808:
1554:(PPS) may slow the progression of the disease, and may have contributed to the longer than expected survival of the seven people studied. The CJD Therapy Advisory Group to the UK Health Departments advises that data are not sufficient to support claims that pentosan polysulfate is an effective treatment and suggests that further research in animal models is appropriate. A 2007 review of the treatment of 26 people with PPS finds no proof of efficacy because of the lack of accepted objective criteria, but it was unclear to the authors whether that was caused by PPS itself. In 2012 it was claimed that the lack of significant benefits has likely been caused because of the drug being administered very late in the disease in many patients.
891:
60:
1362:
electrodes previously used in the brain of a person with CJD was first reported. Transmission occurred despite the decontamination of the electrodes with ethanol and formaldehyde. Retrospective studies identified four other cases likely of similar cause. The rate of transmission from a single contaminated instrument is unknown, although it is not 100%. In some cases, the exposure occurred weeks after the instruments were used on a person with CJD. In the 1980s it was discovered that
666:. This mass of misfolded proteins disrupts neuronal cell function and causes cell death. Mutations in the gene for the prion protein can cause a misfolding of the dominantly alpha helical regions into beta pleated sheets. This change in conformation disables the ability of the protein to undergo digestion. Once the prion is transmitted, the defective proteins invade the brain and induce other prion protein molecules to misfold in a self-sustaining
936:
685:
1350:, giving it the name Creutzfeldt–Jakob disease. Some of the clinical findings described in their first papers do not match current criteria for Creutzfeldt–Jakob disease, and it has been speculated that at least two of the people in initial studies had a different ailment. An early description of familial CJD stems from the German psychiatrist and neurologist
1414:-like particle in naturally and experimentally infected animals. "The high infectivity of comparable, isolated virus-like particles that show no intrinsic PrP by antibody labeling, combined with their loss of infectivity when nucleic acid–protein complexes are disrupted, make it likely that these 25-nm particles are the causal TSE virions".
875:(NSE) is often elevated in CJD cases; however, its diagnostic utility is seen primarily when combined with a test for the 14-3-3 protein. As of 2010, screening tests to identify infected asymptomatic individuals, such as blood donors, are not yet available, though methods have been proposed and evaluated.
974:, in all six cortical layers in the cerebral cortex or with diffuse involvement of the cerebellar molecular layer. These vacuoles appear glassy or eosinophilic and may coalesce. Neuronal loss and gliosis are also seen. Plaques of amyloid-like material can be seen in the neocortex in some cases of CJD.
794:
2p12-pter. This occurs in only 10–15% of all CJD cases. In sporadic cases, the misfolding of the prion protein is a process that is hypothesized to occur as a result of the effects of aging on cellular machinery, explaining why the disease often appears later in life. An EU study determined that "87%
1620:
conducted from 2018 to 2022. The treatment appeared to be well-tolerated and was able to access the brain, where it might have helped to clear PrP. While the treated patients still showed progressive neurological decline, and while none of them survived longer than expected from the normal course of
1422:
Australia has documented 10 cases of healthcare-acquired CJD (iatrogenic or ICJD). Five of the deaths resulted after the patients, who were in treatment either for infertility or short stature, were treated using contaminated pituitary extract hormone but no new cases have been noted since 1991. The
883:
Imaging of the brain may be performed during medical evaluation, both to rule out other causes and to obtain supportive evidence for diagnosis. Imaging findings are variable in their appearance, and also variable in sensitivity and specificity. While imaging plays a lesser role in diagnosis of CJD,
1301:
Although not fully understood, additional information suggests that CJD rates in
African American and nonwhite groups are lower than in whites. While the mean onset is approximately 67 years of age, cases of sCJD have been reported as young as 17 years and over 80 years of age. Mental capabilities
899:
Focal or diffuse diffusion-restriction involving the cerebral cortex and/or basal ganglia. In about 24% of cases DWI shows only cortical hyperintensity; in 68%, cortical and subcortical abnormalities; and in 5%, only subcortical anomalies. The most iconic and striking cortical abnormality has been
3243:
Bongianni M, Orrù C, Groveman BR, Sacchetto L, Fiorini M, Tonoli G, Triva G, Capaldi S, Testi S, Ferrari S, Cagnin A, Ladogana A, Poleggi A, Colaizzo E, Tiple D, Vaianella L, Castriciano S, Marchioni D, Hughson AG, Imperiale D, Cattaruzza T, Fabrizi GM, Pocchiari M, Monaco S, Caughey B, Zanusso G
1322:
The intensity of CJD surveillance increases the number of reported cases, often in countries where CJD epidemics have occurred in the past and where surveillance resources are greatest. An increase in surveillance and reporting of CJD is most likely in response to BSE and vCJD. Possible factors
819:
Testing for CJD has historically been problematic, due to nonspecific nature of early symptoms and difficulty in safely obtaining brain tissue for confirmation. The diagnosis may initially be suspected in a person with rapidly progressing dementia, particularly when they are also found with the
1856:
Notas K, Papaliagkas V, Spilioti M, Papagiannis I, Nemtsas P, Poulopoulos A, Kouskouras K, Diakogiannis I, Kimiskidis VK. Primary Sjögren's
Syndrome Presenting with Rapidly Progressive Dementia: A Case Report. Curr Alzheimer Res. 2022;19(6):479-484. doi: 10.2174/1567205019666220627094707. PMID
3287:
Sanchez-Juan P, Green A, Ladogana A, Cuadrado-Corrales N, Sáanchez-Valle R, Mitrováa E, Stoeck K, Sklaviadis T, Kulczycki J, Hess K, Bodemer M, Slivarichová D, Saiz A, Calero M, Ingrosso L, Knight R, Janssens AC, van Duijn CM, Zerr I (August 2006). "CSF tests in the differential diagnosis of
1463:
three months before his death, and a surgical probe used in the procedure was subsequently reused in other operations. Public health officials identified thirteen people at three hospitals who may have been exposed to the disease through the contaminated probe, but said the risk of anyone's
1361:
CJD was published in 1974. Animal experiments showed that corneas of infected animals could transmit CJD, and the causative agent spreads along visual pathways. A second case of CJD associated with a corneal transplant was reported without details. In 1977, CJD transmission caused by silver
962:
of brain tissue is the definitive diagnostic test for all other forms of prion disease. Due to its invasiveness, biopsy will not be done if clinical suspicion is sufficiently high or low. A negative biopsy does not rule out CJD, since it may predominate in a specific part of the brain.
2929:
Will RG, Alperovitch A, Poser S, Pocchiari M, Hofman A, Mitrova E, de Silva R, D'Alessandro M, Delasnerie-Laupretre N, Zerr I, van Duijn C (June 1998). "Descriptive epidemiology of
Creutzfeldt–Jakob disease in six European countries, 1993-1995. EU Collaborative Study Group for CJD".
1228:. Psychiatric symptoms like anxiety and depression can be treated with sedatives and antidepressants. Myoclonic jerks can be handled with clonazepam or sodium valproate. Opiates can help in pain. Seizures are very uncommon but can nevertheless be treated with antiepileptic drugs.
781:
also destroys prions in infected organic and inorganic matter, since the process chemically attacks protein at the molecular level, although more effective and practical methods involve destruction by combinations of detergents and enzymes similar to biological washing powders.
1580:
have been investigated as treatments for CJD, but as yet there is no strong evidence that either drug is effective in stopping the disease. Further study has been taken with other medical drugs, but none are effective. However, anticonvulsants and anxiolytic agents, such as
1323:
contributing to an increase of CJD incidence are an aging population, population increase, clinician awareness, and more accurate diagnostic methods. Since CJD symptoms are similar to other neurological conditions, it is also possible that CJD is mistaken for stroke, acute
776:
recommend that instrumentation used in such cases be immediately destroyed after use; short of destruction, it is recommended that heat and chemical decontamination be used in combination to process instruments that come in contact with high-infectivity tissues.
1599:, has been evaluated as a treatment for CJD. The efficacy of quinacrine was assessed in a rigorous clinical trial in the UK and the results were published in Lancet Neurology, and concluded that quinacrine had no measurable effect on the clinical course of CJD.
1459:, former Vice President of Netscape, had been experiencing consistent memory problems which led to his diagnosis. In September 2013, another person in Manchester was posthumously determined to have died of the disease. The person had undergone brain surgery at
1423:
other five deaths occurred due to dura grafting procedures that were performed during brain surgery, in which the covering of the brain is repaired. There have been no other ICJD deaths documented in
Australia due to transmission during healthcare procedures.
651:. Prions are misfolded proteins that occur in the neurons of the central nervous system (CNS). They are thought to affect signaling processes, damaging neurons and resulting in degeneration that causes the spongiform appearance in the affected brain.
4916:
5444:
Mead S, Khalili-Shirazi A, Potter C, Mok T, Nihat A, Hyare H, Canning S, Schmidt C, Campbell T, Darwent L, Muirhead N, Ebsworth N, Hextall P, Wakeling M, Linehan J, Libri V, Williams B, Jaunmuktane Z, Brandner S, Rudge P, Collinge J (2022).
1533:
In 2010, a team from New York described detection of PrP in sheep's blood, even when initially present at only one part in one hundred billion (10) in sheep's brain tissue. The method combines amplification with a novel technology called
3050:
Geschwind MD, Martindale J, Miller D, DeArmond SJ, Uyehara-Lock J, Gaskin D, Kramer JH, Barbaro NM, Miller BL (June 2003). "Challenging the clinical utility of the 14-3-3 protein for the diagnosis of sporadic
Creutzfeldt–Jakob disease".
4886:
2593:
Brown P, Gibbs CJ, Rodgers-Johnson P, Asher DM, Sulima MP, Bacote A, Goldfarb LG, Gajdusek DC (May 1994). "Human spongiform encephalopathy: the
National Institutes of Health series of 300 cases of experimentally transmitted disease".
2139:
Murray ED, Buttner N, Price BH. (2012) Depression and
Psychosis in Neurological Practice. In: Neurology in Clinical Practice, 6th Edition. Bradley WG, Daroff RB, Fenichel GM, Jankovic J (eds.) Butterworth Heinemann. April 12, 2012.
3006:
Satoh J, Kurohara K, Yukitake M, Kuroda Y (1999). "The 14-3-3 protein detectable in the cerebrospinal fluid of patients with prion-unrelated neurological diseases is expressed constitutively in neurons and glial cells in culture".
1236:
Life expectancy is greatly reduced for people with
Creutzfeldt–Jakob disease, with the average being less than 6 months. As of 1981, no one was known to have lived longer than 2.5 years after the onset of CJD symptoms. In 2011,
4852:
900:
called "cortical ribboning" or "cortical ribbon sign" due to hyperintensities resembling ribbons appearing in the cortex on MRI. The involvement of the thalamus can be found in sCJD, is even stronger and constant in vCJD.
3381:
Fragoso DC, Gonçalves Filho AL, Pacheco FT, Barros BR, Aguiar Littig I, Nunes RH, Maia Júnior AC, da Rocha AJ (2017-01-01). "Imaging of
Creutzfeldt–Jakob Disease: Imaging Patterns and Their Differential Diagnosis".
1019:
CJD). Medical procedures that are associated with the spread of this form of CJD include blood transfusion from the infected person, use of human-derived pituitary growth hormones, gonadotropin hormone therapy, and
5555:
5536:
5513:
539:
may help with involuntary movements. CJD affects about one person per million people per year. Onset is typically around 60 years of age. The condition was first described in 1920. It is classified as a type of
2029:
4908:
1369:
A review article published in 1979 indicated that 25 dura mater cases had occurred by that date in
Australia, Canada, Germany, Italy, Japan, New Zealand, Spain, the United Kingdom, and the United States.
1366:, a dura mater transplant product, was shown to transmit CJD from the donor to the recipient. This led to the product being banned in Canada but it was used in other countries such as Japan until 1993.
3194:
Foutz A, Appleby BS, Hamlin C, Liu X, Yang S, Cohen Y, Chen W, Blevins J, Fausett C, Wang H, Gambetti P, Zhang S, Hughson A, Tatsuoka C, Schonberger LB, Cohen ML, Caughey B, Safar JG (January 2017).
754:. While the men of the tribe ate the muscle tissue of the deceased, women and children consumed other parts, such as the brain, and were more likely than men to contract kuru from infected tissue.
616:. The duration of the disease varies greatly, but sporadic (non-inherited) CJD can be fatal within months or even weeks. Most affected people die six months after initial symptoms appear, often of
5188:
Rainov NG, Tsuboi Y, Krolak-Salmon P, Vighetto A, Doh-Ura K (May 2007). "Experimental treatments for human transmissible spongiform encephalopathies: is there a role for pentosan polysulfate?".
