiumLuxembourgLiechtenstein19861990199419982000ItalyCzechRepGreeceSlovakRepJapanSloveniaAustriaFinlandDenmarkSpainGermanyNUMBEROFBSECASESINTHEWORLD18204893361257041316810673593285322211111101001000100001000001000000countrylognoofcasesBSE发病例数英国：英国：182779182779爱尔兰：爱尔兰：10701070法国：法国：707707葡萄牙：葡萄牙：688688欧盟仅瑞典未发现欧盟仅瑞典未发现BSEBSE日本：日本：55（根据新闻报道）（根据新闻报道）英国出口牛及牛制品情况1979-19950500010000150002000025000300003500019791980198119821983198419851986198719881989199019911992199319941995EUNon-EUcountries欧盟对存在BSE国家分类评估GeographicalBSErisk,GBRGBRlevelGBRlevelRiskRiskIIHighlyunlikelyHighlyunlikelyIIIIUnlikelybutnotexcludedUnlikelybutnotexcludedIIIIIILikelybutnotconfirmedLikelybutnotconfirmedororconfirmedatalowerlevelconfirmedatalowerlevelIVIVConfirmedatahigherlevelConfirmedatahigherlevelCountrycategories(GBR)Countrycategories(GBR)GBRlevelCountriesIArgentina,Australia,Botswana,Brazil,Chile,Namibia,NewZealand,Nicaragua,Norway,Paraguay,Singapore,Swaziland,Uruguay,ElSalvador,Panama,CostaRicaIIColombia,India,Kenya,Mauritius,Nigeria,Pakistan,Sweden,Canada,USAIIIAlbania,Belgium,Denmark,Cyprus,CzechRepublic,Estonia,France,Germany,Hungary,Ireland,Italy,Lithuania,Luxembourg,Poland,TheNetherlands,Romania,SlovakRepublic,Spain,Switzerland,Austria,Slovenia,Finland,IVUnitedKingdom,Portugal特别指定的危险组织和器官EUUKandPortugalSwitzerlandcattlescullinclusivebrainandeyes>12mths>6mths>6mthstonsils>12mths>6mths>6mthsspinalcord>12mths>6mths>6mthsvertebralcolumn(spinalganglia)>12mths>12mthscowsintestines(ileum)everyageeveryage>6mthsspleenno>6mths>6mthsthymusno>6mths>6mthsvisiblelymphandnerv.tissuenonoeveryageBSE和vCJD相关的证据临床表现流行病学神经病理学朊蛋白（prionprotein）isotyping实验室传播的特征疯牛病简介疯牛病（MadCowDisease）是牛海绵样脑病（BovineSpongiformEncephalitis，BSE）的俗称。1986年首先在英国发现。临床表现：•紧张不安，攻击行为，姿势异常，动作不协调，爬起困难。•食欲不减但体重减轻，泌乳量减少。•潜伏期长达2-8年。•病程2周-6个月。•病牛不屠杀...
