National Prion Disease Pathology Surveillance Center Cases Examined¹
Updated quarterly.
Last updated on: July 5th, 2022
| Year | Total 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 | 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 | 433 | 281 | 259 | 22 | 0 | 0 |
| 2020 | 364 | 252 | 227 | 24 | 1 | 0 |
| 2021 | 341 | 247 | 222 | 22 | 0 | 0 |
| 2022 | 128 | 75 | 58 | 8 | 0 | 0 |
| TOTAL | 81226 | 50327 | 45158 | 4879 | 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 23 cases in which the diagnosis is pending (1 from 2020, 4 from 2021 and 18 from 2022), and 20 inconclusive cases;
7Includes 12 (3 from 2021 and 9 from 2022) cases with type determination pending in which the diagnosis of vCJD has been excluded.
8The sporadic cases include 4399 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 79 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 37 cases of sporadic Fatal Insomnia (sFI).
9Total does not include 299 Familial cases diagnosed by blood test only.