克雅氏病是一类由错误折叠的朊蛋白(PrP Sc)相关的致死性朊蛋白病,病理过程涉及脑内异常朊蛋白沉积并导致神经退行性损害和海绵状改变[2][3]。文献将其分为散发型、遗传型和获得型等形式[2]。目前可获得资料显示,该病的临床识别和监测均受到其异质性、潜伏期长以及确证前诊断困难的影响[1][3]。
疾病概览
Prion克雅氏病
Creutzfeldt-Jakob disease
克雅氏病(Creutzfeldt-Jakob disease, CJD)是一组朊蛋白相关的致死性神经退行性疾病,常以快速进展性痴呆和心理运动改变为主要表现[1][2][3]。现有综述提示其临床过程可迅速恶化,多数患者在临床起病后1年内死亡[1]。从公共卫生监测角度看,CJD总体罕见,但与医源性传播风险、长期潜伏以及散发型病例识别有关,因此需要持续警惕[1][3]。
阅读完整临床与流行病学资料8
CJD的核心临床特征是快速进展性神经退行性综合征,表现为快速进展性痴呆,以及心理运动改变[1][2]。综述还提到可见认知功能障碍、运动障碍和行为异常等表现[2]。临床诊断线索通常结合特征性神经精神症状、脑脊液14-3-3蛋白、MRI和EEG等信息,而RT-QuIC等蛋白聚集/扩增检测被认为有助于临床前确证[3]。疾病预后不良,多数患者在临床起病后1年内死亡[1]。
现有综述指出,CJD总体罕见,其中尤以医源性CJD较为少见,但其公共卫生意义在于潜在的传播风险[1]。系统综述还提示,散发型CJD的发病率呈上升趋势[1]。文献同时指出,潜伏期可长达40年,提示既往暴露后的长期监测具有意义[1]。另一项综述强调,散发型病例多见于老年人,临床上可能与快速进展性痴呆相似,从而增加识别难度[1]。
可获得资料支持CJD存在医源性传播风险,且可在人体内无症状潜伏数十年后才表现为临床疾病[1]。这意味着公共卫生关注点主要在既往暴露史、潜伏期管理以及与医疗操作相关的风险识别,而不是常规社区传播[1]。关于其他具体传播途径、暴露场景或不同形式之间的差异性传播机制,所给来源中未提供足够细节。
现有资料中明确提到,散发型CJD多见于老年人[1]。此外,任何存在既往医源性暴露潜在风险的人群都需要在长期随访与监测框架下被关注,因为疾病可在人体内无症状潜伏多年甚至数十年[1]。关于更具体的高风险暴露群体或分层风险特征,所给来源尚未提供足够支持。
来源提示,预防重点在于维持对CJD的持续警觉,以减少未来传播事件并加强相关监测[1][3]。对于已知或疑似散发型病例,更新后的诊断标准和RT-QuIC等技术可提高早期临床确认和监测能力,从而支持公共卫生处置[3]。除上述监测与识别措施外,关于具体暴露控制、器械处理或其他预防程序,本次来源未提供可直接引用的细节。
在监测语境中,CJD应被理解为一种罕见但需要长期警惕的致死性朊蛋白病,尤其应关注散发型病例的识别及其与快速进展性痴呆的重叠[1][3]。由于可有长达数十年的无症状潜伏,病例监测不应仅依赖近期暴露史,而应结合临床演变、神经精神表现及辅助检测结果综合判断[1][3]。更新后的诊断与生物标志物应用被认为可改善早期确认、监测和相关研究评估[3]。 GlobalID 来源数据说明: 挪威 MSIS 发布的来源类别为“Prionsykdommer”(朊病毒病)。挪威已报告病例以 CJD 为主,但该类别及报告标准可以包含其他朊病毒病;因此应按官方来源类别解释,而不应假定每个数值都是已完成亚型确认的 CJD 病例。
- 1Uttley L et al. Creutzfeldt-Jakob disease: a systematic review of global incidence, prevalence, infectivity, and incubation. Lancet Infect Dis. 2020 Jan. PMID: 31876504. doi: 10.1016/S1473-3099(19)30615-2.PubMed: https://pubmed.ncbi.nlm.nih.gov/31876504/
- 2Noor H et al. Creutzfeldt-Jakob disease: A comprehensive review of current understanding and research. J Neurol Sci. 2024 Dec 15. PMID: 39546829. doi: 10.1016/j.jns.2024.123293.PubMed: https://pubmed.ncbi.nlm.nih.gov/39546829/
- 3Hermann P et al. Biomarkers and diagnostic guidelines for sporadic Creutzfeldt-Jakob disease. Lancet Neurol. 2021 Mar. PMID: 33609480. doi: 10.1016/S1474-4422(20)30477-4.PubMed: https://pubmed.ncbi.nlm.nih.gov/33609480/
- 4US Centers for Disease Control and Prevention. Classic Creutzfeldt-Jakob Disease | Classic CJD | CDC [Internet]. cited 4 Sept 2026.Available from: https://www.cdc.gov/creutzfeldt-jakob/about/index.html
数据覆盖
报告国家和地区
各报告国家的趋势
月度变化趋势
数据获取
本页面数据索引,含来源链接和更新信息。
官方数据来源机构、频率与说明49
Austria AGES Radar for Infectious Diseases
Austria
AGES Radar monthly national source. Internal ingestion retains native categories; public release is disabled pending licence review.
Australia NINDSS
Australia
Australian national notifiable diseases surveillance dashboard.
Australia NINDSS — Australian Capital Territory
Australian Capital Territory, Australia
Australian NINDSS monthly state/territory notifications for Australian Capital Territory, kept separate from the Australia national aggregate.
Australia NINDSS — New South Wales
New South Wales, Australia
Australian NINDSS monthly state/territory notifications for New South Wales, kept separate from the Australia national aggregate.
Australia NINDSS — Northern Territory
Northern Territory, Australia
Australian NINDSS monthly state/territory notifications for Northern Territory, kept separate from the Australia national aggregate.
Australia NINDSS — Queensland
Queensland, Australia
Australian NINDSS monthly state/territory notifications for Queensland, kept separate from the Australia national aggregate.
Australia NINDSS — South Australia
South Australia, Australia
Australian NINDSS monthly state/territory notifications for South Australia, kept separate from the Australia national aggregate.
Australia NINDSS — Tasmania
Tasmania, Australia
Australian NINDSS monthly state/territory notifications for Tasmania, kept separate from the Australia national aggregate.
Australia NINDSS — Victoria
Victoria, Australia
Australian NINDSS monthly state/territory notifications for Victoria, kept separate from the Australia national aggregate.
Australia NINDSS — Western Australia
Western Australia, Australia
Australian NINDSS monthly state/territory notifications for Western Australia, kept separate from the Australia national aggregate.
ECDC Surveillance Atlas — Belgium Annual Baseline
Belgium
Belgium annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
ECDC Surveillance Atlas — Bulgaria Annual Baseline
Bulgaria
Bulgaria annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
Brazil DATASUS SINAN
Brazil
Brazil Ministry of Health DATASUS/SINAN public DBC microdata aggregated to national monthly notification counts.
Canada PHAC CNDSS Annual
Canada
Canada is the national jurisdiction; subdivision feeds are registered separately.
Switzerland FOPH IDD
Switzerland
Switzerland FOPH/BAG IDD mandatory reporting API normalized to national case rows. Monthly series may use the dashboard CHFL aggregate where CH-only monthly series are not exposed.
