Tables of Cases Examined

Updated Feb 1, 2019

Year Total Referrals² Prion Disease Sporadic Familial Iatrogenic vCJD
1998 & earlier 259 157 135 20 2 0
1999 121 73 65 7 1 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
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 402 246 218 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 354 208 185 21 1 1⁵
2015 402 264 244 20 0 0
2016 397 278 248 29 0 0
2017 373 266 244 19 0 0
2018 296 209 171 14 0 0
2019 8 4 0 0 0 0
TOTAL 6850 4169 3714 407 12 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 12 cases in which the diagnosis is pending, and 20 inconclusive cases; 7Includes 13 (8 from 2018) cases with type determination pending in which the diagnosis of vCJD has been excluded. 8The sporadic cases include 3560 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 63 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 33 cases of sporadic Fatal Insomnia (sFI). 9Total does not include 247 Familial cases diagnosed by blood test only.

Updated Feb 14, 2019

Year1

CSF RT-QuIC (+) Cases

Probable2 & Definite Prion Disease Cases Submitted to NPDPSC

2016

356

467
2017 395 492
2018 420 498

1Listed based on the year of the patient’s first CSF submission
2RT-QuIC positive without neuropathological examination