4882:
887:
Brain MRI is the most useful imaging modality for changes related to CJD. Of the MRI sequences, diffuse-weighted imaging sequences are most sensitive. Characteristic findings are as follows:
1835:
4860:
2706:
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".
5167:
6327:
4803:
4934:
2886:
Ridley RM, Baker HF, Crow TJ (February 1986). "Transmissible and non-transmissible neurodegenerative disease: similarities in age of onset and genetics in relation to aetiology".
843:– may have characteristic generalized periodic sharp wave pattern. Periodic sharp wave complexes develop in half of the people with sporadic CJD, particularly in the later stages.
504:
manner. Exposure to brain or spinal tissue from an infected person may also result in spread. There is no evidence that sporadic CJD can spread among people via normal contact or
4418:
2161:
Brown P, Cathala F, Castaigne P, Gajdusek DC (November 1986). "Creutzfeldt–Jakob disease: clinical analysis of a consecutive series of 230 neuropathologically verified cases".
6713:
6708:
3455:
Ukisu R, Kushihashi T, Kitanosono T, Fujisawa H, Takenaka H, Ohgiya Y, Gokan T, Munechika H (February 2005). "Serial diffusion-weighted MRI of Creutzfeldt–Jakob disease".
6725:
6703:
2021:
1241:, a Northern Irish man who lived 10 years after his diagnosis, was reported to be one of the world's longest survivors of variant Creutzfeldt–Jakob disease (vCJD).
7275:
5876:
3332:
943:
Testing of tissue remains the most definitive way of confirming the diagnosis of CJD, although it must be recognized that even biopsy is not always conclusive.
635:, many tiny holes can be seen where the nerve cells have died. Parts of the brain may resemble a sponge where the prions were infecting the areas of the brain.
4829:
496:
proteins that can cause normally folded proteins to also become misfolded. About 85% of cases of CJD occur for unknown reasons, while about 7.5% of cases are
1515:, March 21, 2021, from CJD. In April 2024, two hunters from the same lodge were found to be afflicted with Sporadic CJD after eating deer meat infected with
3244:(February 2017). "Diagnosis of Human Prion Disease Using Real-Time Quaking-Induced Conversion Testing of Olfactory Mucosa and Cerebrospinal Fluid Samples".
2655:
Collinge J, Sidle KC, Meads J, Ironside J, Hill AF (October 1996). "Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD".
1625:
who conducted the study see these early-stage results as encouraging and suggest to conduct a larger study, ideally at the earliest possible intervention.
53:
Classic Creutzfeldt–Jakob disease, subacute spongiform encephalopathy, neurocognitive disorder due to prion disease, (historical) spastic pseudosclerosis
8064:
631:, which are associated with the build-up of abnormal prion proteins forming in the brain. When brain tissue from a person with CJD is examined under a
5722:
1277:
The disease is found most frequently in people 55–65 years of age, but cases can occur in people older than 90 years and younger than 55 years of age.
807:
2501:, Rabinowicz T, Gajdusek DC, Gibbs CJ (February 1977). "Danger of accidental person-to-person transmission of Creutzfeldt–Jakob disease by surgery".
6072:
1827:
1141:
3555:"Diffusion-weighted and fluid-attenuated inversion recovery imaging in Creutzfeldt–Jakob disease: high sensitivity and specificity for diagnosis"
3135:
Sano K, Satoh K, Atarashi R, Takashima H, Iwasaki Y, Yoshida M, Sanjo N, Murai H, Mizusawa H, Schmitz M, Zerr I, Kim YS, Nishida N (2013-01-25).
1354:(1880–1953). A study published in 1997 counted more than 100 cases worldwide of transmissible CJD and new cases continued to appear at the time.
6067:
2498:
1605:, a medication approved for human use, has been found to have anti-prion activity and may lead to a treatment for Creutzfeldt–Jakob disease.
1012:
Familial (fCJD), caused by an inherited mutation in the prion-protein gene. This accounts for the majority of the other 15% of cases of CJD.
5869:
5163:
1410:
has challenged the prion protein (PrP) explanation for the disease. In January 2007, she and her colleagues reported that they had found a
5088:
Orrú CD, Bongianni M, Tonoli G, Ferrari S, Hughson AG, Groveman BR, Fiorini M, Pocchiari M, Monaco S, Caughey B, Zanusso G (August 2014).
1541:
In 2014, a human study showed a nasal brushing method that can accurately detect PrP in the olfactory epithelial cells of people with CJD.
4930:
4799:
4657:
4379:
7747:
7703:
6806:
1297:
CJD deaths and age-adjusted death rate in the United States indicate an increasing trend in the number of deaths between 1979 and 2017.
1249:
773:
5447:"Prion protein monoclonal antibody (PRN100) therapy for Creutzfeldt–Jakob disease: Evaluation of a first-in-human treatment programme"
4955:
2800:
Jackson GS, McKintosh E, Flechsig E, Prodromidou K, Hirsch P, Linehan J, Brandner S, Clarke AR, Weissmann C, Collinge J (March 2005).
2212:
6695:
4313:
853:
could be supportive in the diagnosis of sCJD. However, a positive result should not be regarded as sufficient for the diagnosis. The
17:
5142:
576:(jerky movements) typically occurs in 90% of cases, but may be absent at initial onset. Other frequently occurring features include
7562:
7179:
6992:
5677:
5012:
4740:
4780:
3553:
Young GS, Geschwind MD, Fischbein NJ, Martindale JL, Henry RG, Liu S, Lu Y, Wong S, Liu H, Miller BL, Dillon WP (June–July 2005).
3333:"A highly sensitive immunoassay for the detection of prion-infected material in whole human blood without the use of proteinase K"
1435:
transplant. Five New Zealanders have been confirmed to have died of the sporadic form of Creutzfeldt–Jakob disease (CJD) in 2012.
704:
implants and pituitary human growth hormone, which has been replaced by recombinant human growth hormone that poses no such risk.
6972:
6685:
5862:
5763:
5690:
5385:
Karapetyan YE, Sferrazza GF, Zhou M, Ottenberg G, Spicer T, Chase P, Fallahi M, Hodder P, Weissmann C, Lasmézas CI (April 2013).
5233:
4001:
3420:
Finkenstaedt M, Szudra A, Zerr I, Poser S, Hise JH, Stoebner JM, Weber T (June 1996). "MR imaging of Creutzfeldt–Jakob disease".
1635:
1391:
644:
541:
465:. Early symptoms include memory problems, behavioral changes, poor coordination, and visual disturbances. Later symptoms include
4435:
Ironside JW (1996). "Neuropathological diagnosis of human prion disease; morphological studies". In Baker HF, Ridley RM (eds.).
2544:
Brown P, Brandel JP, Sato T, Nakamura Y, MacKenzie J, Will RG, Ladogana A, Pocchiari M, Leschek EW, Schonberger LB (June 2012).
2111:
7298:
6665:
6338:
1252:
monitors the occurrence of CJD in the United States through periodic reviews of national mortality data. According to the CDC:
1009:
Sporadic (sCJD), caused by the spontaneous misfolding of prion-protein in an individual. This accounts for 85% of cases of CJD.
4357:
6690:
6322:
5786:
5737:
4452:
2990:
2862:
2293:
1755:
1395:
1294:
Approximately 85% of CJD cases are sporadic and 10–15% of CJD cases are due to inherited mutations of the prion protein gene.
1032:
509:
305:
33:
4019:
Bonda DJ, Manjila S, Mehndiratta P, Khan F, Miller BR, Onwuzulike K, Puoti G, Cohen ML, Schonberger LB, Cali I (July 2016).
3584:
8069:
6528:
6516:
5978:
5190:
4468:
Meggendorfer F (1930). "Klinische und genealogische Beobachtungen bei einem Fall von spastischer Pseudokosklerose Jakobs".
3670:
854:
4288:
2637:
1309:
Surveillance studies from 2005 and later show the estimated global incidence is 1–2 cases per million population per year.
1270:
In the United States, CJD deaths among people younger than 30 years of age are extremely rare (fewer than five deaths per
970:
appearance is spongiform change in the gray matter: the presence of many round vacuoles from one to 50 micrometers in the
790:
People can also develop CJD because they carry a mutation of the gene that codes for the prion protein (PRNP), located on
7759:
6907:
5570:
1015:
Acquired CJD, caused by contamination with tissue from an infected person, usually as the result of a medical procedure (
7009:
6984:
6819:
6747:
6680:
6235:
2149:
2089:
2059:
1535:
1280:
In more than 85% of cases, the duration of CJD is less than one year (median: four months) after the onset of symptoms.
867:
of the brain – often shows high signal intensity in the caudate nucleus and putamen bilaterally on T2-weighted images.
857:(RT-QuIC) assay has a diagnostic sensitivity of more than 80% and a specificity approaching 100%, tested in detecting
707:
It can be familial (fCJD); or it may appear without clear risk factors (sporadic form: sCJD). In the familial form, a
8079:
7795:
7615:
7513:
6450:
6253:
5833:
195:
147:
4068:
Belay ED, Schonberger LB (December 2002). "Variant Creutzfeldt–Jakob disease and bovine spongiform encephalopathy".
1538:(SOFIA) and some specific antibodies against PrP. The technique allowed improved detection and testing time for PrP.
7887:
7124:
7095:
7036:
5838:
5813:
4825:
4680:"Cells infected with scrapie and Creutzfeldt–Jakob disease agents produce intracellular 25-nm virus-like particles"
3137:"Early detection of abnormal prion protein in genetic human prion diseases now possible using real-time QUIC assay"
1036:
725:
545:
5257:
Pfeifer A, Eigenbrod S, Al-Khadra S, Hofmann A, Mitteregger G, Moser M, Bertsch U, Kretzschmar H (December 2006).
7129:
6924:
6849:
6569:
5818:
958:. Diagnosis of vCJD can be supported by biopsy of the tonsils, which harbor significant amounts of PrP; however,
724:
It is thought that humans can contract the variant form of the disease by eating food from animals infected with
7782:
7567:
7538:
6799:
6084:
6060:
5890:
5843:
5259:"Lentivector-mediated RNAi efficiently suppresses prion protein and prolongs survival of scrapie-infected mice"
4975:"Two Hunters from the Same Lodge Afflicted with sporadic CJD: Is Chronic Wasting Disease to Blame? (P7-13.002)"
2145:
2026:
Joint United Kingdom (UK) Blood Transfusion and Tissue Transplantation Services Professional Advisory Committee
1448:
861:
in CSF samples of people with CJD. It is therefore suggested as a high-value diagnostic method for the disease.
658:
of the cellular prion protein into the diseased state. The number of misfolded protein molecules will increase
4108:"Creutzfeldt–Jakob disease: updated diagnostic criteria, treatment algorithm, and the utility of brain biopsy"
1933:"Creutzfeldt–Jakob disease: updated diagnostic criteria, treatment algorithm, and the utility of brain biopsy"
1373:
By 1985, a series of case reports in the United States showed that when injected, cadaver-extracted pituitary
7026:
7002:
6763:
6648:
6564:
6506:
6055:
5655:
5139:"Intraventricular Pentosan Polysulphate in Human Prion Diseases: A study of Experience in the United Kingdom"
4380:"Creutzfeldt–Jakob disease: a systematic review of global incidence, prevalence, infectivity, and incubation"
1465:
473:. About 70% of people die within a year of diagnosis. The name "Creutzfeldt–Jakob disease" was introduced by
5387:"Unique drug screening approach for prion diseases identifies tacrolimus and astemizole as antiprion agents"
922:
Brain FDG PET-CT tends to be markedly abnormal, and is increasingly used in the investigation of dementias.
7971:
7508:
7041:
6886:
6732:
6106:
5670:
1155:
7216:
7014:
6242:
4756:
4604:
Ae R, Hamaguchi T, Nakamura Y, Yamada M, Tsukamoto T, Mizusawa H, Belay ED, Schonberger LB (March 2018).
4147:
8029:
4223:"Panencephalopathic type of Creutzfeldt–Jakob disease: primary involvement of the cerebral white matter"
1315:
Probable or definite sCJD deaths also increased from the years 1996–2018 in twelve additional countries.
7966:
6881:
6720:
6270:
5942:
738:
has also been implicated as a transmission mechanism for abnormal prions, causing the disease known as
521:
67:
5338:"Safety and efficacy of quinacrine in human prion disease (PRION-1 study): a patient-preference trial"
7976:
7945:
7323:
7206:
6792:
6670:
6638:
6612:
6590:
6439:
6018:
1980:
Brandel J, Vlaciu M, Culeux A, Belondrade M, Grznarova K, Plu I, Levasseur M, Haik S (July 2, 2020).
