632:, who died in 2019, at the age of 33, had been the victim of a work accident in 2010, during which she had pricked herself with a tool contaminated with infected brain. The efficacy of this route of contamination has been unambiguously demonstrated in primates. Pierrette C., who died in 2021, had been victim of the same type of work accident. After her diagnosis, a moratorium was initiated in all French laboratories on research activities on infectious prions. In March 2022, INRAE recognized the occupational cause of these two deaths. This raises serious questions about the safety of personnel in these laboratories. Indeed, inspections have noted serious failures in the protection of agents in the face of this deadly risk, and the long incubation period of this disease leads to fears of new cases in the future, hence great concern.
536:(NZBS) in 2000 introduced measures to preclude permanently donors having resided in the United Kingdom (including the Isle of Man and the Channel Islands) for a total of six months or more between January 1980 and December 1996. The measure resulted in ten percent of New Zealand's active blood donors at the time becoming ineligible to donate blood. In 2003, the NZBS further extended restrictions to permanently preclude donors having received a blood transfusion in the United Kingdom since January 1980, and in April 2006, restrictions were further extended to include the
920:
962:, particularly with cows being fed with cow and sheep remains. Furthermore, the report was critical of the way the crisis had been handled. There was a reluctance to consider the possibility that BSE could cross the species barrier. The government assured the public that British beef was safe to eat, with agriculture minister John Gummer famously feeding his daughter a burger. The British government were reactive more than proactive in response; the worldwide ban on all British beef exports in March 1996 was a serious economic blow.
906:
than found previously and in all genotypes, indicating "infection" may be relatively common. This new study examined over 32,000 anonymous appendix samples. Of these, 16 samples were positive for abnormal prion protein, indicating an overall prevalence of 493 per million population, or one in 2,000 people are likely to be carriers. No difference was seen in different birth cohorts (1941–1960 and 1961–1985), in both sexes, and there was no apparent difference in abnormal prion prevalence in three broad geographical areas.
809:
894:
60:
691:
469:, stated that bovine spongiform encephalopathy (BSE) is the first man-made epidemic, or "Frankenstein" disease, because a human decision to feed meat and bone meal to previously herbivorous cattle (as a source of protein) caused what was previously an animal pathogen to enter into the human food chain, and from there to begin causing humans to contract vCJD.
489:, and other countries in 2003 immediately following the first reported case of BSE in American cattle. Stringent preventative and surveillance practices implemented since then to prevent the disease from entering the human and cattle food chains have caused some to conclude that such bans are unnecessary.
910:
of the 16 positive samples revealed a higher proportion of valine homozygous (VV) genotype on the codon 129 of the gene encoding the prion protein (PRNP) compared with the general UK population. This also differs from the 176 people with vCJD, all of whom to date have been methionine homozygous (MM)
863:
Prion protein is detectable in lymphoid and appendix tissue up to two years before the onset of neurological symptoms in vCJD. Large scale studies in the UK have yielded an estimated prevalence of 493 per million, higher than the actual number of reported cases. This finding indicates a large number
551:
In Canada, individuals were formerly ineligible to donate blood or plasma if they had spent a cumulative total of three months or more in the mainland UK or its Crown
Dependencies or six months or more in Saudi Arabia from January 1, 1980, through December 31, 1996. They were also ineligible if they
517:. Whilst these restrictions may go some way to preventing a self-sustaining epidemic of secondary infections, the number of infected blood donations is unknown and could be considerable. In June 2013 the government was warned that deaths, then at 176, could rise five-fold through blood transfusions.
884:
scientists expressed concern over the possibility of a second wave of human cases due to the wide exposure and long incubation of some cases of vCJD. In 2015, a man from New York developed vCJD after eating squirrel brains. From
November 2017 to April 2018, four suspected cases of the disease arose
524:
instituted a policy that excludes from blood donation anyone having spent at least six months in certain
European countries (or three months in the United Kingdom) from 1980 to 1996. Given the large number of U.S. military personnel and their dependents residing in Europe, it was expected that over
793:
prions). Both classic and variant CJD are subtypes of
Creutzfeldt–Jakob disease. There are three main categories of CJD disease: sporadic CJD, hereditary CJD, and acquired CJD, with variant CJD being in the acquired group along with iatrogenic CJD. The classic form includes sporadic and hereditary
905:
teenager
Stephen Churchill died on 23 May 1995, aged 19. Researchers believe one in 2,000 people in the UK is a carrier of the disease, linked to eating contaminated beef. The survey provides the most robust prevalence measure to date—and identifies abnormal prion protein across a wider age group
927:
In 2000 a voluntary support group was formed by families of people who had died from vCJD. The goal was to support other families going through a similar experience. This support was provided through a
National Helpline, a Carer's Guide, a website and a network of family befriending. The support
965:
The foundation had been calling for compensation to include a care package to help relatives look after those with vCJD. There have been widespread complaints of inadequate health and social services support. Following the
Phillips Report in October 2001, the government announced a compensation
525:
7% of donors would be deferred due to the policy. Later changes to this policy first relaxed the restriction to a cumulative total of five years or more of civilian travel in
European countries (six months or more if military) then, in 2022, removed it entirely.
2333:
677:
at this position. Only a single person with vCJD tested was found to be heterozygous; most of those affected had two copies of the methionine-containing form. It is not yet known whether those unaffected are actually immune or only have a longer
837:. In the 1950s, cannibalism was banned in Papua New Guinea. In the late 20th century, however, kuru reached epidemic proportions in certain Papua New Guinean communities, therefore suggesting that vCJD may also have a similar
472:
The risk of contracting vCJD from ingestion of cattle products has led to many countries banning the import of beef from countries where BSE has been known to occur, such as the ban on beef from the United States imposed by
547:
Similar regulations are in place where anyone having spent more than six months for
Germany or one year for France living in the UK between January 1980 and December 1996 is permanently banned from donating blood.
409:, and 50 cases in the rest of the world. The disease has become less common since 2000. The typical age of onset is less than 30 years old. It was first identified in 1996 by the National CJD Surveillance Unit in
508:
of the disease could result in the near future. A blood test for vCJD infection is possible but is not yet available for screening blood donations. Significant restrictions exist to protect the blood supply. The
3438:
3423:
2415:
656:
Despite the consumption of contaminated beef in the UK being high, vCJD has infected a small number of people. One explanation for this can be found in the genetics of people with the disease. The human
2308:
841:
of 20 to 50 years. A critique to this theory is that while mortuary cannibalism was banned in Papua New Guinea in the 1950s, that does not necessarily mean that the practice ended. Fifteen years later
624:
In France, the last two victims of variant
Creutzfeldt–Jakob disease, who died in 2019 and 2021, were research technicians at the National Research Institute for Agriculture, Food and the Environment (
1770:
513:
banned anyone who had received a blood transfusion since January 1980 from donating blood. Since 1999 there has been a ban in the UK for using UK blood to manufacture fractional products such as
1427:
552:
had spent a cumulative total of five years or more in France or the Republic of Ireland from January 1, 1980, through 31 December 2001. These restrictions were removed by December 2023.
1842:
1595:
Edgeworth JA, Farmer M, Sicilia A, Tavares P, Beck J, Campbell T, et al. (February 2011). "Detection of prion infection in variant Creutzfeldt-Jakob disease: a blood-based assay".
2748:
Collinge J, Whitfield J, McKintosh E, Beck J, Mead S, Thomas DJ, Alpers MP (June 2006). "Kuru in the 21st century--an acquired human prion disease with very long incubation periods".
2358:
2383:
3167:
812:
Dark green areas are countries that have confirmed human cases of variant Creutzfeldt–Jakob disease and light green are countries that have bovine spongiform encephalopathy cases
2232:
1719:
856:. They have also proposed that individuals having contracted CJD in the early 1990s represent a distinct genetic subpopulation, with unusually short incubation periods for
928:
groups had an internet presence at the turn of the 21st century. The driving force behind the foundation was Lester Firkins, whose young son had died from the disease.
