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.