Tables of Cases Examined

Updated Apr 11, 2019

Year Total Referrals² Prion Disease Sporadic Familial Iatrogenic vCJD
1999 & earlier 380 230 200 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
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 402 264 244 20 0 0
2016 397 278 248 29 0 0
2017 374 266 245 19 0 0
2018 304 219 194 17 1 0
2019 95 45 28 1 0 0
TOTAL 69466 4220 3766 411 13 4

1Listed based on the year of death or, if not available, on 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 25 cases in which the diagnosis is pending, and 19 inconclusive cases; 7Includes 26 (16 from 2019) cases with type determination pending in which the diagnosis of vCJD has been excluded. 8The sporadic cases include 3669 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 64 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 33 cases of sporadic Fatal Insomnia (sFI). 9Total does not include 249 Familial cases diagnosed by blood test only.

Updated Mar 31, 2019

Year1

CSF RT-QuIC (+) Cases

Probable2 & Definite Prion Disease Cases Submitted to NPDPSC

2016

356

462
2017 395 491
2018 420 494
20193 96 133

1Listed based on the year of the patient’s first CSF submission
2RT-QuIC positive without neuropathological examination
32019 data based on the date range of 1/1/2019-3/31/2019