1997:
2207:
897:
Deaths in the UK from Creutzfeldt–Jakob disease 1990–2014: while cases of vCJD have declined (green), reported cases of sporadic CJD continue to increase (blue)
2086:
1763:
497:
As of 2018, evidence suggests that there may be prions in the blood of individuals with vCJD, but this is not the case in individuals with sporadic CJD.
3697:
3550:
2623:
1419:
969:
A memorial plaque for those who have died due to vCJD was installed in central London in approximately 2000. It is located on the boundary wall of
951:
936:
1552:
Peden AH, Head MW, Ritchie DL, Bell JE, Ironside JW (2004). "Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient".
616:
sperm. Despite this, the scientific consensus is that the risk is negligible, as there is no evidence Creutzfeldt–Jakob is sexually transmitted.
3012:
2046:
Mortimer D, Barratt CL (December 2006). "Is there a real risk of transmitting variant Creutzfeldt-Jakob disease by donor sperm insemination?".
1850:
911:
genotype. The concern is that individuals with this VV genotype may be susceptible to developing the condition over longer incubation periods.
761:, or visual signs. (If persistent painful sensory symptoms exist, ≥4 months' delay in the development of the neurologic signs is not required).
2359:"Deuxième mission d'expertise de la sécurité dans les laboratoires de recherche sur les prions infectieux - conditions de sortie du moratoire"
1921:
625:
2965:
2441:
2334:"Creutzfeldt-Jakob : la contamination de deux anciennes salariées relevait bien de l'accident du travail, confirme l'INRAE de Toulouse"
1817:
669:
at amino acid 129, without any apparent physiological difference. Of the overall white population, about 40% have two methionine-containing
1454:
943:
3180:
2020:
2724:"International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10)-WHO Version for 2016 — A81.0"
1939:
3505:
860:(BSE). This means that there may be many more people with vCJD with longer incubation periods, which may surface many years later.
749:
Dementia, and development ≥4 months after illness onset of at least two of the following five neurologic signs: poor coordination,
629:
1727:
363:. The length of time between exposure and the development of symptoms is unclear, but is believed to be years to decades. Average
3591:
3518:
2987:
2467:
344:
202:
739:
Current age or age at death less than 55 years (a brain autopsy is recommended, however, for all physician-diagnosed CJD cases).
1896:
729:
Spongiform change and extensive prion protein deposition shown by immunohistochemistry throughout the cerebellum and cerebrum.
3565:
2550:
786:
2416:"ENTRETIEN. Maladie de Creutzfeldt Jakob : " On demande un recensement des personnels qui ont travaillé sur le prion ""
3091:"Prevalent abnormal prion protein in human appendixes after bovine spongiform encephalopathy epizootic: large scale survey"
1369:"Prevalence in Britain of abnormal prion protein in human appendices before and after exposure to the cattle BSE epizootic"
206:
2309:"L'Inrae reconnaît que la technicienne toulousaine morte de Creutzfeldt-Jakob a été victime d'un accident de laboratoire"
932:
3272:
1989:
966:
scheme for British people affected with vCJD. The multi-million-pound financial package was overseen by the vCJD Trust.
3075:
437:. The length of time between exposure and the development of symptoms is unclear, but is believed to be years. Average
589:
between the years 1980 and 1996 or received transfusion in the UK after the year 1980 is not allowed to donate blood.
3661:
1638:
1479:
1351:
3666:
3641:
880:
brains. A coincidental relationship between the disease and this dietary practice may have been involved. In 2008,
857:
510:
455:
375:
336:
185:
50:
29:
2082:
3646:
81:
600:
for a total of over six months between 1 January 1980 and 31 December 1996 is permanently barred from donating.
3671:
2233:"Toulouse : la technicienne de l'Inrae morte de Creutzfeldt-Jakob avait déclaré deux accidents du travail"
559:, anyone having spent cumulatively six months or longer between 1 January 1980 and 31 December 1996 in the UK,
340:
3609:
3581:
3545:
3483:
1749:
391:
22:
800:
has no separate code for vCJD and such cases are reported under the Creutzfeldt–Jakob disease code (A81.0).
3692:
3498:
3471:
1451:"Summary results of the second national survey of abnormal prion prevalence in archived appendix specimens"
1027:
852:
These researchers noticed a genetic variation in some people with kuru that has been known to promote long
521:
2838:
2533:
Sikorska, B, Liberski, PP (2012). "Human Prion Diseases: From Kuru to Variant Creutzfeldt-Jakob Disease".
2384:"Recherche sur les prions : un rapport d'inspection dénonce des conditions de sécurité insuffisantes"
710:
is required to confirm a diagnosis of variant CJD. The following confirmatory features should be present:
682:
until symptoms appear. Studies in transgenetic mice indicate that all of these genotypes can be affected.
574:
a total of over one year between 1 January 1980 and 31 December 1996 is permanently barred from donating.
3138:
3040:
406:
3354:
2912:
Berger JR, Waisman E, Weisman B (August 1997). "Creutzfeldt-Jakob disease and eating squirrel brains".
425:, behavioral changes, and painful sensations. In the later stages of the illness, patients may exhibit
351:, behavioral changes, and painful sensations. In the later stages of the illness, patients may exhibit
3299:
533:
223:
742:
Psychiatric symptoms at illness onset and/or persistent painful sensory symptoms (frank pain and/or
3560:
459:
2723:
1872:
1341:
612:, motivated by a risk of variant Creutzfeldt–Jakob disease, inhibiting the once popular import of
3656:
3491:
3089:
Gill ON, Spencer Y, Richard-Loendt A, Kelly C, Dabaghian R, Boyes L, et al. (October 2013).
2955:
1821:
940:
821:
in 2006 suggested that it may take more than 50 years for vCJD to develop, from their studies of
465:
Jonathan Quick, instructor of medicine at the Department of Global Health and Social Medicine at
132:
2863:
Diack AB, Head MW, McCutcheon S, Boyle A, Knight R, Ironside JW, et al. (1 November 2014).
2108:
Brandel JP, Vlaicu MB, Culeux A, Belondrade M, Bougard D, Grznarova K, et al. (July 2020).
1450:
1420:"Creutzfeldt-Jakob Disease Fact Sheet | National Institute of Neurological Disorders and Stroke"
3707:
970:
466:
379:
214:
3326:
3205:
3432:
1469:
398:
2574:
Diack AB, Head MW, McCutcheon S, Boyle A, Knight R, Ironside JW, Manson JC, Will RG (2014).
2024:
1028:"Clinical and Pathologic Characteristics | Variant Creutzfeldt-Jakob Disease, Classic (CJD)"
2266:
1082:
833:
in Papua New Guinea when family members would eat the body of a dead relative as a sign of
694:
422:
253:
137:
101:
1367:
Gill ON, Spencer Y, Richard-Loendt A, Kelly C, Brown D, Sinka K, et al. (June 2020).
776:
No history of CJD in a first degree relative or prion protein gene mutation in the person.
773:
No history of getting human pituitary growth hormone or a dura mater graft from a cadaver.
764:
A normal or an abnormal EEG, but not the diagnostic EEG changes often seen in classic CJD.
8:
3175:
1943:
560:
537:
2270:
1286:
3115:
3090:
2937:
2889:
2864:
2819:
2773:
2674:
2649:
2600:
2575:
2290:
2183:
2158:
2139:
1695:
1670:
1620:
1577:
1529:
1496:
1393:
1368:
1181:
1121:
697:
of a person with suspected CJD showing typical periodic bursts of triphasic sharp waves
597:
219:
92:
2925:
2761:
2157:
Mikol J, Delmotte J, Jouy D, Vaysset E, Bastian C, Deslys JP, Comoy E (January 2021).
