National Prion Disease Pathology Surveillance Center Cases Examined¹
Updated quarterly.
Last updated on: September 2nd, 2023
| Year | Total Neuropath 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 | 330 | 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 | 402 | 247 | 219 | 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 | 262 | 243 | 19 | 0 | 0 |
| 2016 | 395 | 277 | 248 | 29 | 0 | 0 |
| 2017 | 375 | 266 | 247 | 18 | 0 | 0 |
| 2018 | 308 | 221 | 202 | 18 | 1 | 0 |
| 2019 | 434 | 281 | 259 | 22 | 0 | 0 |
| 2020 | 367 | 253 | 228 | 24 | 1 | 0 |
| 2021 | 344 | 248 | 224 | 22 | 0 | 0 |
| 2022 | 337 | 228 | 206 | 21 | 0 | 0 |
| 2023 | 193 | 114 | 101 | 8 | 0 | 0 |
| TOTAL | 85346 | 53017 | 47208 | 5079 | 14 | 4 |
| Year | CSF Only and RT-QuIC Positive10 |
|---|---|
| 2015 | 140 |
| 2016 | 183 |
| 2017 | 227 |
| 2018 | 266 |
| 2019 | 310 |
| 2020 | 309 |
| 2021 | 331 |
| 2022 | 347 |
| 2022 | 240 |
| TOTAL | 2357 |
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 28 cases in which the diagnosis is pending (2 from 2022 and 31 from 2023), and 20 inconclusive cases;
7Includes 8 (2 from 2021, 1 from 2022, and 5 from 2023) cases with type determination pending in which the diagnosis of vCJD has been excluded.
8The sporadic cases include 4641 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 88 cases of Variably Protease-Sensitive Prionopathy (VPSPr), and 39 cases of sporadic Fatal Insomnia (sFI).
9Total does not include 312 Familial cases diagnosed by blood test only.
10Lists number of patients (deceased and alive) who have had a positive RT-QuIC and no neuropath examination.
For a downloadable PDF version of our quarterly table, please click the link below: