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Tables of Cases Examined

 
National Prion Disease Pathology Surveillance Center Cases Examined¹

Updated quarterly.
Last updated on: January 9th, 2021

Year Total Referrals² Prion Disease Sporadic Familial Iatrogenic vCJD
1999 & earlier 382 231 201 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 396 277 248 29 0 0
2017 375 266 247 19 0 0
2018 309 223 204 18 1 0
2019 426 275 255 20 0 0
2020 330 207 172 15 1 0
2021 23 0 0 0 0 0
TOTAL 76366 46607 41778 4469 14 4

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 50 case in which the diagnosis is pending (2 from 2019, 25 from 2020 and 23 from 2021), and 19 inconclusive cases; 
7Includes 19 (19from 2020) cases with type determination pending in which the diagnosis of vCJD has been excluded. 
8The sporadic cases include 4068 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 74 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 35 cases of sporadic Fatal Insomnia (sFI). 
9Total does not include 279 Familial cases diagnosed by blood test only.