2059:
1608:
1565:
3702:
3254:
3249:
3232:
3120:
2929:
2894:
2811:
2765:
2679:
2605:
2556:
2546:
2515:
2391:
2294:
2282:
2188:
2143:
2131:
2063:
1700:
1612:
1569:
1534:
1516:
1475:
1398:
1347:
1268:
1173:
1168:
1151:
978:
959:
919:
853:
838:
830:
679:
501:
192:
76:
2941:
2823:
2777:
1900:
1624:
1581:
1185:
49:
New variant Creutzfeldt–Jakob disease (nvCJD) (dated), human mad cow disease, human
3380:
3244:
3110:
3102:
3045:
2921:
2884:
2876:
2803:
2757:
2669:
2661:
2595:
2587:
2538:
2505:
2274:
2178:
2170:
2121:
2110:"Variant Creutzfeldt-Jakob Disease Diagnosed 7.5 Years after Occupational Exposure"
2055:
1690:
1682:
1604:
1561:
1524:
1508:
1388:
1380:
1163:
826:
390:
but can be suspected based on certain other criteria. It is different from typical
958:. The report concluded that the escalation of BSE into a crisis was the result of
3017:
2665:
2542:
2278:
1202:
907:
438:
374:, which are misfolded proteins. Spread is believed to be primarily due to eating
364:
348:
241:
160:
141:
2535:
Protein Aggregation and Fibrillogenesis in Cerebral and Systemic Amyloid Disease
1512:
3619:
3586:
3555:
2208:"Recherche sur les prions, la sécurité des laboratoires français mise en cause"
2174:
1384:
990:
829:. The reasoning behind the claim is that kuru was possibly transmitted through
822:
715:
609:
578:
571:
402:
303:
121:
3513:
3447:
3375:
3686:
3304:
3067:
2395:
1686:
1520:
1314:
1228:
842:
758:
383:
2697:
2257:
Casassus B (July 2021). "France halts prion research amid safety concerns".
644:
is not recommended as one person contracted vCJD from eating the brain of a
3331:
3258:
3210:
3124:
2960:
2898:
2815:
2769:
2683:
2609:
2560:
2519:
2286:
2192:
2135:
2067:
1704:
1616:
1573:
1538:
1402:
1272:
1177:
955:
808:
707:
674:
462:
found that around 1 in 2000 had abnormal prions present in appendix cells.
387:
198:
2933:
1645:
923:
Commemorative plaque in London paying tribute to people who died from vCJD
2842:
2471:
2159:"Direct neural transmission of vCJD/BSE in macaque after finger incision"
2126:
2109:
947:
743:
613:
529:
478:
273:
179:
105:
3415:
1083:"Diagnostic Criteria | Variant Creutzfeldt-Jakob Disease, Classic (CJD)"
770:
Routine investigations do not suggest an alternative, non-CJD diagnosis.
1471:
The End of Epidemics: The Looming Threat to Humanity and How to Stop it
817:
719:
662:
454:
In the UK, the primary cause of vCJD has been eating beef tainted with
265:
248:
3106:
2880:
2591:
2510:
2493:
596:, anyone having lived or stayed in the mainland United Kingdom or its
378:(BSE) infected beef. Infection is also believed to require a specific
2807:
2494:"The genetics of susceptibility to variant Creutzfeldt-Jakob disease"
1964:
996:
902:
750:
641:
434:
410:
360:
281:
269:
261:
125:
85:
1795:
1259:
Ironside JW (2012). "Variant Creutzfeldt-Jakob disease: an update".
3466:
2988:"Man Dies from Extremely Rare Disease After Eating Squirrel Brains"
2839:"Eating Brains: Cannibal Tribe Evolved Resistance to Fatal Disease"
2468:"Man Dies from Extremely Rare Disease After Eating Squirrel Brains"
877:
873:
834:
723:
645:
505:
430:
356:
257:
117:
2442:"Kentucky Doctors Warn Against a Regional Dish: Squirrels' Brains"
1942:(in Czech). Fakultní nemocnice Královské Vinohrady. Archived from
661:
protein which is subverted in prion disease can occur with either
3651:
3376:"memorial should be moved from listed wall, say Lambeth planners"
974:
846:
593:
514:
1764:"CJD (Creutzfeldt–Jakob Disease) - Information for blood donors"
1229:"Variant Creutzfeldt-Jakob Disease (VCJD) | Prion Diseases"
901:
The first human death from vCJD occurred in the United Kingdom;
673:, 10% have two valine-containing alleles, and the other 50% are
3442:
3427:
893:
797:
754:
670:
666:
586:
567:
556:
541:
486:
482:
426:
352:
113:
71:
in variant CJD. PrP deposits are stained brown and are visible.
68:
3088:
3013:"Agonising decline that led to first diagnosis of new illness"
2747:
1366:
59:
3514:
2107:
1594:
474:
371:
171:
3233:"From nannyism to public disclosure: the BSE inquiry report"
3536:
2537:. Subcellular Biochemistry. Vol. 65. pp. 457–96.
1922:"Quelles sont les contre-indications au don de sang ?"
790:
658:
504:. The finding alarmed healthcare officials because a large
690:
401:. As of 2020, 178 cases of vCJD have been recorded in the
2626:. National Institute of Neurological Disorders and Stroke
718:-type amyloid plaques surrounded by vacuoles in both the
500:
In 2004, a report showed that vCJD can be transmitted by
2862:
2573:
2156:
1287:"Creutzfeldt-Jakob disease in the UK (by calendar year)"
544:. The restriction was rescinded in late February 2024.
2532:
881:
876:
developed CJD. It was discovered that all had consumed
582:
3355:"CREUTZFELDT-JAKOB DISEASE SUPPORT NETWORK NEWSLETTER"
2698:"About CJD | Creutzfeldt-Jakob Disease, Classic (CJD)"
1897:"Permanent exclusion criteria / Dyskwalifikacja stała"
1820:(in German). Blutspendedienst Hamburg. Archived from
1644:. National Blood Service. August 2004. Archived from
1442:
1233:
U.S. Centers for Disease Control and Prevention (CDC)
3405:
3160:
3004:
2911:
2865:"Variant CJD. 18 years of research and surveillance"
2794:
Diamond JM (September 2000). "Talk of cannibalism".
1551:
386:
or contaminated surgical equipment. Diagnosis is by
3297:
2743:
2741:
1495:Kim KS, Kim T, Choi H, Ahn C, Lee CC (July 2016).
1343:Ferri's Clinical Advisor 2018 E-Book: 5 Books in 1
1077:
1075:
1073:
1071:
1069:
581:, anyone having spent more than six months in the
3265:
3198:
1750:"Learn More About FDA's Updated Guidance on VCJD"
1067:
1065:
1063:
1061:
1059:
1057:
1055:
1053:
1051:
1049:
563:, or France is permanently barred from donating.
3684:
3146:The Patient - Patient-Centered Outcomes Research
3131:
1448:
939:was published. The BSE report criticised former
640:Eating other types of brains such as those from
3032:
2738:
2045:
864:of asymptomatic cases and the need to monitor.
1309:
1307:
1305:
1303:
1046:
608:In the U.S., the FDA has banned import of any
441:following the onset of symptoms is 13 months.
367:following the onset of symptoms is 13 months.
3499:
1494:
1203:"Treatment Variant Creutzfeldt-Jakob Disease"
1116:
1114:
1112:
1110:
1108:
1106:
1104:
1022:
1020:
1018:
1016:
1014:
1012:
931:In October 2000 the report of the government
794:forms. Sporadic CJD is the most common type.
619:
2205:
1873:"Eligibility for mad cow-affected countries"
1717:
1545:
3291:
3168:"James Lind Alliance Affiliates Newsletter"
1940:"Blood donor guidance / Poučení dárce krve"
1899:(in Polish). RCKiK Warszawa. Archived from
1668:
1300:
1197:
1195:
16:Degenerative brain disease caused by prions
3506:
3492:
3319:
3068:"Estimate doubled for vCJD carriers in UK"
2789:
2787:
1796:"Variant Creutzfeldt-Jakob Disease (vCJD)"
1497:"Beef from the United States: Is It Safe?"
1467:
1414:
1412:
1254:
1252:
1250:
1248:
1246:
1244:
1242:
1145:
1143:
1101:
1009:
197:Suspected based on symptoms, confirmed by
58:
3698:Transmissible spongiform encephalopathies
3248:
3114:
2888:
2673:
2647:
2599:
2567:
2509:
2491:
2439:
2182:
2125:
1694:
1528:
1392:
1346:. Elsevier Health Sciences. p. 343.
