National Prion Disease Pathology Surveillance Center Cases Examined¹
Updated July 8th, 2020
| Year | Total Referrals² | Prion Disease | Sporadic | Familial | Iatrogenic | vCJD |
|---|---|---|---|---|---|---|
| 1999 & earlier | 381 | 230 | 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 | 418 | 270 | 247 | 21 | 0 | 0 |
| 2020 | 189 | 90 | 65 | 6 | 1 | 0 |
| TOTAL | 74636 | 45377 | 40658 | 4389 | 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 32 cases in which the diagnosis is pending, and 19 inconclusive cases; 7Includes 20 (2 from 2019, 18 from 2020) cases with type determination pending in which the diagnosis of vCJD has been excluded. 8The sporadic cases include 3956 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 70 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 35 cases of sporadic Fatal Insomnia (sFI). 9Total does not include 275 Familial cases diagnosed by blood test only.