ECDC Surveillance Atlas — Cyprus Annual Baseline
Cyprus
Cyprus annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
ECDC Surveillance Atlas — Czechia Annual Baseline
Czechia
Czechia annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
ECDC Surveillance Atlas — Denmark Annual Baseline
Denmark
Denmark annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
ECDC Surveillance Atlas — Estonia Annual Baseline
Estonia
Estonia annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
ECDC Surveillance Atlas — Spain Annual Baseline
Spain
Spain annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
ECDC Surveillance Atlas — France Annual Baseline
France
France annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
ECDC Surveillance Atlas — United Kingdom Annual Baseline
United Kingdom
United Kingdom annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
ECDC Surveillance Atlas — Greece Annual Baseline
Greece
Greece annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
Hong Kong, China CHP Notifiable Diseases
Hong Kong, China
Hong Kong, China CHP annual notifiable infectious disease CSVs normalized to national monthly totals
ECDC Surveillance Atlas — Croatia Annual Baseline
Croatia
Croatia annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
ECDC Surveillance Atlas — Hungary Annual Baseline
Hungary
Hungary annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
Ireland HPSC Notifiable Diseases Hub
Ireland
Ireland HPSC national weekly notifiable-disease totals from the official ArcGIS FeatureServer. Internal ingestion is enabled; public release remains disabled pending written redistribution permission.
冰岛卫生署年度传染病看板
Iceland
冰岛全国分病种年度病例通报;历史值可能追溯修订,并保持年度总量口径。
冰岛卫生署性病监测看板
Iceland
实验室与登记监测的月度性病诊断事实;官方看板按季度发布。
冰岛卫生署呼吸道感染周度看板
Iceland
按 ISO 周记录的呼吸道疾病诊断数;住院、样本和疫苗指标不计入诊断数。
冰岛卫生署历史传染病登记
Iceland
1997—2021 年官方历史登记表,年度与月度事实按原始粒度保留。
冰岛卫生署历史 ICD 临床月报
Iceland
Saga 电子病历 ICD 就诊数;与登记通报不可比并保持独立。
ECDC Surveillance Atlas — Italy Annual Baseline
Italy
Italy annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
日本 NIID/JIHS 周报
Japan
日本 NIID/JIHS 周度传染病监测数据。
Korea KDCA EID
South Korea
Korea KDCA notifiable infectious disease OpenAPI or portal/KOSIS downloads aggregated to national monthly notification counts.
ECDC Surveillance Atlas — Liechtenstein Annual Baseline
Liechtenstein
Liechtenstein annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
ECDC Surveillance Atlas — Lithuania Annual Baseline
Lithuania
Lithuania annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
ECDC Surveillance Atlas — Luxembourg Annual Baseline
Luxembourg
Luxembourg annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
ECDC Surveillance Atlas — Latvia Annual Baseline
Latvia
Latvia annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
ECDC Surveillance Atlas — Malta Annual Baseline
Malta
Malta annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
ECDC Surveillance Atlas — Netherlands Annual Baseline
Netherlands
Netherlands annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
Norway FHI MSIS Statistics Bank
Norway
Norway FHI MSIS national monthly notifications from the official Allvis frontend API; adapter contract checks guard the unversioned endpoint.
ECDC Surveillance Atlas — Poland Annual Baseline
Poland
Poland annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
ECDC Surveillance Atlas — Portugal Annual Baseline
Portugal
Portugal annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
ECDC Surveillance Atlas — Romania Annual Baseline
Romania
Romania annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
Sweden Public Health Agency SmiNet
Sweden
Sweden SmiNet national monthly reported cases from the official Public Health Agency statistics pages; public release is enabled with closed-month publication and revisable recent-month refreshes.
ECDC Surveillance Atlas — Slovenia Annual Baseline
Slovenia
Slovenia annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
ECDC Surveillance Atlas — Slovakia Annual Baseline
Slovakia
Slovakia annual Member-State reported case counts from the ECDC Surveillance Atlas. Missing topic/year cells remain unknown and published zeroes are preserved. Data provided by ECDC based on data reported by EU/EEA Member States.
Taiwan, China CDC NIDSS
Taiwan, China
Taiwan, China monthly notifiable infectious disease open-data CSV feed.