1167:
3368:
3298:Ainsworth C, Carrington D (2019-10-25).
2660:(6 Neuroinfectious Disease): 1612–1638.
2256:
2039:
1990:"Mad Cow Rules Hit Sperm Banks' Patrons"
1720:"Mad cow infected blood 'to kill 1,000'"
1258:
1192:
1149:
918:
892:
807:
689:
382:. Spread may potentially also occur via
294:Fewer than 250 reported cases as of 2012
3592:Variably protease-sensitive prionopathy
3551:Gerstmann–Sträussler–Scheinker syndrome
3519:transmissible spongiform encephalopathy
2956:"Warning over second wave of CJD cases"
2836:
2793:
2784:
2643:
2641:
2465:
2080:
2074:
1843:"Les contre-indications au don de sang"
1409:
1239:
1140:
914:
570:, anyone having lived or stayed in the
345:transmissible spongiform encephalopathy
203:Protein misfolding cyclic amplification
3685:
3010:
2624:"Creutzfeldt-Jakob Disease Fact Sheet"
2018:
2012:
1711:
1474:. St. Martin's Press. pp. 51–53.
867:
3487:
3038:
2616:
1987:
1981:
1339:
767:Duration of illness of over 6 months.
416:
321:New variant Creutzfeldt–Jakob disease
2638:
1457:from the original on March 25, 2013.
1449:HPA Press Office (August 10, 2012).
207:Real-time quaking-induced conversion
2114:The New England Journal of Medicine
2089:from the original on 8 January 2017
1152:"Variant Creutzfeldt-Jakob disease"
327:) and referred to colloquially as "
13:
2968:from the original on 28 March 2017
2000:from the original on 26 April 2012
1776:from the original on 10 April 2017
1662:
1631:
1588:
1461:
1360:
1333:
785:vCJD is a separate condition from
520:On 28 May 2002, the United States
14:
3719:
3662:Transmissible mink encephalopathy
3401:
2726:. World Health Organization. 2016
2363:enseignementsup-recherche.gouv.fr
1501:Journal of Korean Medical Science
888:
872:In 1997, a number of people from
787:classic Creutzfeldt–Jakob disease
780:
603:
492:
394:, though both are due to prions.
347:family. Initial symptoms include
313:Variant Creutzfeldt–Jakob disease
41:Variant Creutzfeldt–Jakob disease
3667:Feline spongiform encephalopathy
3642:Bovine spongiform encephalopathy
3139:"Pioneer profile: Lester Firkin"
3078:from the original on 2014-02-10.
1639:"Variant CJD and blood donation"
1430:from the original on 4 July 2017
1169:10.1111/j.1365-2516.2010.02317.x
1122:"Classic CJD versus Variant CJD"
858:bovine spongiform encephalopathy
456:bovine spongiform encephalopathy
376:bovine spongiform encephalopathy
339:counterpart, is a fatal type of
186:bovine spongiform encephalopathy
30:Bovine spongiform encephalopathy
3647:Camel spongiform encephalopathy
3347:
3225:
3082:
3060:
3041:"The 'Mad Cow Disease' Scandal"
2980:
2948:
2905:
2856:
2830:
2716:
2690:
2526:
2485:
2459:
2433:
2408:
2376:
2351:
2326:
2301:
2250:
2225:
2199:
2150:
2101:
2081:Lapidos J (26 September 2007).
2048:Reproductive Biomedicine Online
1957:
1932:
1914:
1889:
1865:
1835:
1810:
1788:
1756:
1742:
1718:Rowena Mason (April 28, 2013).
1669:Regan F, Taylor C (July 2002).
1488:
803:
635:
449:
28:For the disease in cattle, see
3672:Exotic ungulate encephalopathy
2648:Geschwind MD (December 2015).
2466:Rettner R (October 15, 2018).
2440:Blakeslee S (29 August 1997).
2019:Kotler S (27 September 2007).
1847:Etablissement français du sang
1818:"Permanent exclusion criteria"
1279:
1221:
1:
3327:"BSE victims to get millions"
3243:(2): 165, 167. January 2001.
2926:10.1016/S0140-6736(05)63333-8
2762:10.1016/S0140-6736(06)68930-7
2060:10.1016/S1472-6483(10)61024-3
1769:. New Zealand Blood Service.
1609:10.1016/S0140-6736(10)62308-2
1566:10.1016/S0140-6736(04)16811-6
1002:
993:, a person who died from vCJD
977:facing the Riverside Walk of
849:that the practice continued.
701:
335:" to distinguish it from its
272:(for involuntary movements),
3179:. 2012-01-01. Archived from
2666:10.1212/CON.0000000000000251
2543:10.1007/978-94-007-5416-4_17
2365:(in French). 26 January 2022
2279:10.1126/science.373.6554.475
1671:"Blood transfusion medicine"
733:
685:
651:
522:Food and Drug Administration
397:Treatment for vCJD involves
233:Not eating contaminated beef
151:Years after initial exposure
7:
3566:Huntington's disease-like 1
3300:"BSE disaster: the history"
2837:Rettner R (June 12, 2015).
2206:La-Croix.com (2021-12-06).
2023:. LA Weekly. Archived from
1798:. New Zealand Blood Service
1513:10.3346/jkms.2016.31.7.1009
984:
789:(though both are caused by
184:Eating beef from cows with
10:
3724:
3364:. October 2016. p. 3.
3011:Arthur C (19 March 1997).
2340:(in French). 16 March 2022
2175:10.1007/s00401-020-02231-w
1988:Stein R (13 August 2008).
1926:www.santepubliquefrance.fr
1468:Quick HD, Fryer B (2018).
1385:10.1007/s00401-020-02153-7
620:Occupational contamination
27:
20:
3632:
3600:
3574:
3534:
3525:
3457:
3409:
2492:Saba R, Booth SA (2013).
2390:(in French). 2022-01-31.
1150:Ironside JW (July 2010).
534:New Zealand Blood Service
421:Initial symptoms include
392:Creutzfeldt–Jakob disease
298:
290:
280:
247:
237:
229:
224:Creutzfeldt-Jakob disease
213:
191:
178:
167:
155:
147:
131:
91:
75:
66:
57:
45:
40:
23:Creutzfeldt–Jakob disease
3561:PrP systemic amyloidosis
3273:"The BSE Inquiry Report"
3039:Wells C (Jan 16, 2022).
1726:. London. Archived from
1687:10.1136/bmj.325.7356.143
460:Health Protection Agency
444:
21:Not to be confused with
3657:Chronic wasting disease
3587:Sporadic fatal insomnia
3556:Fatal familial insomnia
825:, a similar disease in
163:after onset of symptoms
3206:"BSE crisis: Timeline"
2498:Public Health Genomics
1261:Folia Neuropathologica
924:
898:
813:
698:
467:Harvard Medical School
458:. A 2012 study by the
380:genetic susceptibility
215:Differential diagnosis
104:, behavioral changes,
3277:The Health Foundation
2163:Acta Neuropathologica
1373:Acta Neuropathologica
922:
896:
811:
693:
435:involuntary movements
361:involuntary movements
333:human mad cow disease
319:), formerly known as
126:involuntary movements
3214:. London. 2019-10-26
2127:10.1056/NEJMc2000687
2083:"Is Mad Cow an STD?"
1162:(Suppl 5): 175–180.
915:Human BSE Foundation
714:Numerous widespread
695:Electroencephalogram
423:psychiatric problems
349:psychiatric problems
254:Pentosan polysulfate
138:aspiration pneumonia
102:Psychiatric problems
3693:Foodborne illnesses
3362:CJD Support Network
3176:James Lind Alliance
2756:(9528): 2068–2074.
2271:2021Sci...373..475C
1853:on 2 September 2019
1651:on October 11, 2007
971:St Thomas' Hospital
868:Society and culture
538:Republic of Ireland
264:(for pain relief),
3458:External resources
2446:The New York Times
2338:France 3 Occitanie
2021:"The God of Sperm"
1903:on August 30, 2009
1321:. 10 February 2015
1235:. 25 January 2022.
