NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER SURVEILLANCE TABLES OF CASES EXAMINED January 11th, 2023
Tables of Cases Examined National Prion Disease Pathology Surveillance Center Cases Examined¹
Updated quarterly.
Last updated on: January 11th, 2023
Year Total Neuropath Referrals² Prion Disease Sporadic Genetic Iatrogenic vCJD
1999 & earlier 383 232 202 27 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 315 180 163 16 0 1³
2005 330 179 157 21 1 0
2006 365 179 159 17 1 2⁴
2007 374 210 191 19 0 0
2008 384 221 205 16 0 0
2009 397 231 210 20 1 0
2010 402 246 218 28 0 0
2011 392 238 214 24 0 0
2012 413 244 221 23 0 0
2013 416 258 223 34 1 0
2014 355 208 185 21 1 1⁵
2015 401 263 243 20 0 0
2016 395 277 248 29 0 0
2017 375 266 247 19 0 0
2018 308 221 202 18 1 0
2019 433 280 259 21 0 0
2020 366 252 227 24 1 0
2021 343 248 223 22 0 0
2022 307 199 165 13 0 0
TOTAL 83086 51567 46238 4919 14 4
Year CSF Only and RT-QuIC Positive10
2015 241
2016 360
2017 406
2018 431
2019 538
2020 494
2021 516
2022 492
TOTAL 3478
1Listed based on the year of death or, if not available, on the year of referral;
2Cases with suspected prion disease for which brain tissue was submitted;
3Disease acquired in the United Kingdom;
4Disease acquired in the United Kingdom in one case and in Saudi Arabia in the other;
5Disease possibly acquired in a Middle Eastern or Eastern European country;
6Includes 25 cases in which the diagnosis is pending (1 from 2020, 2 from 2021 and 21 from 2022), and 20 inconclusive cases;
7Includes 24 (3 from 2021 and 21 from 2022) cases with type determination pending in which the diagnosis of vCJD has been excluded.
8The sporadic cases include 4504 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 82 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 37 cases of sporadic Fatal Insomnia (sFI).
9Total does not include 301 Familial cases diagnosed by blood test only.
10Lists number of patients (deceased and alive) who have had a positive RT-QuIC and no neuropath examination.
For a downloadable PDF version of our quarterly table, please click the link below:
NPDPSC Table of Cases Examined
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Tables of Cases Examined | Pathology | School of Medicine | Case Western Reserve University case.edu
National Prion Disease Pathology Surveillance Center Cases Examined1 (September 20, 2022) Year Total Neuropath Referrals2 Prion Disease Sporadic Familial iCJD vCJD
1999 & earlier 383 232 202 27 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 315 180 163 16 0 13
2005 328 179 157 21 1 0
2006 365 179 159 17 1 24
2007 374 210 191 19 0 0
2008 384 221 205 16 0 0
2009 397 231 210 20 1 0
2010 401 246 218 28 0 0
2011 392 238 214 24 0 0
2012 413 244 221 23 0 0
2013 416 258 223 34 1 0
2014 355 208 185 21 1 15
2015 401 263 243 20 0 0
2016 395 277 248 29 0 0
2017 375 266 247 19 0 0
2018 308 221 202 18 1 0
2019 434 281 259 22 0 0
2020 365 252 227 24 1 0
2021 343 248 223 22 0 0
2022 213 124 98 9 0 0
TOTAL 82116 50827 45568 4889 14 4
Year CSF Only & RT-QuIC Positive10
2015 140
2016 183
2017 227
2018 266
2019 311
2020 310
2021 341
2022 262
TOTAL 2040
1 Listed based on the year of death or, if not available, on year of referral;
2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted;
3 Disease acquired in the United Kingdom;
4 Disease acquired in the United Kingdom in one case and in Saudi Arabia in the other;
5 Disease possibly acquired in a Middle Eastern or Eastern European country;
6 Includes 28 cases in which the diagnosis is pending (1 from 2020, 3 from 2021 and 24 from 2022), and 20 inconclusive cases;
7 Includes 20 (3 from 2021 and 17 from 2022) cases with type determination pending in which the diagnosis of vCJD has been excluded.
8 The sporadic cases include 4437 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 82 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 37 cases of sporadic Fatal Insomnia (sFI).
9 Total does not include 300 Familial cases diagnosed by blood only. 10 Lists number of patients (deceased and alive) who have had a positive RT-QuIC and no neuropath examination.
https://case.edu/medicine/pathology/sites/case.edu.pathology/files/2022-10/WebTable%20NPDPSC.pdf
Tuesday APRIL 05, 2022
Incidence of Creutzfeldt-Jakob Disease in the United States 1993-2014
https://creutzfeldt-jakob-disease.blogspot.com/2022/04/incidence-of-creutzfeldt-jakob-disease_5.html
SUNDAY, MAY 08, 2022
USA National Prion Disease Pathology Surveillance Center Surveillance Update April 11th, 2022
https://creutzfeldt-jakob-disease.blogspot.com/2022/05/usa-national-prion-disease-pathology.html
TUESDAY, MAY 24, 2022
Texas Creutzfeldt Jakob Disease CJD TSE Prion Update Singeltary FOIA Request Received May 23, 2022
https://cjdtexas.blogspot.com/2022/05/texas-creutzfeldt-jakob-disease-cjd-tse.html
TUESDAY, MAY 10, 2022
Concordance of CSF RT-QuIC across the European Creutzfeldt-Jakob Disease surveillance network
https://creutzfeldt-jakob-disease.blogspot.com/2022/05/concordance-of-csf-rt-quic-across.html
FRIDAY, DECEMBER 02, 2022
Creutzfeldt Jacob Disease CJD TSE Prion December 2022 Annual Update
https://creutzfeldt-jakob-disease.blogspot.com/2022/12/creutzfeldt-jacob-disease-cjd-tse-prion.html
Friday, DECEMBER 24, 2021
Creutzfeldt Jakob Disease CJD TSE Prion Update December 25, 2021
https://creutzfeldt-jakob-disease.blogspot.com/2021/12/creutzfeldt-jakob-disease-cjd-tse-prion.html
WEDNESDAY, JANUARY 25, 2023
Canada Creutzfeldt-Jakob disease surveillance system (CJDSS) report steady rise in cases as of January 2023 and STILL NO CASES REPORTED OF VPSPr CJD
https://creutzfeldt-jakob-disease.blogspot.com/2023/01/canada-creutzfeldt-jakob-disease.html
Terry