1622:
1472:
1444:
1318:
CJD incidence is greatest in those over the age of 55 years old, with an average age of 67 years old.
1302:
rapidly deteriorate and the average amount of time from onset of symptoms to death is 7 to 9 months.
769:
462:
8089:
8024:
7940:
7935:
7632:
7595:
7587:
7405:
7374:
7115:
7100:
6737:
6511:
5925:
5732:
5581:
4653:
3641:"Thalamic involvement in sporadic Creutzfeldt–Jakob disease: a diffusion-weighted MR imaging study"
1343:
1260:
778:
721:, in that family. All types of CJD are transmissible irrespective of how they occur in the person.
478:
368:
7961:
7468:
7318:
7313:
7270:
7087:
6742:
6675:
6431:
6249:
6101:
6091:
5935:
5828:
5663:
4359:
Prions and Prion Disease of the Central Nervous System (Transmissible Neurodegenerative Diseases)
1640:
1516:
1487:, died of CJD in Kentucky in June 2015, after having been diagnosed in January. American actress
1460:
990:
884:
characteristic findings on brain MRI in some cases may precede onset of clinical manifestations.
872:
360:
257:
5854:
4739:
Infectious Diseases Epidemiology & Surveillance – Department of Health, Victoria, Australia
1828:"Creutzfeldt–Jakob Disease Fact Sheet | National Institute of Neurological Disorders and Stroke"
993:. These vacuoles appear clear and punched-out. Larger vacuoles encircling neurons, vessels, and
8084:
7839:
7478:
7369:
7328:
7303:
7211:
6607:
6599:
6554:
6230:
6202:
6050:
5336:, Rudge P, Siddique D, Spyer M, Thomas D, Walker S, Webb T, Wroe S, Darbyshire J (April 2009).
4338:"Occurrence and Transmission | Creutzfeldt–Jakob Disease, Classic (CJD) | Prion Disease | CDC".
3953:
3086:
Chitravas N, Jung RS, Kofskey DM, Blevins JE, Gambetti P, Leigh RJ, Cohen ML (September 2011).
2982:
1867:
1351:
1291:
CJD incidence was 3.5 cases per million among those over 50 years of age between 1979 and 2017.
982:
840:
620:
due to impaired coughing reflexes. About 15% of people with CJD survive for two or more years.
565:
364:
347:
5621:
5530:
5522:
5047:
Rubenstein R, Chang B, Gray P, Piltch M, Bulgin MS, Sorensen-Melson S, Miller MW (July 2010).
4337:
3894:
544:. Inherited CJD accounts for about 10% of prion disease cases. Sporadic CJD is different from
8034:
7767:
7647:
7554:
7533:
6622:
6496:
6314:
6292:
6207:
6130:
6040:
6027:
5952:
5526:
4974:
2409:"Risk of Creutzfeldt–Jakob disease transmission by ocular surgery and tissue transplantation"
1499:
1028:
4956:"José Baselga, renowned cancer researcher and AstraZeneca oncology R&D head, dies at 61"
2974:
7859:
7772:
7528:
7348:
6949:
6929:
6559:
6376:
6265:
5398:
4691:
4317:
3148:
2664:
2208:
1551:
1374:
1102:
597:
589:
513:
262:
5138:
4270:
332:
Having at least one living or deceased ancestor with the disease (in case of familial CJD)
8:
7909:
7891:
7854:
7844:
7698:
7600:
7577:
7425:
7417:
7196:
7174:
6902:
6488:
6135:
5990:
5930:
5559:
4106:
Manix M, Kalakoti P, Henry M, Thakur J, Menger R, Guthikonda B, Nanda A (November 2015).
1609:
1468:
1387:
1328:
1271:
846:
762:
692:
The defective protein can be transmitted by contaminated harvested human brain products,
5643:
5402:
5023:
4736:
4695:
3152:
2668:
2315:
Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences
7572:
7169:
7021:
6658:
6643:
6631:
6381:
6332:
6169:
6032:
5920:
5476:
5421:
5386:
5362:
5337:
5283:
5258:
5215:
5114:
5089:
4714:
4679:
4630:
4605:
4581:
4556:
4485:
4410:
4198:
4173:
4045:
4020:
4005:
3836:
3801:
3777:
3744:
3725:
3712:
3686:"18FDG PET-CT in sporadic Creutzfeldt–Jakob disease, correlated with MRI and histology"
3685:
3657:
3571:
3530:
3491:
3363:
3313:
3301:
3269:
3220:
3195:
3171:
3136:
3112:
3087:
3032:
2955:
2911:
2777:
2750:
2731:
2688:
2619:
2570:
2545:
2526:
2384:
2359:
2335:
2310:
2186:
1723:
1698:
1569:. The RNA blocks production of the protein that the CJD process transforms into prions.
1347:
1220:
As of 2024, there is no cure or effective treatment for CJD. Some of the symptoms like
894:
dwMRI, FDG PET and post mortem histology from a patient who presented with sCJD aged 66
796:
659:
505:
501:
482:
474:
356:
225:
5564:
5463:
5353:
4398:
4247:
4222:
4081:
3331:
Tattum MH, Jones S, Pal S, Khalili-Shirazi A, Collinge J, Jackson GS (December 2010).
2719:
2514:
2474:
2449:
1931:
Manix M, Kalakoti P, Henry M, Thakur J, Menger R, Guthikonda B, Nanda A (2015-11-01).
890:
836:, and visual disturbances. Further testing can support the diagnosis and may include:
8074:
7818:
7343:
7333:
7265:
7191:
7075:
6421:
6387:
6359:
6350:
6282:
6258:
6125:
5973:
5592:
5480:
5468:
5426:
5367:
5288:
5207:
5119:
5070:
4994:
4931:"Barbara Tarbuck, 'General Hospital' and 'American Horror Story' Actress, Dies at 74"
4785:
4719:
4635:
4586:
4526:
4489:
4448:
4414:
4402:
4252:
4203:
4129:
4085:
4050:
3978:
3876:
3841:
3823:
3782:
3764:
3729:
3717:
3662:
3621:
3576:
3535:
3517:
3472:
3437:
3399:
3355:
3351:
3305:
3261:
3225:
3176:
3117:
3068:
3024:
2986:
2975:
2947:
2903:
2858:
2823:
2782:
2723:
2680:
2611:
2575:
2518:
2479:
2430:
2389:
2340:
2289:
2264:
2256:
2178:
2141:
2119:
2003:
1962:
1954:
1751:
1728:
1483:
1477:
1431:
A case was reported in 1989 in a 25-year-old man from New Zealand, who also received
735:
581:
337:
213:
6784:
5632:
5219:
3492:"Diffusion-weighted imaging: As the first diagnostic clue to Creutzfeldt Jacob [
3433:
3367:
3317:
3273:
3036:
2959:
2915:
2751:"Kuru: A Journey Back in Time from Papua New Guinea to the Neanderthals' Extinction"
2735:
2623:
2530:
1714:
7688:
7642:
7610:
7458:
7436:
7401:
7164:
7046:
6997:
6944:
6919:
6914:
6871:
6859:
6544:
6415:
6410:
6195:
6190:
5458:
5416:
5406:
5357:
5349:
5278:
5270:
5199:
5109:
5101:
5060:
4986:
4709:
4699:
4625:
4617:
4576:
4568:
4516:
4477:
4440:
4394:
4242:
4234:
4193:
4185:
4119:
4077:
4040:
4032:
4021:"Human prion diseases: surgical lessons learned from iatrogenic prion transmission"
3968:
3925:
3831:
3813:
3772:
3756:
3707:
3697:
3652:
3611:
3566:
3525:
3507:
3464:
3429:
3391:
3347:
3297:
3253:
3215:
3207:
3166:
3156:
3107:
3099:
3060:
3016:
2939:
2895:
2850:
2813:
2772:
2762:
2715:
2692:
2672:
2603:
2565:
2557:
2510:
2469:
2461:
2420:
2379:
2371:
2330:
2322:
2246:
2190:
2170:
1993:
1982:"Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure"
1944:
1718:
1710:
1558:
1493:
1407:
981:
can also be seen in other disease states. Diffuse cortical vacuolization occurs in
912:
758:
747:
536:
168:
162:
89:
7817:
5446:
4606:"Update: Dura Mater Graft-Associated Creutzfeldt–Jakob Disease - Japan, 1975-2017"
3954:"The end of the BSE saga: do we still need surveillance for human prion diseases?"
2802:"An enzyme-detergent method for effective prion decontamination of surgical steel"
2450:"Creutzfeldt–Jakob disease and lyophilised dura mater grafts: report of two cases"
7864:
7822:
7693:
7680:
7627:
7622:
7605:
7485:
7280:
7252:
7053:
6876:
6815:
6466:
6157:
5586:
4990:
4744:
3554:
3257:
3196:"Diagnostic and prognostic value of human prion detection in cerebrospinal fluid"
3161:
2375:
2283:
1488:
1401:
1225:
1137:
1116:
951:
946:
In one-third of people with sporadic CJD, deposits of "prion protein (scrapie)",
908:
751:
655:
517:
493:
387:
83:
7914:
5306:
4292:
3640:
1504:
1305:
According to a 2020 systematic review on the international epidemiology of CJD:
7849:
7790:
7714:
7523:
7490:
7473:
7463:
6523:
6472:
6300:
6009:
5886:
5791:
5758:
5727:
5391:
Proceedings of the National Academy of Sciences of the United States of America
4909:"John S. Carroll, acclaimed newspaper editor in Baltimore and L.A., dies at 73"
4775:
4684:
Proceedings of the National Academy of Sciences of the United States of America
4189:
3468:
1668:
1645:
1617:
1586:
1573:
1404:
1398:
in 1997 "for his discovery of Prions—a new biological principle of infection".
1256:
CJD occurs worldwide at a rate of about 1 case per million population per year.
1238:
850:
739:
663:
609:
577:
437:
5685:
5597:
5549:
5545:
4124:
4107:
4036:
4002:"who.int: "Fact sheets no 180: Variant Creutzfeldt–Jakob disease" Feb 2012 ed"
3616:
3599:
3064:
2899:
2251:
2235:"Creutzfeldt–Jakob disease: report of four cases and review of the literature"
2234:
1949:
1932:
1880:
8058:
7883:
7834:
7152:
6549:
6501:
6371:
6140:
6096:
5947:
5333:
5203:
4998:
4883:"Officials: Lockhart, 'Utah's Iron Lady' died from Creutzfeldt–Jakob disease"
3827:
3818:
3768:
3521:
3512:
2767:
2260:
1958:
1456:
978:
904:
791:
672:
667:
569:
342:
Based on symptoms and medical tests after other possible causes are ruled out
236:: memory problems, behavioral changes, poor coordination, visual disturbances
59:
5411:
4704:
3088:"Treatable neurological disorders misdiagnosed as Creutzfeldt–Jakob disease"
2081:
2051:
1899:
7737:
7518:
7070:
6045:
5915:
5898:
5472:
5430:
5371:
5292:
5211:
5123:
5074:
5065:
5048:
4723:
4639:
4621:
4572:
4530:
4521:
4504:
4406:
4207:
4133:
4089:
4054:
3982:
3973:
3880:
3871:
Liberski, P.P. (2004). "Spongiform change – an electron microscopic view".
3845:
3786:
3745:"Clinical Laboratory Tests Used To Aid in Diagnosis of Human Prion Disease"
3721:
3702:
3666:
3625:
3580:
3539:
3476:
3403:
3359:
3309:
3265:
3229:
3180:
3121:
3072:
3028:
2827:
2786:
2727:
2579:
2561:
2434:
2393:
2344:
2326:
2268:
2007:
1966:
1884:
1732:
1464:
contracting CJD is "extremely low". In January 2015, former speaker of the
1381:
1358:
1312:
Sporadic CJD (sCJD) incidence increased from the years 1990–2018 in the UK.
1093:
1016:
821:
662:
and the process leads to a large quantity of insoluble protein in affected
605:
352:
5164:"Use of Pentosan Polysulphate in the treatment of, or prevention of, vCJD"
5105:
4590:
4256:
4238:
3930:
3913:
3441:
3395:
3286:
2951:
2943:
2907:
2842:
2818:
2801:
2684:
2615:
2607:
2483:
2465:
2182:
2174:
7722:
7453:
7184:
6934:
6004:
5965:
5907:
5616:
3760:
2854:
2425:
2408:
1998:
1981:
1616:
was given to six people with Creutzfeldt–Jakob disease in an early-stage
1577:
1508:
1451:
believed the person acquired the disease from a surgical instrument at a
1384:
administered by injection could also transmit CJD from person to person.