1209:. 10 February 2015
1128:. 11 February 2015
1089:. 10 February 2015
1034:. 10 February 2015
941:Conservative Party
925:
899:
885:in Rochester, NY.
854:incubation periods
814:
699:
598:Crown Dependencies
502:blood transfusions
417:Signs and symptoms
220:Multiple sclerosis
174:, specifically PrP
106:painful sensations
82:Infectious disease
3680:
3679:
3628:
3627:
3481:
3480:
3385:. 23 January 2016
3383:community website
3335:. 22 October 2000
3107:10.1136/bmj.f5675
2964:. 8 August 2008.
2881:10.4161/pri.29237
2592:10.4161/pri.29237
2552:978-94-007-5415-7
2511:10.1159/000345203
2265:(6554): 475–476.
1681:(7356): 143–147.
1603:(9764): 487–493.
1560:(9433): 527–529.
1340:Ferri FF (2017).
979:Albert Embankment
960:intensive farming
839:incubation period
726:– florid plaques.
680:incubation period
427:poor coordination
353:poor coordination
310:
309:
193:Diagnostic method
114:Poor coordination
35:Medical condition
3715:
3635:in other animals
3532:
3531:
3508:
3501:
3494:
3485:
3484:
3407:
3406:
3395:
3394:
3392:
3390:
3372:
3366:
3365:
3359:
3351:
3345:
3344:
3342:
3340:
3323:
3317:
3316:
3314:
3313:
3295:
3289:
3288:
3286:
3284:
3269:
3263:
3262:
3252:
3229:
3223:
3222:
3220:
3219:
3202:
3196:
3195:
3193:
3191:
3185:
3172:
3164:
3158:
3157:
3155:
3153:
3143:
3135:
3129:
3128:
3118:
3086:
3080:
3079:
3064:
3058:
3057:
3055:
3053:
3036:
3030:
3029:
3027:
3025:
3008:
3002:
3001:
2999:
2998:
2984:
2978:
2977:
2975:
2973:
2952:
2946:
2945:
2909:
2903:
2902:
2892:
2860:
2854:
2853:
2851:
2849:
2834:
2828:
2827:
2808:10.1038/35024175
2791:
2782:
2781:
2745:
2736:
2735:
2733:
2731:
2720:
2714:
2713:
2711:
2709:
2704:. 2 October 2018
2694:
2688:
2687:
2677:
2650:"Prion Diseases"
2645:
2636:
2635:
2633:
2631:
2620:
2614:
2613:
2603:
2571:
2565:
2564:
2530:
2524:
2523:
2513:
2489:
2483:
2482:
2480:
2478:
2463:
2457:
2456:
2454:
2452:
2437:
2431:
2430:
2428:
2427:
2412:
2406:
2405:
2403:
2402:
2380:
2374:
2373:
2371:
2370:
2355:
2349:
2348:
2346:
2345:
2330:
2324:
2323:
2321:
2320:
2305:
2299:
2298:
2254:
2248:
2247:
2245:
2244:
2229:
2223:
2222:
2220:
2219:
2203:
2197:
2196:
2186:
2154:
2148:
2147:
2129:
2105:
2099:
2098:
2096:
2094:
2078:
2072:
2071:
2043:
2037:
2036:
2034:
2032:
2016:
2010:
2009:
2007:
2005:
1985:
1979:
1978:
1976:
1975:
1961:
1955:
1954:
1952:
1951:
1936:
1930:
1929:
1918:
1912:
1911:
1909:
1908:
1893:
1887:
1886:
1884:
1883:
1869:
1863:
1862:
1860:
1858:
1849:. Archived from
1839:
1833:
1832:
1830:
1829:
1824:on 9 August 2016
1814:
1808:
1807:
1805:
1803:
1792:
1786:
1785:
1783:
1781:
1775:
1768:
1760:
1754:
1753:
1746:
1740:
1739:
1737:
1735:
1715:
1709:
1708:
1698:
1666:
1660:
1659:
1657:
1656:
1650:
1643:
1635:
1629:
1628:
1592:
1586:
1585:
1549:
1543:
1542:
1532:
1507:(7): 1009–1010.
1492:
1486:
1485:
1465:
1459:
1458:
1446:
1440:
1439:
1437:
1435:
1416:
1407:
1406:
1396:
1364:
1358:
1357:
1337:
1331:
1330:
1328:
1326:
1311:
1298:
1297:
1294:www.cjd.ed.ac.uk
1291:
1283:
1277:
1276:
1256:
1237:
1236:
1225:
1219:
1218:
1216:
1214:
1199:
1190:
1189:
1171:
1147:
1138:
1137:
1135:
1133:
1118:
1099:
1098:
1096:
1094:
1079:
1044:
1043:
1041:
1039:
1024:
933:inquiry into BSE
845:was informed by
827:Papua New Guinea
370:It is caused by
256:(experimental),
62:
38:
37:
3723:
3722:
3718:
3717:
3716:
3714:
3713:
3712:
3683:
3682:
3681:
3676:
3634:
3624:
3602:
3596:
3570:
3527:
3521:
3512:
3482:
3477:
3476:
3453:
3452:
3418:
3404:
3399:
3398:
3388:
3386:
3374:
3373:
3369:
3357:
3353:
3352:
3348:
3338:
3336:
3325:
3324:
3320:
3311:
3309:
3296:
3292:
3282:
3280:
3271:
3270:
3266:
3231:
3230:
3226:
3217:
3215:
3204:
3203:
3199:
3189:
3187:
3183:
3170:
3166:
3165:
3161:
3151:
3149:
3141:
3137:
3136:
3132:
3087:
3083:
3066:
3065:
3061:
3051:
3049:
3037:
3033:
3023:
3021:
3018:The Independent
3009:
3005:
2996:
2994:
2986:
2985:
2981:
2971:
2969:
2954:
2953:
2949:
2910:
2906:
2861:
2857:
2847:
2845:
2835:
2831:
2802:(6800): 25–26.
2792:
2785:
2746:
2739:
2729:
2727:
2722:
2721:
2717:
2707:
2705:
2696:
2695:
2691:
2646:
2639:
2629:
2627:
2622:
2621:
2617:
2572:
2568:
2553:
2531:
2527:
2490:
2486:
2476:
2474:
2464:
2460:
2450:
2448:
2438:
2434:
2425:
2423:
2414:
2413:
2409:
2400:
2398:
2382:
2381:
2377:
2368:
2366:
2357:
2356:
2352:
2343:
2341:
2332:
2331:
2327:
2318:
2316:
2307:
2306:
2302:
2255:
2251:
2242:
2240:
2231:
2230:
2226:
2217:
2215:
2204:
2200:
2155:
2151:
2106:
2102:
2092:
2090:
2079:
2075:
2044:
2040:
2030:
2028:
2017:
2013:
2003:
2001:
1994:Washington Post
1986:
1982:
1973:
1971:
1963:
1962:
1958:
1949:
1947:
1938:
1937:
1933:
1920:
1919:
1915:
1906:
1904:
1895:
1894:
1890:
1881:
1879:
1871:
1870:
1866:
1856:
1854:
1841:
1840:
1836:
1827:
1825:
1816:
1815:
1811:
1801:
1799:
1794:
1793:
1789:
1779:
1777:
1773:
1766:
1762:
1761:
1757:
1748:
1747:
1743:
1733:
1731:
1730:on July 3, 2013
1724:Daily Telegraph
1716:
1712:
1667:
1663:
1654:
1652:
1648:
1641:
1637:
1636:
1632:
1593:
1589:
1550:
1546:
1493:
1489:
1482:
1466:
1462:
1447:
1443:
1433:
1431:
1418:
1417:
1410:
1365:
1361:
1354:
1338:
1334:
1324:
1322:
1313:
1312:
1301:
1289:
1285:
1284:
1280:
1257:
1240:
1227:
1226:
1222:
1212:
1210:
1201:
1200:
1193:
1148:
1141:
1131:
1129:
1120:
1119:
1102:
1092:
1090:
1081:
1080:
1047:
1037:
1035:
1026:
1025:
1010:
1005:
987:
917:
908:Genetic testing
891:
870:
806:
783:
736:
706:Examination of
704:
688:
654:
638:
622:
606:
495:
452:
447:
439:life expectancy
419:
399:supportive care
365:life expectancy
329:mad cow disease
276:(for agitation)
242:Supportive care
161:life expectancy
142:akinetic mutism
108:
36:
33:
26:
17:
12:
11:
5:
3721:
3711:
3710:
3705:
3700:
3695:
3678:
3677:
3675:
3674:
3669:
3664:
3659:
3654:
3649:
3644:
3638:
3636:
3633:Prion diseases
3630:
3629:
3626:
3625:
3623:
3622:
3617:
3612:
3606:
3604:
3603:transmissible:
3598:
3597:
3595:
3594:
3589:
3584:
3578:
3576:
3572:
3571:
3569:
3568:
3563:
3558:
3553:
3548:
3542:
3540:
3529:
3526:Prion diseases
3523:
3522:
3515:Prion diseases
3511:
3510:
3503:
3496:
3488:
3479:
3478:
3475:
3474:
3462:
3461:
3459:
3455:
3454:
3451:
3450:
3435:
3419:
3414:
3413:
3411:
3410:Classification
3403:
3402:External links
3400:
3397:
3396:
3367:
3346:
3318:
3290:
3264:
3224:
3197:
3159:
3130:
3081:
3074:. 2013-10-15.
