NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD SURVEILLANCE TABLES OF CASES EXAMINED NPDPSC Last updated March 31st, 2026
NPDPSC Table of Cases Examined Updated quarterly. Last updated on: March 31st, 2026
Year Total Neuropath Referrals2 Prion Disease Sporadic Familial iCJD vCJD
1999 & earlier 386 234 203 28 3 0
2000 145 102 90 12 0 0
2001 209 118 110 8 0 0
2002 241 144 124 18 2 0
2003 259 160 137 21 2 0
2004 314 180 163 16 0 13
2005 331 179 157 21 1 0
2006 364 179 159 17 1 24
2007 374 211 192 19 0 0
2008 384 220 204 16 0 0
2009 396 231 210 20 1 0
2010 402 247 219 28 0 0
2011 390 238 214 24 0 0
2012 413 244 221 23 0 0
2013 418 258 223 34 1 0
2014 355 208 185 21 1 15
2015 400 261 242 19 0 0
2016 394 278 249 29 0 0
2017 377 265 246 19 0 0
2018 311 222 203 18 1 0
2019 434 279 258 21 0 0
2020 369 254 228 24 1 0
2021 343 246 224 22 0 0
2022 343 229 209 20 0 0
2023 325 239 217 21 1 0
2024 344 266 249 16 0 0
2025 351 236 214 29 0 0
2026 98 11 2 1 0 0
TOTAL 9,4706 5,9597 5,3528 5659 15 4
Year CSF Only & RT-QuIC Positive10
2015 139
2016 186
2017 225
2018 261
2019 306
2020 308
2021 325
2022 339
2023 314
2024 411
2025 401
2026 104
TOTAL 331911
1 Listed based on the year of death or, if not available, on the year of referral;
2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted. Includes 44 autopsy-coordinated cases pending tissue receipt;
3 Disease acquired in the United Kingdom;
4 Disease acquired in the United Kingdom in one case and Saudi Arabia in the other.
5 Disease possibly acquired in a Middle Eastern or Eastern European country;
6 Includes 61 cases in which the diagnosis is pending (12 from 2025, and 49 from 2026), and 25 inconclusive cases;
7 Includes 23 (23 from 2025) cases with type determination pending, in which the diagnosis of vCJD has been excluded.
8 The sporadic cases include 5,214 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 96 cases of Variably Protease-Sensitive Prionopathy (VPSPr), and 42 cases of sporadic Fatal Insomnia (sFI).
9 Total does not include 349 Familial cases diagnosed by blood only.
10 Lists the number of positive RT-QuIC cases for which we have not received tissue.
11 Includes the number of positive RT-QuIC results reported from Mayo Clinic (12 from 2024, 90 from 2025, and 26 from 2026).
2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted. Includes 44 autopsy-coordinated cases pending tissue receipt;
3 Disease acquired in the United Kingdom;
4 Disease acquired in the United Kingdom in one case and Saudi Arabia in the other.
5 Disease possibly acquired in a Middle Eastern or Eastern European country;
6 Includes 61 cases in which the diagnosis is pending (12 from 2025, and 49 from 2026), and 25 inconclusive cases;
7 Includes 23 (23 from 2025) cases with type determination pending, in which the diagnosis of vCJD has been excluded.
8 The sporadic cases include 5,214 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 96 cases of Variably Protease-Sensitive Prionopathy (VPSPr), and 42 cases of sporadic Fatal Insomnia (sFI).
9 Total does not include 349 Familial cases diagnosed by blood only.
10 Lists the number of positive RT-QuIC cases for which we have not received tissue.
11 Includes the number of positive RT-QuIC results reported from Mayo Clinic (12 from 2024, 90 from 2025, and 26 from 2026).
ai answer to my question on funding for prion disease 2025 2026;
Federal funding for prion disease surveillance through the Centers for Disease Control and Prevention (CDC) was heavily contested for the 2026 fiscal year. While the President’s proposed budget requested the complete elimination of specific funding lines for human prion disease surveillance, Congress restored the budget. The final budget legislation provided level or slightly increased funding for these critical initiatives.
please note; FY26 President's Budget Prion = 0 $
Budget Area Total Current FY25 Funding FY26 President's Budget % Change from Current
snip…
Emerging and Zoonotic Infectious Diseases
snip…
Prion Disease $8,000,000 $0 $9,000,000 $1,000,000
snip…
Total* $9,217,495,000 $5,470,349,000 -40.65% $9,152,090,000 -0.71% $7,480,880,000 -18.84% -18.26% $9,202,991,000 -0.16%
***> 2023 Professor John Collinge on tackling prion diseases <***
“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”
There is accumulating evidence also for iatrogenic AD.