1340:
1324:
743:
420:
Life expectancy greatly shortened, varies from 3 months to multiple years
327:
4678:
Manuelidis L, Yu ZX, Barquero N, Banquero N, Mullins B (February 2007).
4316:. Centers for Disease Control and Prevention. 2007-01-04. Archived from
4291:. Centers for Disease Control and Prevention. 2008-02-26. Archived from
3639:
Tschampa HJ, Mürtz P, Flacke S, Paus S, Schild HH, Urbach H (May 2003).
2522:
8039:
7732:
7637:
7338:
7063:
7058:
6854:
6115:
5985:
5638:
4481:
4444:
1602:
1592:
1452:
1432:
1189:
967:
697:
632:
624:
613:
532:
395:
301:
3211:
3103:
3020:
2052:"About CJD | Creutzfeldt–Jakob Disease, Classic (CJD) | Prion Disease"
935:
7992:
7924:
7742:
7201:
6939:
6864:
6225:
6176:
6164:
6147:
6079:
5627:
5274:
4654:"The Nobel Prize in Physiology or Medicine 1997: Stanley B. Prusiner"
2676:
2022:"Transfusion Handbook/ 5.4: Variant Creutzfeldt–Jakob disease (vCJD)"
1582:
1259:
On the basis of mortality surveillance from 1979 to 1994, the annual
1221:
1185:
1124:
829:
815:, the obvious precipitation of prion protein in the brain is visible.
765:
701:
617:
593:
573:
415:
408:
309:
266:
218:
5505:
4378:
Uttley L, Carroll C, Wong R, Hilton DA, Stevenson M (January 2020).
7727:
7397:
7364:
7290:
6700:
Spinal muscular atrophy with lower extremity predominance (SMALED)
6405:
6397:
6363:
6217:
6120:
3380:
2799:
2448:
Esmonde T, Lueck CJ, Symon L, Duchen LW, Will RG (September 1993).
1512:
1170:
1025:
986:
971:
708:
585:
557:
497:
466:
404:
297:
293:
243:
2496:
903:
Varying degree of symmetric T2 hyperintense signal changes in the
5884:
5823:
5187:
3552:
3454:
3049:
1900:"Creutzfeldt–Jakob Disease, Classic (CJD) | Prion Diseases | CDC"
1596:
1562:
1363:
1264:
1120:
1021:
825:
561:
528:
5575:
5384:
5256:
4554:
4505:"Sporadic and familial CJD: classification and characterisation"
3799:
548:(mad cow disease) and variant Creutzfeldt–Jakob disease (vCJD).
7445:
7260:
6444:
6185:
6152:
5540:
5517:
4973:
Trout J, Roberts M, Tabet M, Kotkowski E, Horn S (2024-04-09).
3490:
Kumaran SP, Gupta K, Pushpa BT, Viswamitra S, Joshy EV (2012).
1443:
In 1988, there was a confirmed death from CJD of a person from
1040:
959:
955:
926:
Patients with CJD will normally have hypometabolism on FDG PET.
833:
693:
628:
601:
3242:
2705:
2592:
1144:. Also posterior thalamic involvement on sagittal T2 sequences
101:
7882:
7223:
5686:
3330:
2928:
2160:
1979:
1411:
947:
858:
648:
612:. In most people with CJD, these symptoms are accompanied by
489:
320:
177:
5443:
5234:"The rise and fall of pentosan polysulfate in prion disease"
5090:"A test for Creutzfeldt–Jakob disease using nasal brushings"
5049:"A novel method for preclinical detection of PrPSc in blood"
4853:"5 patients at Cape hospital at risk for rare brain disease"
4781:"Mike Homer dies at 50; a former vice president of Netscape"
4677:
3914:"Transmissible Spongiform Encephalopathies Affecting Humans"
3489:
2233:
Atalay FO, Tolunay S, Ozgun G, Bekar A, Zarifoglu M (2013).
684:
7396:
5708:
5332:
Collinge J, Gorham M, Hudson F, Kennedy A, Keogh G, Pal S,
5331:
4018:
3683:
3419:
3085:
3005:
2082:"Creutzfeldt–Jakob Disease, Classic (CJD) | Prion Diseases"
1692:
1690:
1613:
1566:
1035:(vCJD) is a type of acquired CJD potentially acquired from
994:
871:
In recent years, studies have shown that the tumour marker
717:
712:
512:. Diagnosis involves ruling out other potential causes. An
470:
247:
183:
113:
5087:
4972:
4555:
Ricketts MN, Cashman NR, Stratton EE, ElSaadany S (1997).
3134:
2654:
2112:"A 49-Year-Old Man With Forgetfulness and Gait Impairment"
488:
CJD is caused by abnormal folding of a protein known as a
136:
5046:
4603:
4362:. www.clinicalkey.com: Elsevier, Inc. pp. 2288–2300.
4221:
Mizutani T, Okumura A, Oda M, Shiraki H (February 1981).
4220:
3912:
G B D, Kumar A, C M M, M M A, H B P, S K M (2012-08-14).
864:
812:
174:
130:
127:
4105:
2546:"Iatrogenic Creutzfeldt–Jakob disease, final assessment"
2543:
1930:
1687:
1519:(CWD), suggesting a potential link between CWD and CJD.
1198:
Marked accumulation of protease-resistant prion protein
1164:
Marked accumulation of protease-resistant prion protein
556:
The first symptom of CJD is usually rapidly progressive
7669:
4377:
3861:
Sternberg's Diagnostic Surgical Pathology, 5th edition.
3800:
Igel-Egalon A, Béringue V, Rezaei H, Sibille P (2018).
3742:
3638:
3600:"An ominous radiographic feature: cortical ribbon sign"
2447:
2232:
742:, once found primarily among women and children of the
670:. These neurodegenerative diseases are commonly called
4470:
Zeitschrift für die Gesamte Neurologie und Psychiatrie
3743:
Connor A, Wang H, Appleby BS, Rhoads DD (2019-09-24).
6814:
4503:
Gambetti P, Kong Q, Zou W, Parchi P, Chen SG (2003).
4502:
3193:
2406:
2308:
1589:, may be administered to relieve associated symptoms.
1471:
died of the disease within a few weeks of diagnosis.
196:
180:
148:
133:
110:
107:
95:
92:
5495:
4557:"Is Creutzfeldt–Jakob disease transmitted in blood?"
3911:
3684:
Morley NC, Hofer M, Wilkinson P, Bradley KM (2021).
907:(i.e., caudate and putamen), and to a lesser extent
186:
139:
119:
116:
104:
98:
5166:. Department of Health:CJD Therapy Advisory Group.
4800:"Autopsy confirms rare brain disease in NH patient"
2407:Armitage WJ, Tullo AB, Ironside JW (October 2009).
2309:Clarke AR, Jackson GS, Collinge J (February 2001).
1263:of CJD remained stable at approximately 1 case per
1092:Prominent psychiatric/behavioral symptoms; painful
985:, and superficial cortical vacuolization occurs in
761:of CJD, may not be inactivated by means of routine
596:. This is accompanied by physical problems such as
171:
124:
6832:
4227:Journal of Neurology, Neurosurgery, and Psychiatry
3802:"Prion Strains and Transmission Barrier Phenomena"
2454:Journal of Neurology, Neurosurgery, and Psychiatry
623:The symptoms of CJD are caused by the progressive
600:impairment, balance and coordination dysfunction (
469:, involuntary movements, blindness, weakness, and
732:. However, it can also cause sCJD in some cases.
8056:
7241:
4174:"Treating seizures in Creutzfeldt–Jakob disease"
4067:
1140:sign-bilateral high signal intensities on axial
834:difficulty with coordination/balance and walking
4101:
4099:
3947:
3945:
3943:
3941:
2885:
654:The CJD prion is dangerous because it promotes
8014:
4826:"NH Patient Likely Died of Rare Brain Disease"
4789:, February 5, 2009. Accessed February 6, 2009.
4271:"Belfast man with VCJD dies after long battle"
4171:
3870:
3597:
2840:
1039:or caused by consuming food contaminated with
246:, involuntary movements, blindness, weakness,
6800:
5870:
5671:
3857:
3855:
2211:. The Merck Manuals: Online Medical Library.
1663:
1661:
1192:analysis of protease-resistant prion protein
4737:Creutzfeldt–Jakob Disease (CJD) – the facts.
4467:
4096:
3938:
2311:"The molecular biology of prion propagation"
1503:) died of the disease on December 26, 2016.
1203:Presence of amyloid plaques in brain tissue
728:(BSE), the bovine form of TSE also known as
459:neurocognitive disorder due to prion disease
6589:
4610:MMWR. Morbidity and Mortality Weekly Report
4550:
4548:
4546:
4544:
4542:
4540:
4355:
2202:
2200:
2116:reference.medscape.com/viewarticle/881806_3
1224:can be managed, but otherwise treatment is
378:Gene editing of children at risk (for fCJD)
7704:Other specified feeding or eating disorder
6971:
6807:
6793:
5877:
5863:
5678:
5664:
4314:"vCJD (Variant Creutzfeldt–Jakob Disease)"
4289:"CJD (Creutzfeldt–Jakob Disease, Classic)"
4172:Ng MC, Westover MB, Cole AJ (2014-02-07).
3852:
3500:Journal of Neurosciences in Rural Practice
1822:
1820:
1818:
1816:
1814:
1812:
1810:
1808:
1806:
1804:
1802:
1800:
1798:
1796:
1794:
1792:
1790:
1788:
1786:
1669:"Creutzfeldt–Jakob Disease, Classic (CJD)"
1658:
1339:The disease was first described by German
1288:Risk of developing CJD increases with age.
774:Centers for Disease Control and Prevention
58:
8065:Transmissible spongiform encephalopathies
5462:
5420:
5410:
5361:
5282:
5113:
5064:
4713:
4703:
4629:
4580:
4520:
4246:
4197:
4123:
4044:
3996:
3994:
3992:
3972:
3951:
3929:
3835:
3817:
3776:
3711:
3701:
3656:
3615:
3570:
3529:
3511:
3219:
3170:
3160:
3111:
2817:
2776:
2766:
2569:
2497:Bernoulli C, Siegfried J, Baumgartner G,
2473:
2424:
2383:
2357:
2334:
2250:
1997:
1948:
1926:
1924:
1894:
1892:
1784:
1782:
1780:
1778:
1776:
1774:
1772:
1770:
1768:
1766:
1722:
1696:
7967:Disorganized (hebephrenic) schizophrenia
7563:Depersonalization-derealization disorder
4919:from the original on September 19, 2016.
4537:
4434:
3895:"Pathology of Degenerative CNS Diseases"
3645:AJNR. American Journal of Neuroradiology
3559:AJNR. American Journal of Neuroradiology
2748:
2638:"New form of BSE resembles sporadic CJD"
2197:
1507:, clinical oncologist having headed the
1048:Clinical and pathologic characteristics
934:
889:
806:
683:
527:There is no specific treatment for CJD.
5764:Variably protease-sensitive prionopathy
5723:Gerstmann–Sträussler–Scheinker syndrome
5691:transmissible spongiform encephalopathy
4906:
1636:Transmissible spongiform encephalopathy
1392:University of California, San Francisco
795:of cases were sporadic, 8% genetic, 5%
645:transmissible spongiform encephalopathy
542:transmissible spongiform encephalopathy
477:in 1922, after the German neurologists
14:
8057:
6666:Distal hereditary motor neuronopathies
6339:Template:Demyelinating diseases of CNS
4806:from the original on 21 September 2013
4373:
4371:
4369:
4351:
4349:
3989:
3457:AJR. American Journal of Roentgenology
2972:
2281:
1921:
1889:
1763:
1380:In 1992, it was recognized that human
849:(CSF) analysis for elevated levels of
8013:
7881:
7816:
7668:
7395:
7240:
7150:
6970:
6831:
6788:
5858:
5659:
5263:The Journal of Clinical Investigation
4850:
4832:from the original on 8 September 2013
3952:Budka H, Will RG (12 November 2015).
3673:from the original on 10 October 2008.
3415:
3413:
2881:
2879:
1745:
1396:Nobel Prize in Physiology or Medicine
688:MRI of iCJD because of growth hormone
551:
531:may be used to help with pain, while
7151:
6517:Idiopathic intracranial hypertension
5906:
5191:Expert Opinion on Biological Therapy
5145:from the original on 3 November 2012
5136:
4178:Epilepsy & Behavior Case Reports
2841:Wallesch CW, Förstl H, eds. (2012),
2206:
1916:Classic CJD is a human prion disease
1595:, a medicine originally created for
997:are a possible processing artifact.
855:Real-Time Quaking-Induced Conversion
7670:Physiological and physical behavior
5094:The New England Journal of Medicine
4366:
4346:
4343:. 2019-05-08. Retrieved 2020-11-04.