3059:
3031:
3003:
2979:
2947:
2904:
2875:(4): 286–295.
2855:
2829:
2783:
2737:
2715:
2689:
2637:
2615:
2586:(4): 286–295.
2566:
2551:
2525:
2504:(1–2): 17–24.
2484:
2458:
2432:
2407:
2375:
2350:
2325:
2300:
2249:
2224:
2198:
2169:(1): 119–122.
2149:
2100:
2073:
2054:(6): 778–790.
2038:
2027:on 6 July 2009
2011:
1980:
1956:
1931:
1913:
1888:
1864:
1834:
1809:
1787:
1755:
1752:. 23 May 2022.
1741:
1710:
1661:
1630:
1587:
1544:
1487:
1480:
1460:
1441:
1426:. March 2003.
1408:
1379:(6): 965–976.
1359:
1352:
1332:
1299:
1278:
1238:
1220:
1191:
1139:
1100:
1045:
1007:
1006:
1004:
1001:
1000:
999:
994:
991:Jonathan Simms
986:
983:
952:John MacGregor
916:
913:
890:
889:United Kingdom
887:
869:
866:
805:
802:
782:
781:Classification
779:
778:
777:
774:
771:
768:
765:
762:
747:
740:
735:
732:
731:
730:
727:
703:
700:
687:
684:
653:
650:
637:
634:
630:Emilie Jaumain
621:
618:
605:
604:Sperm donation
602:
579:Czech Republic
572:United Kingdom
494:
493:Blood products
491:
451:
448:
446:
443:
418:
415:
407:1990s outbreak
403:United Kingdom
384:blood products
308:
307:
304:United Kingdom
300:
296:
295:
292:
288:
287:
284:
278:
277:
251:
245:
244:
239:
235:
234:
231:
227:
226:
217:
211:
210:
195:
189:
188:
182:
176:
175:
169:
165:
164:
157:
153:
152:
149:
145:
144:
135:
129:
128:
122:hallucinations
95:
89:
88:
79:
73:
72:
67:Biopsy of the
64:
63:
55:
54:
47:
43:
42:
34:
15:
9:
6:
4:
3:
2:
3720:
3709:
3708:Rare diseases
3706:
3704:
3701:
3699:
3696:
3694:
3691:
3690:
3688:
3673:
3670:
3668:
3665:
3663:
3660:
3658:
3655:
3653:
3650:
3648:
3645:
3643:
3640:
3639:
3637:
3631:
3621:
3618:
3616:
3613:
3611:
3608:
3607:
3605:
3599:
3593:
3590:
3588:
3585:
3583:
3580:
3579:
3577:
3573:
3567:
3564:
3562:
3559:
3557:
3554:
3552:
3549:
3547:
3544:
3543:
3541:
3538:
3533:
3530:
3524:
3520:
3516:
3509:
3504:
3502:
3497:
3495:
3490:
3489:
3486:
3473:
3469:
3468:
3464:
3463:
3460:
3456:
3449:
3445:
3444:
3440:
3436:
3434:
3430:
3429:
3425:
3421:
3420:
3417:
3412:
3408:
3384:
3382:
3377:
3371:
3363:
3356:
3350:
3334:
3333:
3328:
3322:
3307:
3306:
3305:New Scientist
3301:
3294:
3278:
3274:
3268:
3260:
3256:
3251:
3246:
3242:
3238:
3234:
3228:
3213:
3212:
3207:
3201:
3186:on 2020-03-31
3182:
3178:
3177:
3169:
3163:
3147:
3140:
3134:
3126:
3122:
3117:
3112:
3108:
3104:
3100:
3096:
3092:
3085:
3077:
3073:
3069:
3063:
3048:
3047:
3042:
3035:
3020:
3019:
3014:
3007:
2993:
2989:
2983:
2967:
2963:
2962:
2957:
2951:
2943:
2939:
2935:
2931:
2927:
2923:
2920:(9078): 642.
2919:
2915:
2908:
2900:
2896:
2891:
2886:
2882:
2878:
2874:
2870:
2866:
2859:
2844:
2840:
2833:
2825:
2821:
2817:
2813:
2809:
2805:
2801:
2797:
2790:
2788:
2779:
2775:
2771:
2767:
2763:
2759:
2755:
2751:
2744:
2742:
2725:
2719:
2703:
2699:
2693:
2685:
2681:
2676:
2671:
2667:
2663:
2659:
2655:
2651:
2644:
2642:
2625:
2619:
2611:
2607:
2602:
2597:
2593:
2589:
2585:
2581:
2577:
2576:"Variant CJD"
2570:
2562:
2558:
2554:
2548:
2544:
2540:
2536:
2529:
2521:
2517:
2512:
2507:
2503:
2499:
2495:
2488:
2473:
2469:
2462:
2447:
2443:
2436:
2421:
2417:
2411:
2397:
2393:
2389:
2385:
2379:
2364:
2360:
2354:
2339:
2335:
2329:
2314:
2310:
2304:
2296:
2292:
2288:
2284:
2280:
2276:
2272:
2268:
2264:
2260:
2253:
2238:
2234:
2228:
2213:
2209:
2202:
2194:
2190:
2185:
2180:
2176:
2172:
2168:
2164:
2160:
2153:
2145:
2141:
2137:
2133:
2128:
2123:
2119:
2115:
2111:
2104:
2088:
2084:
2077:
2069:
2065:
2061:
2057:
2053:
2049:
2042:
2026:
2022:
2015:
1999:
1995:
1991:
1984:
1970:
1969:Blood Service
1966:
1960:
1946:on 2011-07-18
1945:
1941:
1935:
1927:
1923:
1917:
1902:
1898:
1892:
1878:
1874:
1868:
1852:
1848:
1844:
1838:
1823:
1819:
1813:
1797:
1791:
1772:
1765:
1759:
1751:
1745:
1729:
1725:
1721:
1714:
1706:
1702:
1697:
1692:
1688:
1684:
1680:
1676:
1672:
1665:
1647:
1640:
1634:
1626:
1622:
1618:
1614:
1610:
1606:
1602:
1598:
1591:
1583:
1579:
1575:
1571:
1567:
1563:
1559:
1555:
1548:
1540:
1536:
1531:
1526:
1522:
1518:
1514:
1510:
1506:
1502:
1498:
1491:
1483:
1481:9781250117779
1477:
1473:
1472:
1464:
1456:
1452:
1445:
1429:
1425:
1421:
1415:
1413:
1404:
1400:
1395:
1390:
1386:
1382:
1378:
1374:
1370:
1363:
1355:
1353:9780323529570
1349:
1345:
1344:
1336:
1320:
1316:
1310:
1308:
1306:
1304:
1295:
1288:
1282:
1274:
1270:
1266:
1262:
1255:
1253:
1251:
1249:
1247:
1245:
1243:
1234:
1230:
1224:
1208:
1204:
1198:
1196:
1187:
1183:
1179:
1175:
1170:
1165:
1161:
1157:
1153:
1146:
1144:
1127:
1123:
1117:
1115:
1113:
1111:
1109:
1107:
1105:
1088:
1084:
1078:
1076:
1074:
1072:
1070:
1068:
1066:
1064:
1062:
1060:
1058:
1056:
1054:
1052:
1050:
1033:
1029:
1023:
1021:
1019:
1017:
1015:
1013:
1008:
998:
995:
992:
989:
988:
982:
980:
976:
972:
967:
963:
961:
957:
953:
949:
945:
942:
938:
937:Lord Phillips
934:
929:
921:
912:
909:
904:
895:
886:
883:
879:
875:
865:
861:
859:
855:
850:
848:
844:
843:Jared Diamond
840:
836:
832:
828:
824:
820:
819:
810:
801:
799:
795:
792:
788:
775:
772:
769:
766:
763:
760:
759:hyperreflexia
756:
752:
748:
745:
741:
738:
737:
728:
725:
721:
717:
713:
712:
711:
709:
696:
692:
683:
681:
676:
672:
668:
664:
660:
649:
647:
643:
633:
631:
627:
617:
615:
611:
601:
599:
595:
590:
588:
584:
580:
575:
573:
569:
564:
562:
558:
553:
549:
545:
543:
539:
535:
531:
526:
523:
518:
516:
512:
511:UK government
507:
503:
498:
490:
488:
484:
480:
476:
470:
468:
463:
461:
457:
442:
440:
436:
432:
428:
424:
414:
412:
408:
404:
400:
395:
393:
389:
385:
381:
377:
373:
368:
366:
362:
358:
354:
350:
346:
342:
341:brain disease
338:
334:
330:
326:
322:
318:
314:
305:
301:
297:
293:
289:
285:
283:
279:
275:
271:
267:
263:
259:
255:
252:
250:
246:
243:
240:
236:
232:
228:
225:
221:
218:
216:
212:
208:
204:
200:
196:
194:
190:
187:
183:
181:
177:
173:
170:
166:
162:
158:
154:
150:
146:
143:
139:
136:
134:
133:Complications
130:
127:
123:
119:
115:
111:
107:
103:
99:
96:
94:
90:
87:
83:
80:
78:
74:
70:
65:
61:
56:
52:
48:
44:
39:
31:
24:
19:
3614:
3465:
3437:
3422:
3387:. Retrieved
3379:
3370:
3361:
3349:
3337:. Retrieved
3332:The Guardian
3330:
3321:
3310:. Retrieved
3303:
3293:
3281:. Retrieved
3279:. 2000-10-01
3276:
3267:
3240:
3236:
3227:
3216:. Retrieved
3211:The Guardian
3209:
3200:
3188:. Retrieved
3181:the original
3174:
3162:
3150:. Retrieved
3148:. 2009-03-01
3145:
3133:
3098:
3094:
3084:
3071:
3062:
3050:. Retrieved
3044:
3034:
3022:. Retrieved
3016:
3006:
2995:. Retrieved
2992:Live Science
2991:
2982:
2970:. Retrieved
2961:The Observer
2959:
2950:
2917:
2913:
2907:
2872:
2868:
2858:
2846:. Retrieved
2832:
2799:
2795:
2753:
2749:
2728:. Retrieved
2718:
2706:. Retrieved
2701:
2692:
2657:
2653:
2628:. Retrieved
2618:
2583:
2579:
2569:
2534:
2528:
2501:
2497:
2487:
2475:. Retrieved
2461:
2449:. Retrieved
2445:
2435:
2424:. Retrieved
2420:ladepeche.fr
2419:
2410:
2399:. Retrieved
2387:
2378:
2367:. Retrieved
2362:
2353:
2342:. Retrieved
2337:
2328:
2317:. Retrieved
2313:ladepeche.fr
2312:
2303:
2262:
2258:
2252:
2241:. Retrieved
2237:ladepeche.fr
2236:
2227:
2216:. Retrieved
2211:
2201:
2166:
2162:
2152:
2120:(1): 83–85.
2117:
2113:
2103:
2091:. Retrieved
2076:
2051:
2047:
2041:
2029:. Retrieved
2025:the original
2014:
2002:. Retrieved
1993:
1983:
1972:. Retrieved
1968:
1959:
1948:. Retrieved
1944:the original
1934:
1925:
1916:
1905:. Retrieved
1901:the original
1891:
1880:. Retrieved
1876:
1867:
1855:. Retrieved
1851:the original
1846:
1837:
1826:. Retrieved
1822:the original
1812:
1800:. Retrieved
1790:
1778:. Retrieved
1758:
1744:
1732:. Retrieved
1728:the original
1723:
1713:
1678:
1674:
1664:
1653:. Retrieved
1646:the original
1633:
1600:
1596:
1590:
1557:
1553:
1547:
1504:
1500:
1490:
1470:
1463:
1444:
1432:. Retrieved
1423:
1376:
1372:
1362:
1342:
1335:
1323:. Retrieved
1318:
1315:"About vCJD"
1293:
1281:
1267:(1): 50–56.
1264:
1260:
1232:
1223:
1211:. Retrieved
1206:
1159:
1155:
1130:. Retrieved
1125:
1091:. Retrieved
1086:
1036:. Retrieved
1031:
968:
964:
956:Douglas Hogg
930:
926:
900:
871:
862:
851:
816:
815:
804:Epidemiology
796:
784:
708:brain tissue
705:
675:heterozygous
655:
639:
636:Other causes
623:
614:Scandinavian
607:
591:
576:
565:
554:
550:
546:
527:
519:
499:
496:
471:
464:
453:
450:Tainted beef
420:
413:, Scotland.
396:
388:brain biopsy
369:
332:
328:
324:
320:
316:
312:
311:
286:Always fatal
199:brain biopsy
180:Risk factors
109:
97:
53:(colloquial)
18:
2843:LiveScience
2730:21 November
2708:21 November
2630:21 November
2472:LiveScience
2422:(in French)
2315:(in French)
2239:(in French)
2214:(in French)
1877:at.blood.ca
1802:28 December
1156:Haemophilia
948:John Gummer
944:Agriculture
935:chaired by
831:cannibalism
744:dysesthesia
610:donor sperm
530:New Zealand
479:South Korea
405:, due to a
343:within the
302:178 in the
274:haloperidol
205:(PMCA), or
148:Usual onset
46:Other names
3687:Categories
3535:inherited/
3381:London SE1
3312:2020-05-27
3218:2020-05-27
2997:2018-10-18
2426:2022-08-29
2401:2022-08-29
2369:2022-08-29
2344:2022-08-29
2319:2022-08-29
2243:2022-08-29
2218:2022-08-29
1974:2023-12-14
1950:2010-03-20
1907:2010-03-03
1882:2023-12-06
1828:2009-06-20
1655:2009-06-20
1325:22 January
1213:23 January
1132:23 January
1093:23 January
1038:22 January
1003:References
946:Ministers
818:The Lancet
720:cerebellum
702:Definitive
663:methionine
306:as of 2024
266:clonazepam
249:Medication
230:Prevention
222:, classic
159:~13-month
3601:acquired/
3575:sporadic:
3528:in humans
3101:: f5675.
2654:Continuum
2396:0242-6056
2295:236515731
2144:220309455
2093:7 January
2085:. Slate.