Understanding prion biology, and in particular how propagation of prions leads to neurodegeneration, is therefore of central research importance in medicine.
Front. Public Health,
12 June 2024
Sec. Infectious Diseases: Epidemiology and Prevention Volume 12 - 2024 |
Updated global epidemiology atlas of human prion diseases
The top seven countries in PrD cases were the USA (n = 5,156), France (n = 3,276), Germany (n = 3,212), Italy (n = 2,995), China (n = 2,662), the UK (n = 2,521), Spain (n = 1,657), and Canada (n = 1,311).
Research Letter
December 11, 2023
Change in Epidemiology of Creutzfeldt-Jakob Disease in the US, 2007-2020
Matthew A. Crane, BS1; Sameer Nair-Desai, BS2; Alison Gemmill, PhD3; et al John A. Romley, PhD4; John C. Probasco, MD5
Author Affiliations Article Information JAMA Neurol. 2024;81(2):195-197. doi:10.1001/jamaneurol.2023.4678
Creutzfeldt-Jakob disease (CJD) is a rapidly progressive and universally fatal prion disease.1 Research on CJD in the US showed stable incidence from 1979 to 2006, though recent trends are not as well described.2 The incidence of sporadic CJD, the most common type, is higher among older patients.1,2 Due to aging populations worldwide, the epidemiology of CJD is evolving.3 We examined death certificate data from 2007 to 2020 to better understand recent US trends of CJD.
Snip…
Discussion Our findings indicate the reported incidence of CJD has risen considerably, disproportionately affecting older and female individuals. These trends align with data from Japan3 and could be influenced by changing demographics. However, our findings may also reflect improved detection of CJD with new diagnostic tools, such as magnetic resonance imaging and real-time quaking-induced conversion testing. This study is limited by a reliance on death certificate data for estimating CJD incidence. While research supports this approach,5 such data may be subject to miscoding or misdiagnosis. Results from both neuropathologic and genetic testing may complement death certificate data and enhance surveillance.6 The findings underscore the changing landscape of CJD and suggest a need for monitoring among the aging US population.
Eur J Epidemiol. 2023; 38(7): 757–764. Published online 2023 May 16. doi: 10.1007/s10654-023-01004-5 PMCID: PMC10276107PMID: 37191829 Received: 31 January 2023 / Accepted: 6 April 2023 / Published online: 16 May 2023 © The Author(s) 2023
The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis
Abstract
Sporadic Creutzfeldt-Jakob disease (sCJD) is the most common form of prion diseases. The causes of sCJD are still unknown and exogenous factors may play a role. Worldwide, the number of patients with sCJD has progressively increased over time. This increase can be partly explained by increasing life expectancy and better case ascertainment, but a true increase in the number of sCJD cases cannot be excluded. We estimated mortality rates from sCJD in France (1992–2016) and studied variation in mortality rates by age, period, and time.
snip...
The overall sCJD mortality rate was 4.58 per 1,000,000 person-years (95% CI=4.39–4.78) (Table S1).
snip...
Besides risk factors explored in case-control studies, the possibility of zoonotic risk factors remains a possibility that could account for an exogenous origin in some sCJD cases. Research on atypical forms of BSE (L-BSE, H-BSE) has revealed molecular similarities between the L-BSE strain and molecular subtypes of human sCJD, in particular the MV2 subtype [39]. Furthermore, L-BSE has been experimentally transmitted to non-human primates as efficiently as classical BSE responsible for vCJD in humans, and could be even more virulent [40–42]. The zoonotic risk associated with natural sheep scrapie has also been recently updated with the demonstration of an intracerebral transmission of scrapie to mice expressing the human prion protein during serial passages, as well as transmission of scrapie to primates. These observations highlight the possibility of a causal link between exposure to sheep scrapie and sCJD in some cases [43, 44]. A large increase in animal product consumption and the generalization of mechanically separated meat in developed countries over the last century may have contribute to increase the zoonotic prion pressure [45]. It would be of interest to observe the effect of safety measures implemented since the “mad cow crisis” to avoid population prion exposure on sCJD mortality in the next decades.
US NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD TSE REPORT SEPTEMBER 2025
From the first full year of reporting CJD TSE in the US in 2000, where 90 cases of CJD was reported that year, to today, where in September 2025, the number of CJD cases reported in the last full year reporting, which would have been 2024, the number of CJD cases for 2024 was 249 cases. So, from the first full year 2000 CJD cases were 90 cases confirmed in that year, to 2024, where 2024 CJD statistics rose to 249 confirmed CJD cases in a single year. A dramatic increase in deaths, from figures that don’t seem to be dramatic. But thes figures today, they are not from “better surveillance”, that dog don’t hunt no more. They have been saying this for over 25 years, year after year, well it’s time to call it for what it is, Human Transmissible Spongiform Encephalopathy TSE Prion cases are rising, and it’s NOT because of better surveillance, or just a “happenstance of bad luck, that 85%+ of all human cases, sporadic CJD, including VPSPr, just happen spontaneously, no, it’s because of unknown environmental factors, and or iatrogenic factors, imho…terry
US NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD TSE REPORT SEPTEMBER 2025
https://prionunitusaupdate.blogspot.com/2025/10/us-national-prion-disease-pathology.html
From the first full year of reporting CJD TSE in the US in 2000, where 90 cases of CJD was reported that year, to today, where in September 2025, the number of CJD cases reported in the last full year reporting, which would have been 2024, the number of CJD cases for 2024 was 249 cases. So, from the first full year 2000 CJD cases were 90 cases confirmed in that year, to 2024, where 2024 CJD statistics rose to 249 confirmed CJD cases in a single year. A dramatic increase in deaths, from figures that don’t seem to be dramatic. But thes figures today, they are not from “better surveillance”, that dog don’t hunt no more. They have been saying this for over 25 years, year after year, well it’s time to call it for what it is, Human Transmissible Spongiform Encephalopathy TSE Prion cases are rising, and it’s NOT because of better surveillance, or just a “happenstance of bad luck, that 85%+ of all human cases, sporadic CJD, including VPSPr, just happen spontaneously, no, it’s because of unknown environmental factors, and or iatrogenic factors, imho…terry
US NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD TSE REPORT SEPTEMBER 2025
https://prionunitusaupdate.blogspot.com/2025/10/us-national-prion-disease-pathology.html
TUESDAY, DECEMBER 12, 2023
CREUTZFELDT JAKOB DISEASE TSE PRION DISEASE UPDATE USA DECEMBER 2023
https://creutzfeldt-jakob-disease.blogspot.com/2023/12/creutzfeldt-jakob-disease-tse-prion.html
CREUTZFELDT JAKOB DISEASE TSE PRION DISEASE UPDATE USA DECEMBER 2023
https://creutzfeldt-jakob-disease.blogspot.com/2023/12/creutzfeldt-jakob-disease-tse-prion.html
May 28, 2026
New study explores potential cross-species spread of chronic wasting disease
A serious wildlife disease is prompting growing concern among scientists, conservationists and public health experts
https://ucalgary.ca/news/new-study-explores-potential-cross-species-spread-chronic-wasting-disease
i remember R Bradley comments early BSE days…
“I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough”
http://web.archive.org/web/20090506041740/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf
https://web.archive.org/web/20090506002904/http://www.bseinquiry.gov.uk/files/ws/s145d.pdf
https://web.archive.org/web/20090506004507/http://www.bseinquiry.gov.uk/files/ws/s147f.pdf
“I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough”
http://web.archive.org/web/20090506041740/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf
https://web.archive.org/web/20090506002904/http://www.bseinquiry.gov.uk/files/ws/s145d.pdf
https://web.archive.org/web/20090506004507/http://www.bseinquiry.gov.uk/files/ws/s147f.pdf
chronic wasting disease cwd tse prion update May 2026
cwd, tse, prion, cervid, cattle, sheep, pigs, primates, oh my!
think ‘friendly fire’, iatrogenic, terrible thought, but, uwhat if?
WEDNESDAY, MAY 27, 2026
https://prpsc.proboards.com/thread/220/limited-transmission-cervid-nonhuman-primates
Limited transmission of cervid prions to nonhuman primates provides insights into the zoonotic potential of chronic wasting disease
2001 Singeltary on CJD, Journal of American Medical Association
February 14, 2001
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Terry S. Singeltary, Sr
Author Affiliations
JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214
To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.
February 14, 2001
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Terry S. Singeltary, Sr
Author Affiliations
JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214
https://jamanetwork.com/journals/jama/article-abstract/1031186
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 Singeltary Journal of Neurology
26 MARCH 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States
Terry S. Singeltary, retired (medically)
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?
https://www.neurology.org/doi/10.1212/01.WNL.0000036913.87823.D6
February 14, 2001
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Terry S. Singeltary, Sr
Author Affiliations
JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214
To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.
February 14, 2001
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Terry S. Singeltary, Sr
Author Affiliations
JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214
https://jamanetwork.com/journals/jama/article-abstract/1031186
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 Singeltary Journal of Neurology
26 MARCH 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States
Terry S. Singeltary, retired (medically)
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?
https://www.neurology.org/doi/10.1212/01.WNL.0000036913.87823.D6
terry
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