3598:Abdulmassih R, Min Z (March 2016).
24:
7616:Psychogenic non-epileptic seizures
7010:Emotional and behavioral disorders
6748:Infantile progressive bulbar palsy
5307:"Revamp of brain 'could slow CJD'"
5022:: 195. August 2010. Archived from
3410:
3302:10.1212/01.wnl.0000230159.67128.00
2876:
2849:, Stuttgart: Georg Thieme Verlag,
2062:from the original on 8 August 2017
1536:surround optical fiber immunoassay
1284:Further information from the CDC:
455:subacute spongiform encephalopathy
284:70% die within a year of diagnosis
27:Degenerative neurological disorder
25:
8101:
7796:Hypoactive sexual desire disorder
6451:Template:Cerebrovascular diseases
6254:Frontotemporal lobar degeneration
5834:Transmissible mink encephalopathy
5491:
2209:"Creutzfeldt–Jakob Disease (CJD)"
2092:from the original on 18 July 2017
1612:(code name PRN100) targeting the
1346:in 1920 and shortly afterward by
1089:Dementia; early neurologic signs
1033:Variant Creutzfeldt–Jakob disease
1000:
930:
510:variant Creutzfeldt–Jakob disease
400:Various experimental treatments,
34:Variant Creutzfeldt–Jakob disease
7096:X-linked intellectual disability
6471:For more detailed coverage, see
6449:For more detailed coverage, see
6420:For more detailed coverage, see
6386:For more detailed coverage, see
6337:For more detailed coverage, see
5839:Feline spongiform encephalopathy
5814:Bovine spongiform encephalopathy
5437:
5378:
4757:"Mad cow link in hunter's death"
4424:from the original on 2022-10-09.
3749:Journal of Clinical Microbiology
3587:from the original on 2008-09-07.
3352:10.1111/j.1537-2995.2010.02731.x
2981:. Chicago: Pathoma LLC. p.
2843:"3.3 Demenz mit Lewy-Körperchen"
1838:from the original on 4 July 2017
1748:Longman Pronunciation Dictionary
1438:
1037:bovine spongiform encephalopathy
726:bovine spongiform encephalopathy
546:bovine spongiform encephalopathy
390:as this condition is untreatable
167:
88:
6925:Intermittent explosive disorder
6850:Ego-dystonic sexual orientation
5819:Camel spongiform encephalopathy
5325:
5299:
5250:
5226:
5181:
5170:from the original on 2007-07-05
5156:
5130:
5081:
5053:The Journal of General Virology
5040:
5005:
4966:
4948:
4937:from the original on 2016-12-30
4923:
4900:
4889:from the original on 2015-01-20
4875:
4844:
4818:
4792:
4767:
4749:
4730:
4671:
4660:from the original on 2011-02-20
4646:
4597:
4496:
4461:
4439:. Vol. 3. pp. 35–57.
4428:
4387:The Lancet. Infectious Diseases
4331:
4306:
4281:
4263:
4214:
4165:
4140:
4061:
4012:
3905:
3887:
3864:
3793:
3736:
3677:
3632:
3604:Internal and Emergency Medicine
3591:
3546:
3483:
3448:
3434:10.1148/radiology.199.3.8638007
3374:
3324:
3280:
3236:
3187:
3128:
3079:
3043:
2999:
2966:
2922:
2834:
2806:The Journal of General Virology
2793:
2742:
2699:
2648:
2630:
2586:
2537:
2490:
2441:
2400:
2351:
2302:
2275:
2226:
2215:from the original on 2011-06-04
2154:
2133:
2104:
2074:
2044:
2032:from the original on 2017-03-05
2014:
1986:New England Journal of Medicine
1973:
1715:10.1136/practneurol-2016-001571
1621:the disease, the scientists at
1244:
679:
508:, although this is possible in
265:due to difficulty coughing and
7568:Dissociative identity disorder
6833:Adult personality and behavior
5844:Exotic ungulate encephalopathy
4070:Clinics in Laboratory Medicine
1874:
1860:
1850:
1739:
1697:Mead S, Rudge P (April 2017).
1545:
1527:
1449:Massachusetts General Hospital
1426:
1377:could transmit CJD to humans.
1357:The first report of suspected
13:
1:
6764:Amyotrophic lateral sclerosis
6649:Hereditary spastic paraplegia
6507:Normal pressure hydrocephalus
5464:10.1016/S1474-4422(22)00082-5
5354:10.1016/S1474-4422(09)70049-3
4399:10.1016/S1473-3099(19)30615-2
4082:10.1016/S0272-2712(02)00024-0
2720:10.1016/s0140-6736(06)68930-7
2515:10.1016/s0140-6736(77)91958-4
1651:
1466:Utah House of Representatives
1115:Signal hyperintensity in the
7972:Pseudoneurotic schizophrenia
7509:Generalized anxiety disorder
7242:Neurological and symptomatic
6887:Sexual relationship disorder
6733:Progressive muscular atrophy
5141:. Medical Research Council.
4991:10.1212/WNL.0000000000204407
4561:Emerging Infectious Diseases
3288:Creutzfeldt–Jakob disease".
3258:10.1001/jamaneurol.2016.4614
3162:10.1371/journal.pone.0054915
2550:Emerging Infectious Diseases
2376:10.1212/CON.0000000000000251
2288:. Pathoma.com. p. 189.
2239:Turkish Journal of Pathology
1417:
1267:people in the United States.
1231:
1215:
1156:Immunohistochemical analysis
1150:Present in >75% of cases
802:
750:, who previously engaged in
7:
8070:Neurodegenerative disorders
7748:REM sleep behavior disorder
7217:Seasonal affective disorder
7015:Separation anxiety disorder
6243:Primary progressive aphasia
5738:Huntington's disease-like 1
5013:"Detecting Prions in Blood"
4962:. Stat News. 21 March 2021.
4907:Schudel M (June 14, 2015).
4802:. MyFoxBoston. 2013-09-20.
1868:"Creutzfeldt–Jakob disease"
1699:"CJD mimics and chameleons"
1629:
1561:to slow the progression of
1522:
1511:Oncology division, died in
1096:; delayed neurologic signs
1086:Clinical signs and symptoms
799:and less than 1% variant."
785:
647:(TSE), which are caused by
524:may support the diagnosis.
10:
8106:
8015:Symptoms and uncategorized
7116:developmental disabilities
6882:Sexual maturation disorder
6565:Hashimoto's encephalopathy
6271:Posterior cortical atrophy
6092:Striatonigral degeneration
5943:Cavernous sinus thrombosis
4356:Bosque P, Tyler K (2020).
4190:10.1016/j.ebcr.2014.01.004
3469:10.2214/ajr.184.2.01840560
1334:
1123:on diffusion-weighted and
1075:Median duration of illness
878:
830:involuntary muscle jerking
522:magnetic resonance imaging
31:
8020:
8009:
7985:
7977:Simple-type schizophrenia
7954:
7946:Schizophreniform disorder
7923:
7902:
7898:
7877:
7830:
7812:
7781:
7758:
7712:
7679:
7675:
7664:
7586:
7553:
7501:
7444:
7435:
7416:
7412:
7391:
7357:
7324:Mild cognitive impairment
7309:Creutzfeldt–Jakob disease
7289:
7251:
7247:
7236:
7207:Major depressive disorder
7160:
7146:
7112:Psychological development
7111:
7086:
6983:
6979:
6966:
6895:
6842:
6838:
6827:
6671:Spinal muscular atrophies
6639:Primary lateral sclerosis
6621:
6598:
6582:
6537:
6497:Intracranial hypertension
6487:
6459:
6430:
6396:
6358:
6347:
6313:
6291:
6277:Creutzfeldt–Jakob disease
6216:
6026:
6017:
6003:
5963:
5897:
5804:
5772:
5746:
5706:
5697:
5644:Creutzfeldt–Jakob disease
5607:
5499:
4125:10.3171/2015.8.FOCUS15328
4037:10.3171/2016.5.FOCUS15126
3617:10.1007/s11739-015-1287-4
3065:10.1001/archneur.60.6.813
2977:Fundamentals of Pathology
2900:10.1017/s0033291700002634
2285:Fundamentals of Pathology
2252:10.5146/tjpath.2013.01195
1950:10.3171/2015.8.FOCUS15328
1881:Creutzfeldt–Jakob disease
1750:(3rd ed.), Longman,
1623:University College London
1445:Manchester, New Hampshire
770:World Health Organization
463:neurodegenerative disease
447:Creutzfeldt–Jakob disease
432:
424:
414:
394:
382:
374:
346:
336:
326:
316:
288:
280:
272:
256:
224:
212:
74:
66:
57:
49:
45:Creutzfeldt–Jakob disease
44:
18:Creutzfeldt-Jakob disease
8080:Rare infectious diseases
8025:Impulse-control disorder
7941:Schizoaffective disorder
7936:Brief reactive psychosis
7633:Mass psychogenic illness
7596:Body dysmorphic disorder
7375:Post-concussion syndrome
6985:Emotional and behavioral
6738:Progressive bulbar palsy
6529:Intracranial hypotension
6512:Choroid plexus papilloma
5926:Herpesviral encephalitis
5733:PrP systemic amyloidosis
5204:10.1517/14712598.7.5.713
4509:British Medical Bulletin
3918:ISRN Infectious Diseases
3819:10.3390/pathogens7010005
3513:10.4103/0976-3147.102645
3342:(Submitted manuscript).
2768:10.3390/pathogens2030472
1344:Hans Gerhard Creutzfeldt
1327:, general dementia, and
1101:Periodic sharp waves on
977:However, extra-neuronal
939:Spongiform change in CJD
779:Thermal depolymerization
638:
492:. Infectious prions are
479:Hans Gerhard Creutzfeldt
68:Magnetic resonance image
32:Not to be confused with
7962:Childhood schizophrenia
7314:Frontotemporal dementia
7271:High-functioning autism
7088:Intellectual disability
6250:Frontotemporal dementia
5936:Encephalitis lethargica
5829:Chronic wasting disease
5759:Sporadic fatal insomnia
5728:Fatal familial insomnia
5412:10.1073/pnas.1303510110
5137:Bone I (12 July 2006).
4705:10.1073/pnas.0610999104
1641:Chronic wasting disease
1517:chronic wasting disease
1461:Catholic Medical Center
1394:(UCSF) was awarded the
1161:Variable accumulation.
991:frontotemporal dementia
873:neuron-specific enolase
820:characteristic medical
7479:Specific social phobia
7370:Organic brain syndrome
7212:Melancholic depression
6973:Childhood and learning
6555:Hepatic encephalopathy
5313:. BBC News. 2006-12-04
5066:10.1099/vir.0.020164-0
4622:10.15585/mmwr.mm6709a3
4573:10.3201/eid0302.970208
3974:10.4414/smw.2015.14212
3875:. 42, suppl B: 59–70.
3873:Folia Neuropathologica
3703:10.4103/wjnm.wjnm_5_21
2888:Psychological Medicine
2562:10.3201/eid1806.120116
2327:10.1098/rstb.2000.0764
1352:Friedrich Meggendorfer
1005:Types of CJD include:
950:, can be found in the
940:
895:
841:Electroencephalography
816:
689:
428:1 per million per year
348:Differential diagnosis
8035:Psychomotor agitation
7825:and substance-related
7768:Postpartum depression
7648:Somatization disorder
7534:Acute stress reaction
7299:AIDS dementia complex
6613:Ataxia–telangiectasia
6570:Static encephalopathy
6293:Mitochondrial disease
6131:Spasmodic torticollis
6041:Basal ganglia disease
5342:The Lancet. Neurology
5106:10.1056/NEJMoa1315200
4239:10.1136/jnnp.44.2.103
3396:10.1148/rg.2017160075
3053:Archives of Neurology
2944:10.1002/ana.410430611
2819:10.1099/vir.0.80484-0
2608:10.1002/ana.410350504
2466:10.1136/jnnp.56.9.999
2358:Geschwind MD (2015).
2175:10.1002/ana.410200507
1500:American Horror Story
1169:Presence of agent in
938:
893:
811:Through the image of
810:
687:
614:involuntary movements
8030:Klüver–Bucy syndrome
7860:Substance dependence
7773:Postpartum psychosis
7319:Huntington's disease
7101:Lujan–Fryns syndrome
6950:Personality disorder
6560:Toxic encephalopathy
6266:Lewy bodies dementia
5451:The Lancet Neurology
4522:10.1093/bmb/66.1.213
4008:on February 4, 2016.
3961:Swiss Medical Weekly
3899:library.med.utah.edu
3761:10.1128/JCM.00769-19
2855:10.1055/b-0034-22435
2749:Liberski PP (2013).
