Tables of Cases Examined

National Prion Disease Pathology Surveillance Center Cases Examined¹

Updated quarterly.
Last updated on: December 10th, 2021

Year	Total Referrals²	Prion Disease	Sporadic	Familial	Iatrogenic	vCJD
1999 & earlier	383	231	201	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	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	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	395	277	248	29	0	0
2017	375	266	247	19	0	0
2018	308	221	202	18	1	0
2019	432	280	259	21	0	0
2020	362	250	225	24	1	0
2021	282	183	157	15	0	0
TOTAL	7932⁶	4889⁷	4389⁸	460⁹	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 35 cases in which the diagnosis is pending (1 from 1999, 2 from 2020 and 32 from 2021), and 19 inconclusive cases;
⁷Includes 11 (11 from 2021) cases with type determination pending in which the diagnosis of vCJD has been excluded.
⁸The sporadic cases include 4276 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 76 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 37 cases of sporadic Fatal Insomnia (sFI).
⁹Total does not include 291 Familial cases diagnosed by blood test only.