CJD is a prion disease caused by misfolded prion proteins, with neuropathology described as deposition of misfolded prions in the brain leading to apoptotic change and spongiform alterations [2][3]. The literature in the supplied sources classifies CJD into sporadic, hereditary, and acquired forms, indicating heterogeneity in prion type and genetic profile [2]. Source-backed detail on formal ICD coding or additional etiologic subtyping is not yet available from the provided material [2].
Disease overview
PrionCreutzfeldt-Jakob disease
克雅氏病
Evidence-backed informationEN 7/7 applicable sections · ZH 7/7 applicable sectionsUpdated Sep 4, 2026
Creutzfeldt-Jakob disease (CJD) is a fatal prion disease characterized by rapidly progressive dementia and other rapidly progressive neurodegenerative features [1][2]. The available sources emphasize its public-health relevance because asymptomatic incubation can extend for decades, including periods as long as 40 years, and because iatrogenic transmission is a recognized concern [1]. Although CJD is rare, surveillance attention remains warranted because sporadic CJD incidence is reported to be increasing and older patients may present with rapidly evolving dementia that can be clinically difficult to distinguish from other conditions [1].
Read the full clinical and epidemiological profile8
Creutzfeldt-Jakob disease manifests as a rapidly progressive neurodegenerative disorder characterized by psychomotor decline, cognitive impairment, motor dysfunction, and behavioral alterations [2]. Pathologically, the condition stems from the deposition of misfolded prion proteins in the brain, which induces neuronal apoptosis and spongiform tissue changes [2]. The clinical course is uniformly fatal, with survival typically measured in months to a year after symptom emergence [4]. Clinical evaluation identifies characteristic neuropsychiatric manifestations alongside supportive cerebrospinal fluid markers and protein amplification assays [3].
The supplied review describes CJD as rare, with particular note that iatrogenic CJD is uncommon, while sporadic CJD incidence is increasing [1]. CJD has been discussed in the context of global incidence, prevalence, infectivity, and incubation, indicating that surveillance concerns are not limited to one setting [1]. Older people are specifically highlighted as an important clinical context for sporadic CJD, where the disease may resemble rapidly developing dementia [1]. Beyond these points, source-backed detail on regional distribution, outbreak clustering, or burden estimates is not yet available from the provided snippets [1].
The disease spreads via three distinct pathways: sporadic, genetic, and acquired [4]. Sporadic forms develop spontaneously when endogenous prion proteins misfold through incompletely understood mechanisms [4]. Genetic variants originate from heritable mutations in the prion protein gene, often clustering within families [4]. Acquired or iatrogenic transmission results from direct exposure to prion-contaminated medical resources, such as historical human growth hormone preparations, dural grafts, corneal transplants, or contaminated surgical instruments [4].
The supplied sources most clearly identify older people as an important group for awareness in sporadic CJD, because presentation may resemble rapidly progressive dementia in this age range [1].
Primary prevention strategies emphasize strict prion-control protocols within healthcare settings to prevent iatrogenic transmission [4]. Implementation of enhanced decontamination procedures for surgical and medical devices has successfully eliminated reported cases associated with contaminated equipment since the late 1970s [4]. Ongoing prevention efforts continue to prioritize safe handling practices for relevant biological products and tissues to minimize cross-exposure risks [4].
For surveillance purposes, CJD should be read as a rare but high-consequence prion disease with prolonged silent incubation and potential iatrogenic implications [1]. Surveillance utility is strongest when rapidly progressive dementia or neuropsychiatric decline is investigated with attention to prion-compatible biomarkers and updated diagnostic criteria, including RT-QuIC where available [3]. Because sporadic CJD may present in older adults as rapidly developing dementia, case finding depends on maintaining awareness of this diagnostic possibility in routine monitoring [1]. GlobalID source-data note: Norway MSIS publishes the source category ‘Prionsykdommer’ (prion diseases). Reported Norwegian cases have predominantly been CJD, but the category and reporting criteria can include other prion diseases; interpret the series as the official source category rather than assuming every value is a subtype-confirmed CJD case.
- 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
Coverage
Reporting countries and regions
AustraliaAU
AustriaAT
BelgiumBE
BrazilBR
BulgariaBG
CanadaCA
CroatiaHR
CyprusCY
CzechiaCZ
DenmarkDK
EstoniaEE
FranceFR
GreeceGR
Hong Kong, ChinaHK
HungaryHU
IcelandIS
IrelandIE
ItalyIT
JapanJP
LatviaLV
LiechtensteinLI
LithuaniaLT
LuxembourgLU
MaltaMT
NetherlandsNL
NorwayNO
PolandPL
PortugalPT
RomaniaRO
SlovakiaSK
SloveniaSI
South KoreaKR
SpainES
SwedenSE
SwitzerlandCH
Taiwan, ChinaTW
United KingdomGB
Trends by reporting country
Monthly patterns over time
Data access
Page dataset index with source links and update metadata.
Official sourcesAuthority, cadence, notes49
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.
Iceland Directorate of Health Annual Dashboard
Iceland
National annual case notifications by disease. Published values can be revised retrospectively and remain annual period totals.
Iceland Directorate of Health STI Dashboard
Iceland
Monthly STI diagnosis facts from laboratory and registry surveillance; the official dashboard is published on a quarterly schedule.
Iceland Directorate of Health Respiratory Dashboard
Iceland
ISO-week respiratory diagnosis counts. Hospitalizations, samples, and vaccination indicators are separate measures and are not counted as diagnoses.
Iceland Directorate of Health Historical Registry
Iceland
Historical official registry tables for 1997–2021, with annual totals and disease-specific monthly notification facts retained at source grain.
Iceland Directorate of Health Legacy ICD Monthly
Iceland
Historical Saga EHR ICD encounter counts for 1997–2020. This clinical measure is non-comparable with registry notifications and is kept separate.
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.
JP NIID Weekly
Japan
Japan weekly infectious disease surveillance via NIID/JIHS.
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.