2426:10.1038/eye.2008.381
2058:. 11 February 2015.
1999:10.1056/NEJMc2000687
1565:has been studied in
1552:Pentosan polysulfate
1455:'s office. In 2007,
1375:human growth hormone
1176:Not readily detected
1103:electroencephalogram
752:funerary cannibalism
711:has occurred in the
590:obsessive-compulsive
514:electroencephalogram
361:Huntington's disease
263:Aspiration pneumonia
7910:Delusional disorder
7855:Stimulant psychosis
7845:Physical dependence
7699:Rumination syndrome
7601:Conversion disorder
7578:Psychogenic amnesia
7428:with depressed mood
7426:Adjustment disorder
7329:Parkinson's disease
7304:Alzheimer's disease
7197:Atypical depression
7033:Social functioning
6908:Munchausen syndrome
6903:Factitious disorder
6608:Friedreich's ataxia
5991:Meningoencephalitis
5931:Limbic encephalitis
5403:2013PNAS..110.7044K
4985:(17_supplement_1).
4913:The Washington Post
4863:on 9 September 2013
4763:. 16 November 2012.
4696:2007PNAS..104.1965M
4112:Neurosurgical Focus
4076:(4): 849–62, v–vi.
4025:Neurosurgical Focus
3931:10.5402/2013/387925
3200:Annals of Neurology
3153:2013PLoSO...854915S
3092:Annals of Neurology
2932:Annals of Neurology
2669:1996Natur.383..685C
2644:. 19 February 2004.
2596:Annals of Neurology
2163:Annals of Neurology
2088:. 6 February 2015.
2028:. 4 February 2014.
1937:Neurosurgical Focus
1703:Practical Neurology
1614:prion protein (PrP)
1610:monoclonal antibody
1475:, former editor of
1469:Rebecca D. Lockhart
1388:Stanley B. Prusiner
1329:hyperparathyroidism
1064:Median age at death
1049:
983:Alzheimer's disease
847:Cerebrospinal fluid
763:surgical instrument
365:Alzheimer's disease
7929:schizophrenia-like
7573:Dissociative fugue
7022:Movement disorders
6644:Pseudobulbar palsy
6382:Status epilepticus
6333:Multiple sclerosis
6170:Myoclonic epilepsy
6033:movement disorders
5979:Acute disseminated
5921:Viral encephalitis
5608:External resources
5020:Microbiology Today
4743:2015-06-28 at the
4656:. NobelPrize.org.
4482:10.1007/bf02864269
4445:10.1385/0896033422
3009:European Neurology
2973:Sattar HA (2011).
2282:Sattar HA (2017).
1870:. 23 October 2017.
1348:Alfons Maria Jakob
1047:
941:
918:Cerebellar atrophy
896:
817:
690:
552:Signs and symptoms
506:blood transfusions
502:autosomal dominant
483:Alfons Maria Jakob
475:Walther Spielmeyer
369:Sjögren's syndrome
357:chronic meningitis
8052:
8051:
8048:
8047:
8005:
8004:
8001:
8000:
7873:
7872:
7808:
7807:
7804:
7803:
7660:
7659:
7656:
7655:
7549:
7548:
7387:
7386:
7383:
7382:
7344:Vascular dementia
7266:Asperger syndrome
7232:
7231:
7142:
7141:
7138:
7137:
7076:Tourette syndrome
6962:
6961:
6958:
6957:
6782:
6781:
6778:
6777:
6774:
6773:
6578:
6577:
6483:
6482:
6422:Template:Headache
6388:Template:Epilepsy
6309:
6308:
6283:Vascular dementia
6126:Status dystonicus
5999:
5998:
5974:Encephalomyelitis
5852:
5851:
5800:
5799:
5653:
5652:
5059:(Pt 7): 1883–92.
4786:Los Angeles Times
4454:978-0-89603-342-9
3755:(10): e00769–19.
3212:10.1002/ana.24833
3104:10.1002/ana.22454
3021:10.1159/000008054
2992:978-0-9832246-0-0
2864:978-3-13-136912-3
2714:(9528): 2068–74.
2295:978-0-9832246-3-1
1906:. 1 February 2019
1757:978-1-4058-8118-0
1746:Wells JC (2008),
1484:Los Angeles Times
1478:The Baltimore Sun
1213:
1212:
1179:Readily detected
643:CJD is a type of
453:), also known as
444:
443:
402:For pain relief:
338:Diagnostic method
39:Medical condition
16:(Redirected from
8097:
8011:
8010:
7900:
7899:
7879:
7878:
7814:
7813:
7689:Anorexia nervosa
7677:
7676:
7666:
7665:
7643:Psychogenic pain
7611:Globus pharyngis
7459:Childhood phobia
7442:
7441:
7414:
7413:
7393:
7392:
7249:
7248:
7238:
7237:
7148:
7147:
7047:Selective mutism
6998:Conduct disorder
6981:
6980:
6968:
6967:
6945:Trichotillomania
6920:Gender dysphoria
6915:Fear of intimacy
6872:Sexual anhedonia
6840:
6839:
6829:
6828:
6816:Mental disorders
6809:
6802:
6795:
6786:
6785:
6596:
6595:
6587:
6586:
6545:Brain herniation
6356:
6355:
6196:Intention tremor
6191:Essential tremor
6056:Postencephalitic
6024:
6023:
6015:
6014:
5904:
5903:
5885:Diseases of the
5879:
5872:
5865:
5856:
5855:
5807:in other animals
5704:
5703:
5680:
5673:
5666:
5657:
5656:
5497:
5496:
5485:
5484:
5466:
5441:
5435:
5434:
5424:
5414:
5382:
5376:
5375:
5365:
5329:
5323:
5322:
5320:
5318:
5303:
5297:
5296:
5286:
5275:10.1172/JCI29236
5254:
5248:
5247:
5245:
5244:
5230:
5224:
5223:
5185:
5179:
5178:
5176:
5175:
5160:
5154:
5153:
5151:
5150:
5134:
5128:
5127:
5117:
5085:
5079:
5078:
5068:
5044:
5038:
5037:
5035:
5034:
5029:on 31 March 2012
5028:
5017:
5009:
5003:
5002:
4970:
4964:
4963:
4952:
4946:
4945:
4943:
4942:
4927:
4921:
4920:
4904:
4898:
4897:
4895:
4894:
4879:
4873:
4872:
4870:
4868:
4859:. Archived from
4848:
4842:
4841:
4839:
4837:
4822:
4816:
4815:
4813:
4811:
4796:
4790:
4771:
4765:
4764:
4753:
4747:
4734:
4728:
4727:
4717:
4707:
4675:
4669:
4668:
4666:
4665:
4650:
4644:
4643:
4633:
4601:
4595:
4594:
4584:
4552:
4535:
4534:
4524:
4500:
4494:
4493:
4465:
4459:
4458:
4432:
4426:
4425:
4423:
4384:
4375:
4364:
4363:
4353:
4344:
4335:
4329:
4328:
4326:
4325:
4310:
4304:
4303:
4301:
4300:
4285:
4279:
4278:
4267:
4261:
4260:
4250:
4218:
4212:
4211:
4201:
4169:
4163:
4162:
4160:
4159:
4144:
4138:
4137:
4127:
4103:
4094:
4093:
4065:
4059:
4058:
4048:
4016:
4010:
4009:
4004:. Archived from
3998:
3987:
3986:
3976:
3958:
3949:
3936:
3935:
3933:
3909:
3903:
3902:
3891:
3885:
3884:
3868:
3862:
3859:
3850:
3849:
3839:
3821:
3797:
3791:
3790:
3780:
3740:
3734:
3733:
3715:
3705:
3690:World J Nucl Med
3681:
3675:
3674:
3660:
3636:
3630:
3629:
3619:
3595:
3589:
3588:
3574:
3550:
3544:
3543:
3533:
3515:
3487:
3481:
3480:
3452:
3446:
3445:
3417:
3408:
3407:
3378:
3372:
3371:
3337:
3328:
3322:
3321:
3284:
3278:
3277:
3240:
3234:
3233:
3223:
3191:
3185:
3184:
3174:
3164:
3132:
3126:
3125:
3115:
3083:
3077:
3076:
3047:
3041:
3040:
3003:
2997:
2996:
2980:
2970:
2964:
2963:
2926:
2920:
2919:
2883:
2874:
2873:
2872:
2871:
2838:
2832:
2831:
2821:
2812:(Pt 3): 869–78.
2797:
2791:
2790:
2780:
2770:
2746:
2740:
2739:
2703:
2697:
2696:
2677:10.1038/383685a0
2663:(6602): 685–90.
2652:
2646:
2645:
2634:
2628:
2627:
2590:
2584:
2583:
2573:
2541:
2535:
2534:
2494:
2488:
2487:
2477:
2445:
2439:
2438:
2428:
2404:
2398:
2397:
2387:
2370:(6): 1612–1638.
2360:"Prion Diseases"
2355:
2349:
2348:
2338:
2321:(1406): 185–95.
2306:
2300:
2299:
2279:
2273:
2272:
2254:
2230:
2224:
2223:
2221:
2220:
2204:
2195:
2194:
2158:
2152:
2137:
2131:
2130:
2128:
2127:
2118:. Archived from
2108:
2102:
2101:
2099:
2097:
2078:
2072:
2071:
2069:
2067:
2048:
2042:
2041:
2039:
2037:
2018:
2012:
2011:
2001:
1977:
1971:
1970:
1952:
1928:
1919:
1918:
1913:
1911:
1896:
1887:
1878:
1872:
1871:
1864:
1858:
1854:
1848:
1847:
1845:
1843:
1824:
1761:
1760:
1743:
1737:
1736:
1726:
1694:
1685:
1684:
1682:
1680:
1675:. 2 October 2018
1665:
1559:RNA interference
1494:General Hospital
1408:Laura Manuelidis
1405:neuropathologist
1158:of brain tissue
1050:
1046:
913:occipital cortex
768:procedures. The
759:infectious agent
748:Papua New Guinea
537:sodium valproate
204:
200:
193:
192:
189:
188:
185:
182:
179:
176:
173:
166:
155:
151:
146:
145:
142:
141:
138:
135:
132:
129:
126:
122:
121:
118:
115:
112:
109:
106:
103:
100:
97:
94:
87:
62:
42:
41:
21:
8105:
8104:
8100:
8099:
8098:
8096:
8095:
8094:
8090:1920 in biology
8055:
8054:
8053:
8044:
8016:
7997:
7981:
7950:
7928:
7919:
7894:
7869:
7826:
7823:substance abuse
7800:
7777:
7754:
7708:
7694:Bulimia nervosa
7671:
7652:
7628:Hypochondriasis
7623:False pregnancy
7606:Ganser syndrome
7588:Somatic symptom
7582:
7545:
7497:
7486:Specific phobia
7431:
7408:
7379:
7353:
7285:
7281:Savant syndrome
7253:Autism spectrum
7243:
7228:
7156:
7134:
7113:
7107:
7082:
6975:
6954:
6930:Dermatillomania
6891:
6877:Sexual anorexia
6834:
6823:
6813:
6783:
6770:
6696:Congenital DSMA
6617:
6574:
6533:
6479:
6467:Sleep disorders
6455:
6432:Cerebrovascular
6426:
6392:
6349:
6343:
6305:
6287:
6212:
6158:Choreoathetosis
6031:
6008:
5995:
5959:
5893:
5883:
5853:
5848:
5806:
5796:
5774:
5768:
5742:
5699:
5693:
5684:
5654:
5649:
5648:
5603:
5602:
5508:
5494:
5489:
5488:
5442:
5438:
5383:
5379:
5330:
5326:
5316:
5314:
5305:
5304:
5300:
5269:(12): 3204–10.
5255:
5251:
5242:
5240:
5238:www.cureffi.org
5232:
5231:
5227:
5186:
5182:
5173:
5171:
5162:
5161:
5157:
5148:
5146:
5135:
5131:
5086:
5082:
5045:
5041:
5032:
5030:
5026:
5015:
5011:
5010:
5006:
4971:
4967:
4954:
4953:
4949:
4940:
4938:
4929:
4928:
4924:
4905:
4901:
4892:
4890:
4881:
4880:
4876:
4866:
4864:
4849:
4845:
4835:
4833:
4824:
4823:
4819:
4809:
4807:
4798:
4797:
4793:
4772:
4768:
4755:
4754:
4750:
4745:Wayback Machine
4735:
4731:
4676:
4672:
4663:
4661:
4652:
4651:
4647:
4602:
4598:
4553:
4538:
4501:
4497:
4476:(1–4): 337–41.