2004:4 October
1521:1011-8934
997:Mepacrine
903:Wiltshire
751:myoclonus
734:Suspected
686:Diagnosis
652:Mechanism
642:squirrels
411:Edinburgh
291:Frequency
282:Prognosis
270:valproate
262:methadone
238:Treatment
209:(RT-QuIC)
86:Neurology
77:Specialty
3703:Zoonoses
3467:Orphanet
3308:. London
3259:11332300
3125:24129059
3076:Archived
3072:BBC News
2972:27 March
2966:Archived
2942:42158648
2899:25495404
2848:April 9,
2824:36954017
2816:10993054
2778:11506094
2770:16798390
2684:26633779
2610:25495404
2561:23225013
2520:23548713
2477:April 9,
2451:18 April
2388:La Croix
2287:34326214
2212:La Croix
2193:33025140
2136:32609989
2087:Archived
2068:17169195
1998:Archived
1771:Archived
1705:12130612
1625:39891588
1617:21295339
1582:18617259
1574:15302196
1539:27365995
1455:Archived
1428:Archived
1403:32232565
1273:22505363
1186:24635924
1178:20590878
985:See also
878:squirrel
874:Kentucky
835:mourning
724:cerebrum
646:squirrel
506:epidemic
431:dementia
357:dementia
258:morphine
156:Duration
118:dementia
93:Symptoms
3652:Scrapie
3116:3805509
3052:Jun 16,
3024:16 June
2934:9288058
2890:4601215
2675:4879966
2601:4601215
2267:Bibcode
2259:Science
2184:7785535
2031:20 June
1857:20 June
1734:July 2,
1696:1123672
1530:4900988
1434:16 July
1394:7244468
975:Lambeth
847:Papuans
671:alleles
594:Finland
577:In the
561:Ireland
515:albumin
98:Initial
3472:576370
3433:8E01.2
3389:27 May
3339:27 May
3283:27 May
3257:
3247:
3190:27 May
3152:27 May
3123:
3113:
3046:Medium
2940:
2932:
2914:Lancet
2897:
2887:
2822:
2814:
2796:Nature
2776:
2768:
2750:Lancet
2682:
2672:
2608:
2598:
2559:
2549:
2518:
2394:
2293:
2285:
2191:
2181:
2142:
2134:
2066:
1780:31 May
1703:
1693:
1623:
1615:
1597:Lancet
1580:
1572:
1554:Lancet
1537:
1527:
1519:
1478:
1401:
1391:
1350:
1271:
1184:
1176:
798:ICD-10
755:chorea
667:valine
587:France
568:France
557:Poland
542:France
532:, the
487:Canada
483:Mexico
372:prions
331:" or "
299:Deaths
172:Prions
168:Causes
69:tonsil
3448:A81.0
3358:(PDF)
3250:80663
3184:(PDF)
3171:(PDF)
3142:(PDF)
2938:S2CID
2869:Prion
2820:S2CID
2774:S2CID
2580:Prion
2291:S2CID
2140:S2CID
1965:"FAQ"
1774:(PDF)
1767:(PDF)
1649:(PDF)
1642:(PDF)
1621:S2CID
1578:S2CID
1424:NINDS
1290:(PDF)
1182:S2CID
626:INRAE
475:Japan
445:Cause
325:nvCJD
110:Later
3620:Kuru
3615:vCJD
3610:iCJD
3582:sCJD
3546:fCJD
3537:PRNP
3517:and
3391:2020
3341:2020
3285:2020
3255:PMID
3237:CMAJ
3192:2020
3154:2020
3121:PMID
3054:2023
3026:2023
2974:2017
2930:PMID
2895:PMID
2850:2022
2812:PMID
2766:PMID
2732:2018
2710:2018
2680:PMID
2632:2018
2606:PMID
2557:PMID
2547:ISBN
2516:PMID
2479:2022
2453:2019
2392:ISSN
2283:PMID
2189:PMID
2132:PMID
2095:2017
2064:PMID
2033:2009
2006:2008
1859:2019
1804:2023
1782:2017
1736:2013
1701:PMID
1613:PMID
1570:PMID
1535:PMID
1517:ISSN
1476:ISBN
1436:2017
1399:PMID
1348:ISBN
1327:2018
1269:PMID
1215:2018
1174:PMID
1134:2018
1095:2018
1040:2018
954:and
823:kuru
722:and
716:kuru
659:PRNP
540:and
433:and
359:and
317:vCJD
3439:ICD
3424:ICD
3245:PMC
3241:164
3111:PMC
3103:doi
3099:347
3095:BMJ
2922:doi
2918:350
2885:PMC
2877:doi
2804:doi
2800:407
2758:doi
2754:367
2702:CDC
2670:PMC
2662:doi
2596:PMC
2588:doi
2539:doi
2506:doi
2275:doi
2263:373
2179:PMC
2171:doi
2167:141
2122:doi
2118:383
2056:doi
1691:PMC
1683:doi
1679:325
1675:BMJ
1605:doi
1601:377
1562:doi
1558:364
1525:PMC
1509:doi
1389:PMC
1381:doi
1377:139
1319:CDC
1207:CDC
1164:doi
1126:CDC
1087:CDC
1032:CDC
973:in
791:PrP
665:or
628:).
592:In
585:or
566:In
555:In
528:In
337:BSE
51:BSE
3689::
3470::
3446::
3443:10
3431::
3428:11
3378:.
3360:.
3329:.
3302:.
3275:.
3253:.
3239:.
3235:.
3208:.
3173:.
3144:.
3119:.
3109:.
3097:.
3093:.
3070:.
3043:.
3015:.
2990:.
2958:.
2936:.
2928:.
2916:.
2893:.
2883:.
2871:.
2867:.
2841:.
2818:.
2810:.
2798:.
2786:^
2772:.
2764:.
2752:.
2740:^
2700:.
2678:.
2668:.
2658:21
2656:.
2652:.
2640:^
2604:.
2594:.
2582:.
2578:.
2555:.
2545:.
2514:.
2502:16
2500:.
2496:.
2470:.
2444:.
2418:.
2386:.
2361:.
2336:.
2311:.
2289:.
2281:.
2273:.
2261:.
2235:.
2210:.
2187:.
2177:.
2165:.
2161:.
2138:.
2130:.
2116:.
2112:.
2062:.
2052:13
2050:.
1996:.
1992:.
1967:.
1924:.
1875:.
1845:.
1722:.
1699:.
1689:.
1677:.
1673:.
1619:.
1611:.
1599:.
1576:.
1568:.
1556:.
1533:.
1523:.
1515:.
1505:31
1503:.
1499:.
1453:.
1422:.
1411:^
1397:.
1387:.
1375:.
1371:.
1317:.
1302:^
1292:.
1265:50
1263:.
1241:^
1231:.
1205:.
1194:^
1180:.
1172:.
1160:16
1158:.
1154:.
1142:^
1124:.
1103:^
1085:.
1048:^
1030:.
1011:^
981:.
950:,
882:UK
757:,
753:,
746:).
648:.
583:UK
485:,
481:,
477:,
429:,
355:,
268:,
260:,
201:,
140:,
124:,
120:,
116:,
112::
100::
84:,
3539::
3507:e
3500:t
3493:v
3441:-
3426:-
3416:D
3393:.
3343:.
3315:.
3287:.
3261:.
3221:.
3194:.
3156:.
3127:.
3105::
3056:.
3028:.
3000:.
2976:.
2944:.
2924::
2901:.
2879::
2873:8
2852:.
2826:.
2806::
2780:.
2760::
2734:.
2712:.
2686:.
2664::
2634:.
2612:.
2590::
2584:8
2563:.
2541::
2522:.
2508::
2481:.
2455:.
2429:.
2404:.
2372:.
2347:.
2322:.
2297:.
2277::
2269::
2246:.
2221:.
2195:.
2173::
2146:.
2124::
2097:.
2070:.
2058::
2035:.
2008:.
1977:.
1953:.
1928:.
1910:.
1885:.
1861:.
1831:.
1806:.
1784:.
1738:.
1707:.
1685::
1658:.
1627:.
1607::
1584:.
1564::
1541:.
1511::
1484:.
1438:.
1405:.
1383::
1356:.
1329:.
1296:.
1275:.
1217:.
1188:.
1166::
1136:.
1097:.
1042:.
323:(
315:(
32:.
25:.
Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.
