National Prion Disease Pathology Surveillance Center Cases Examined¹
Updated quarterly.
Last updated on: October 8th, 2020
| Year | Total Referrals² | Prion Disease | Sporadic | Familial | Iatrogenic | vCJD |
|---|---|---|---|---|---|---|
| 1999 & earlier | 382 | 231 | 200 | 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 | 316 | 181 | 164 | 16 | 0 | 1³ |
| 2005 | 327 | 178 | 156 | 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 | 422 | 274 | 252 | 21 | 0 | 0 |
| 2020 | 252 | 159 | 125 | 11 | 1 | 0 |
| TOTAL | 75316 | 46117 | 41268 | 4439 | 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 12 cases in which the diagnosis is pending, and 19 inconclusive cases;
7Includes 24 (1 from 1986, 1 from 2019, 22 from 2020) cases with type determination pending in which the diagnosis of vCJD has been excluded.
8The sporadic cases include 4020 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 71 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 35 cases of sporadic Fatal Insomnia (sFI).
9Total does not include 277 Familial cases diagnosed by blood test only.