4466:
4462:
4455:
4433:
4429:
4421:
4382:
4376:
4367:
4354:
4347:
4336:
4332:
4323:
4321:
4312:
4311:
4307:
4298:
4296:
4287:
4286:
4282:
4277:. 7 March 2011.
4269:
4268:
4264:
4219:
4215:
4170:
4166:
4157:
4155:
4146:
4145:
4141:
4104:
4097:
4066:
4062:
4017:
4013:
4000:
3999:
3990:
3956:
3950:
3939:
3910:
3906:
3893:
3892:
3888:
3869:
3865:
3860:
3853:
3798:
3794:
3741:
3737:
3682:
3678:
3637:
3633:
3596:
3592:
3551:
3547:
3488:
3484:
3453:
3449:
3418:
3411:
3379:
3375:
3346:(12): 2619–27.
3335:
3329:
3325:
3285:
3281:
3241:
3237:
3192:
3188:
3133:
3129:
3084:
3080:
3048:
3044:
3004:
3000:
2993:
2971:
2967:
2927:
2923:
2884:
2877:
2869:
2867:
2865:
2839:
2835:
2798:
2794:
2747:
2743:
2704:
2700:
2653:
2649:
2636:
2635:
2631:
2591:
2587:
2542:
2538:
2509:(8009): 478–9.
2495:
2491:
2460:(9): 999–1000.
2446:
2442:
2419:(10): 1926–30.
2405:
2401:
2356:
2352:
2307:
2303:
2296:
2280:
2276:
2231:
2227:
2218:
2216:
2205:
2198:
2159:
2155:
2138:
2134:
2125:
2123:
2110:
2109:
2105:
2095:
2093:
2080:
2079:
2075:
2065:
2063:
2050:
2049:
2045:
2035:
2033:
2020:
2019:
2015:
1978:
1974:
1929:
1922:
1909:
1907:
1898:
1897:
1890:
1879:
1875:
1866:
1865:
1861:
1855:
1851:
1841:
1839:
1826:
1825:
1764:
1758:
1744:
1740:
1695:
1688:
1678:
1676:
1667:
1666:
1659:
1654:
1632:
1548:
1530:
1525:
1489:Barbara Tarbuck
1441:
1429:
1420:
1402:Yale University
1337:
1247:
1234:
1226:palliative care
1218:
1209:May be present
1117:caudate nucleus
1003:
952:skeletal muscle
933:
909:globus pallidus
881:
805:
788:
730:mad cow disease
682:
641:
627:of the brain's
554:
388:supportive care
252:
208:
198:
194:
170:
161:
160:
153:
149:
123:
91:
82:
81:
70:of sporadic CJD
40:
37:
28:
23:
22:
15:
12:
11:
5:
8103:
8093:
8092:
8087:
8082:
8077:
8072:
8067:
8050:
8049:
8046:
8045:
8043:
8042:
8037:
8032:
8027:
8021:
8018:
8017:
8007:
8006:
8003:
8002:
7999:
7998:
7996:
7995:
7989:
7987:
7983:
7982:
7980:
7979:
7974:
7969:
7964:
7958:
7956:
7952:
7951:
7949:
7948:
7943:
7938:
7932:
7930:
7921:
7920:
7918:
7917:
7912:
7906:
7904:
7896:
7895:
7875:
7874:
7871:
7870:
7868:
7867:
7862:
7857:
7852:
7850:Rebound effect
7847:
7842:
7837:
7831:
7828:
7827:
7810:
7809:
7806:
7805:
7802:
7801:
7799:
7798:
7793:
7791:Hypersexuality
7787:
7785:
7779:
7778:
7776:
7775:
7770:
7764:
7762:
7756:
7755:
7753:
7752:
7751:
7750:
7745:
7740:
7730:
7725:
7719:
7717:
7710:
7709:
7707:
7706:
7701:
7696:
7691:
7685:
7683:
7673:
7672:
7662:
7661:
7658:
7657:
7654:
7653:
7651:
7650:
7645:
7640:
7635:
7630:
7625:
7620:
7619:
7618:
7613:
7608:
7598:
7592:
7590:
7584:
7583:
7581:
7580:
7575:
7570:
7565:
7559:
7557:
7551:
7550:
7547:
7546:
7544:
7543:
7542:
7541:
7536:
7526:
7524:Panic disorder
7521:
7516:
7511:
7505:
7503:
7499:
7498:
7496:
7495:
7494:
7493:
7491:Claustrophobia
7483:
7482:
7481:
7476:
7474:Anthropophobia
7466:
7464:Social anxiety
7461:
7456:
7450:
7448:
7439:
7433:
7432:
7430:
7429:
7422:
7420:
7410:
7409:
7389:
7388:
7385:
7384:
7381:
7380:
7378:
7377:
7372:
7367:
7361:
7359:
7355:
7354:
7352:
7351:
7346:
7341:
7336:
7334:Pick's disease
7331:
7326:
7321:
7316:
7311:
7306:
7301:
7295:
7293:
7287:
7286:
7284:
7283:
7278:
7273:
7268:
7263:
7257:
7255:
7245:
7244:
7234:
7233:
7230:
7229:
7227:
7226:
7221:
7220:
7219:
7214:
7209:
7204:
7199:
7189:
7188:
7187:
7182:
7177:
7172:
7161:
7158:
7157:
7144:
7143:
7140:
7139:
7136:
7135:
7133:
7132:
7127:
7121:
7119:
7109:
7108:
7106:
7105:
7104:
7103:
7092:
7090:
7084:
7083:
7081:
7080:
7079:
7078:
7068:
7067:
7066:
7061:
7051:
7050:
7049:
7044:
7039:
7031:
7030:
7029:
7019:
7018:
7017:
7007:
7006:
7005:
6995:
6989:
6987:
6977:
6976:
6964:
6963:
6960:
6959:
6956:
6955:
6953:
6952:
6947:
6942:
6937:
6932:
6927:
6922:
6917:
6912:
6911:
6910:
6899:
6897:
6893:
6892:
6890:
6889:
6884:
6879:
6874:
6869:
6868:
6867:
6862:
6852:
6846:
6844:
6836:
6835:
6825:
6824:
6820:Classification
6812:
6811:
6804:
6797:
6789:
6780:
6779:
6776:
6775:
6772:
6771:
6769:
6768:
6767:
6766:
6755:
6754:
6753:
6752:
6751:
6750:
6745:
6735:
6730:
6729:
6728:
6723:
6718:
6717:
6716:
6711:
6706:
6698:
6693:
6688:
6683:
6678:
6668:
6654:
6653:
6652:
6651:
6646:
6641:
6627:
6625:
6619:
6618:
6616:
6615:
6610:
6604:
6602:
6593:
6584:
6580:
6579:
6576:
6575:
6573:
6572:
6567:
6562:
6557:
6552:
6547:
6541:
6539:
6535:
6534:
6532:
6531:
6526:
6524:Cerebral edema
6521:
6520:
6519:
6514:
6509:
6504:
6493:
6491:
6485:
6484:
6481:
6480:
6478:
6477:
6476:
6475:
6473:Template:Sleep
6463:
6461:
6457:
6456:
6454:
6453:
6447:
6442:
6436:
6434:
6428:
6427:
6425:
6424:
6418:
6413:
6408:
6402:
6400:
6394:
6393:
6391:
6390:
6384:
6379:
6374:
6368:
6366:
6353:
6345:
6344:
6342:
6341:
6335:
6330:
6325:
6319:
6317:
6311:
6310:
6307:
6306:
6304:
6303:
6301:Leigh syndrome
6297:
6295:
6289:
6288:
6286:
6285:
6274:
6273:
6268:
6263:
6262:
6261:
6247:
6246:
6245:
6240:
6239:
6238:
6222:
6220:
6214:
6213:
6211:
6210:
6205:
6200:
6199:
6198:
6193:
6182:
6181:
6180:
6179:
6174:
6173:
6172:
6162:
6161:
6160:
6150:
6145:
6144:
6143:
6138:
6133:
6128:
6112:
6111:
6110:
6109:
6104:
6099:
6094:
6089:
6088:
6087:
6077:
6076:
6075:
6065:
6064:
6063:
6058:
6053:
6037:
6035:
6028:Extrapyramidal
6021:
6012:
6010:encephalopathy
6001:
6000:
5997:
5996:
5994:
5993:
5988:
5983:
5982:
5981:
5970:
5968:
5961:
5960:
5958:
5957:
5956:
5955:
5945:
5940:
5939:
5938:
5933:
5928:
5923:
5912:
5910:
5901:
5895:
5894:
5887:nervous system
5882:
5881:
5874:
5867:
5859:
5850:
5849:
5847:
5846:
5841:
5836:
5831:
5826:
5821:
5816:
5810:
5808:
5805:Prion diseases
5802:
5801:
5798:
5797:
5795:
5794:
5789:
5784:
5778:
5776:
5775:transmissible:
5770:
5769:
5767:
5766:
5761:
5756:
5750:
5748:
5744:
5743:
5741:
5740:
5735:
5730:
5725:
5720:
5714:
5712:
5701:
5698:Prion diseases
5695:
5694:
5687:Prion diseases
5683:
5682:
5675:
5668:
5660:
5651:
5650:
5647:
5646:
5635:
5624:
5612:
5611:
5609:
5605:
5604:
5601:
5600:
5589:
5578:
5567:
5552:
5533:
5509:
5504:
5503:
5501:
5500:Classification
5493:
5492:External links
5490:
5487:
5486:
5457:(4): 342–354.
5436:
5397:(17): 7044–9.
5377:
5324:
5311:news.bbc.co.uk
5298:
5249:
5225:
5180:
5155:
5129:
5080:
5039:
5004:
4965:
4947:
4933:. 2016-12-30.
4922:
4899:
4874:
4843:
4817:
4791:
4776:Bloomberg News
4766:
4748:
4729:
4690:(6): 1965–70.
4670:
4645:
4616:(9): 274–278.
4596:
4536:
4495:
4460:
4453:
4437:Prion Diseases
4427:
4365:
4345:
4330:
4305:
4280:
4262:
4213:
4164:
4139:
4095:
4060:
4011:
3988:
3937:
3904:
3886:
3863:
3851:
3792:
3735:
3696:(4): 411–413.
3676:
3631:
3590:
3565:(6): 1551–62.
3545:
3506:(3): 408–410.
3496:] disease"
3482:
3447:
3409:
3390:(1): 234–257.
3373:
3323:
3279:
3252:(2): 155–162.
3246:JAMA Neurology
3235:
3186:
3127:
3078:
3042:
2998:
2991:
2965:
2921:
2894:(1): 199–207.
2875:
2863:
2833:
2792:
2761:(3): 472–505.
2741:
2698:
2647:
2629:
2585:
2536:
2489:
2440:
2399:
2350:
2301:
2294:
2274:
2245:(2): 148–152.
2225:
2196:
2169:(5): 597–602.
2153:
2150:978-1437704341
2132:
2103:
2073:
2043:
2013:
1972:
1920:
1888:
1873:
1859:
1849:
1834:. March 2003.
1762:
1756:
1738:
1709:(2): 113–121.
1686:
1656:
1655:
1653:
1650:
1649:
1648:
1643:
1638:
1631:
1628:
1627:
1626:
1618:clinical trial
1606:
1600:
1590:
1587:benzodiazepine
1574:amphotericin B
1570:
1555:
1547:
1544:
1543:
1542:
1539:
1529:
1526:
1524:
1521:
1440:
1437:
1428:
1425:
1419:
1416:
1336:
1333:
1320:
1319:
1316:
1313:
1310:
1299:
1298:
1295:
1292:
1289:
1282:
1281:
1278:
1275:
1268:
1257:
1246:
1243:
1239:Jonathan Simms
1233:
1230:
1217:
1214:
1211:
1210:
1207:
1206:May be present
1204:
1200:
1199:
1196:
1193:
1181:
1180:
1177:
1174:
1166:
1165:
1162:
1159:
1152:
1151:
1148:
1145:
1134:
1133:
1130:
1127:
1112:
1111:
1108:
1105:
1098:
1097:
1090:
1087:
1083:
1082:
1079:
1076:
1072:
1071:
1068:
1065:
1061:
1060:
1057:
1054:
1053:Characteristic
1045:
1044:
1013:
1010:
1002:
1001:Classification
999:
932:
931:Histopathology
929:
928:
927:
920:
919:
916:
901:
880:
877:
869:
868:
862:
851:14-3-3 protein
844:
804:
801:
787:
784:
681:
678:
673:prion diseases
640:
637:
604:), changes in
592:symptoms, and
570:hallucinations
553:
550:
442:
441:
438:United Kingdom
434:
430:
429:
426:
422:
421:
418:
412:
411:
398:
392:
391:
384:
380:
379:
376:
372:
371:
350:
344:
343:
340:
334:
333:
330:
324:
323:
318:
314:
313:
290:
286:
285:
282:
278:
277:
274:
270:
269:
260:
254:
253:
251:
250:
237:
230:
228:
222:
221:
216:
210:
209:
207:
206:
78:
76:
72:
71:
64:
63:
55:
54:
51:
47:
46:
38:
26:
9:
6:
4:
3:
2:
8102:
8091:
8088:
8086:
8085:Rare diseases
8083:
8081:
8078:
8076:
8073:
8071:
8068:
8066:
8063:
8062:
8060:
8041:
8038:
8036:
8033:
8031:
8028:
8026:
8023:
8022:
8019:
8012:
8008:
7994:
7991:
7990:
7988:
7984:
7978:
7975:
7973:
7970:
7968:
7965:
7963:
7960:
7959:
7957:
7955:Schizophrenia
7953:
7947:
7944:
7942:
7939:
7937:
7934:
7933:
7931:
7926:
7922:
7916:
7913:
7911:
7908:
7907:
7905:
7901:
7897:
7893:
7889:
7885:
7884:Schizophrenia
7880:
7876:
7866:
7863:
7861:
7858:
7856:
7853:
7851:
7848:
7846:
7843:
7841:
7838:
7836:
7835:Drug overdose
7833:
7832:
7829:
7824:
7820:
7815:
7811:
7797:
7794:
7792:
7789:
7788:
7786:
7784:
7783:Sexual desire
7780:
7774:
7771:
7769:
7766:
7765:
7763:
7761:
7757:
7749:
7746:
7744:
7741:
7739:
7736:
7735:
7734:
7731:
7729:
7726:
7724:
7721:
7720:
7718:
7716:
7711:
7705:
7702:
7700:
7697:
7695:
7692:
7690:
7687:
7686:
7684:
7682:
7678:
7674:
7667:
7663:
7649:
7646:
7644:
7641:
7639:
7636:
7634:
7631:
7629:
7626:
7624:
7621:
7617:
7614:
7612:
7609:
7607:
7604:
7603:
7602:
7599:
7597:
7594:
7593:
7591:
7589:
7585:
7579:
7576:
7574:
7571:
7569:
7566:
7564:
7561:
7560:
7558:
7556:
7552:
7540:
7537:
7535:
7532:
7531:
7530:
7527:
7525:
7522:
7520:
7517:
7515:
7512:
7510:
7507:
7506:
7504:
7500:
7492:
7489:
7488:
7487:
7484:
7480:
7477:
7475:
7472:
7471:
7470:
7469:Social phobia
7467:
7465:
7462:
7460:
7457:
7455:
7452:
7451:
7449:
7447:
7443:
7440:
7438:
7434:
7427:
7424:
7423:
7421:
7419:
7415:
7411:
7407:
7404:-related and
7403:
7399:
7394:
7390:
7376:
7373:
7371:
7368:
7366:
7363:
7362:
7360:
7356:
7350:
7347:
7345:
7342:
7340:
7337:
7335:
7332:
7330:
7327:
7325:
7322:
7320:
7317:
7315:
7312:
7310:
7307:
7305:
7302:
7300:
7297:
7296:
7294:
7292:
7288:
7282:
7279:
7277:
7274:
7272:
7269:
7267:
7264:
7262:
7259:
7258:
7256:
7254:
7250:
7246:
7239:
7235:
7225:
7222:
7218:
7215:
7213:
7210:
7208:
7205:
7203:
7200:
7198:
7195:
7194:
7193:
7190:
7186:
7183:
7181:
7178:
7176:
7173:
7171:
7168:
7167:
7166:
7163:
7162:
7159:
7154:
7149:
7145:
7131:
7128:
7126:
7123:
7122:
7120:
7117:
7110:
7102:
7099:
7098:
7097:
7094:
7093:
7091:
7089:
7085:
7077:
7074:
7073:
7072:
7069:
7065:
7062:
7060:
7057:
7056:
7055:
7052:
7048:
7045:
7043:
7040:
7038:
7035:
7034:
7032:
7028:
7025:
7024:
7023:
7020:
7016:
7013:
7012:
7011:
7008:
7004:
7001:
7000:
6999:
6996:
6994:
6991:
6990:
6988:
6986:
6982:
6978:
6974:
6969:
6965:
6951:
6948:
6946:
6943:
6941:
6938:
6936:
6933:
6931:
6928:
6926:
6923:
6921:
6918:
6916:
6913:
6909:
6906:
6905:
6904:
6901:
6900:
6898:
6894:
6888:
6885:
6883:
6880:
6878:
6875:
6873:
6870:
6866:
6863:
6861:
6858:
6857:
6856:
6853:
6851:
6848:
6847:
6845:
6841:
6837:
6830:
6826:
6821:
6817:
6810:
6805:
6803:
6798:
6796:
6791:
6790:
6787:
6765:
6762:
6761:
6760:
6757:
6756:
6749:
6746:
6744:
6741:
6740:
6739:
6736:
6734:
6731:
6727:
6724:
6722:
6719:
6715:
6712:
6710:
6707:
6705:
6702:
6701:
6699:
6697:
6694:
6692:
6689:
6687:
6684:
6682:
6679:
6677:
6674:
6673:
6672:
6669:
6667:
6664:
6663:
6662:
6660:
6656:
6655:
6650:
6647:
6645:
6642:
6640:
6637:
6636:
6635:
6633:
6629:
6628:
6626:
6624:
6620:
6614:
6611:
6609:
6606:
6605:
6603:
6601:
6597:
6594:
6592:
6588:
6585:
6581:
6571:
6568:
6566:
6563:
6561:
6558:
6556:
6553:
6551:
6550:Reye syndrome
6548:
6546:
6543:
6542:
6540:
6536:
6530:
6527:
6525:
6522:
6518:
6515:
6513:
6510:
6508:
6505:
6503:
6502:Hydrocephalus
6500:
6499:
6498:
6495:
6494:
6492:
6490:
6486:
6474:
6470:
6469:
6468:
6465:
6464:
6462:
6458:
6452:
6448:
6446:
6443:
6441:
6438:
6437:
6435:
6433:
6429:
6423:
6419:
6417:
6414:
6412:
6409:
6407:
6404:
6403:
6401:
6399:
6395:
6389:
6385:
6383:
6380:
6378:
6375:
6373:
6370:
6369:
6367:
6365:
6361:
6357:
6354:
6352:
6346:
6340:
6336:
6334:
6331:
6329:
6326:
6324:
6321:
6320:
6318:
6316:
6315:Demyelinating
6312:
6302:
6299:
6298:
6296:
6294:
6290:
6284:
6281:
6280:
6279:
6278:
6272:
6269:
6267:
6264:
6260:
6257:
6256:
6255:
6251:
6248:
6244:
6241:
6237:
6234:
6233:
6232:
6229:
6228:
6227:
6224:
6223:
6221:
6219:
6215:
6209:
6206:
6204:
6203:Restless legs
6201:
6197:
6194:
6192:
6189:
6188:
6187:
6184:
6183:
6178:
6175:
6171:
6168:
6167:
6166:
6163:
6159:
6156:
6155:
6154:
6151:
6149:
6146:
6142:
6141:Blepharospasm
6139:
6137:
6134:
6132:
6129:
6127:
6124:
6123:
6122:
6119:
6118:
6117:
6114:
6113:
6108:
6105:
6103:
6100:
6098:
6097:Hemiballismus
6095:
6093:
6090:
6086:
6083:
6082:
6081:
6078:
6074:
6071:
6070:
6069:
6066:
6062:
6059:
6057:
6054:
6052:
6049:
6048:
6047:
6044:
6043:
6042:
6039:
6038:
6036:
6034:
6029:
6025:
6022:
6020:
6016:
6013:
6011:
6006:
6002:
5992:
5989:
5987:
5984:
5980:
5977:
5976:
5975:
5972:
5971:
5969:
5967:
5962:
5954:
5951:
5950:
5949:
5948:Brain abscess
5946:
5944:
5941:
5937:
5934:
5932:
5929:
5927:
5924:
5922:
5919:
5918:
5917:
5914:
5913:
5911:
5909:
5905:
5902:
5900:
5896:
5892:
5888:
5880:
5875:
5873:
5868:
5866:
5861:
5860:
5857:
5845:
5842:
5840:
5837:
5835:
5832:
5830:
5827:
5825:
5822:
5820:
5817:
5815:
5812:
5811:
5809:
5803:
5793:
5790:
5788:
5785:
5783:
5780:
5779:
5777:
5771:
5765:
5762:
5760:
5757:
5755:
5752:
5751:
5749:
5745:
5739:
5736:
5734:
5731:
5729:
5726:
5724:
5721:
5719:
5716:
5715:
5713:
5710:
5705:
5702:
5696:
5692:
5688:
5681:
5676:
5674:
5669:
5667:
5662:
5661:
5658:
5645:
5641:
5640:
5636:
5634:
5630:
5629:
5625:
5623:
5619:
5618:
5614:
5613:
5610:
5606:
5599:
5595:
5594:
5590:
5588:
5584:
5583:
5579:
5577:
5573:
5572:
5568:
5566:
5562:
5561:
5557:
5553:
5551:
5547:
5543:
5542:
5538:
5534:
5532:
5528:
5524:
5520:
5519:
5515:
5511:
5510:
5507:
5502:
5498:
5482:
5478:
5474:
5470:
5465:
5460:
5456:
5452:
5448:
5440:
5432:
5428:
5423:
5418:
5413:
5408:
5404:
5400:
5396:
5392:
5388:
5381:
5373:
5369:
5364:
5359:
5355:
5351:
5348:(4): 334–44.
5347:
5343:
5339:
5335:
5328:
5312:
5308:
5302:
5294:
5290:
5285:
5280:
5276:
5272:
5268:
5264:
5260:
5253:
5239:
5235:
5229:
5221:
5217:
5213:
5209:
5205:
5201:
5198:(5): 713–26.
5197:
5193:
5192:
5184:
5169:
5165:
5159:
5144:
5140:
5133:
5125:
5121:
5116:
5111:
5107:
5103:
5100:(6): 519–29.
5099:
5095:
5091:
5084:
5076:
5072:
5067:
5062:
5058:
5054:
5050:
5043:
5025:
5021:
5014:
5008:
5000:
4996:
4992:
4988:
4984:
4980:
4976:
4969:
4961:
4957:
4951:
4936:
4932:
4926:
4918:
4914:
4910:
4903:
4888:
4884:
4878:
4862:
4858:
4854:
4851:Kowalczyk L.
4847:
4831:
4827:
4821:
4805:
4801:
4795:
4788:
4787:
4782:
4778:
4777:
4770:
4762:
4758:
4752:
4746:
4742:
4738:
4733:
4725:
4721:
4716:
4711:
4706:
4701:
4697:
4693:
4689:
4685:
4681:
4674:
4659:
4655:
4649:
4641:
4637:
4632:
4627:
4623:
4619:
4615:
4611:
4607:
4600:
4592:
4588:
4583:
4578:
4574:
4570:
4567:(2): 155–63.
4566:
4562:
4558:
4551:
4549:
4547:
4545:
4543:
4541:
4532:
4528:
4523:
4518:
4514:
4510:
4506:
4499:
4491:
4487:
4483:
4479:
4475:
4471:
4464:
4456:
4450:
4446:
4442:
4438:
4431:
4420:
4416:
4412:
4408:
4404:
4400:
4396:
4393:(1): e2–e10.
4392:
4388:
4381:
4374:
4372:
4370:
4361:
4360:
4352:
4350:
4342:
4339:
4334:
4320:on 2009-05-07
4319:
4315:
4309:
4295:on 2009-05-06
4294:
4290:
4284:
4276:
4272:
4266:
4258:
4254:
4249:
4244:
4240:
4236:
4233:(2): 103–15.
4232:
4228:
4224:
4217:
4209:
4205:
4200:
4195:
4191:
4187:
4183:
4179:
4175:
4168:
4153:
4149:
4143:
4135:
4131:
4126:
4121:
4117:
4113:
4109:
4102:
4100:
4091:
4087:
4083:
4079:
4075:
4071:
4064:
4056:
4052:
4047:
4042:
4038:
4034:
4030:
4026:
4022:
4015:
4007:
4003:
3997:
3995:
3993:
3984:
3980:
3975:
3970:
3966:
3962:
3955:
3948:
3946:
3944:
3942:
3932:
3927:
3923:
3919:
3915:
3908:
3900:
3896:
3890:
3882:
3878:
3874:
3867:
3858:
3856:
3847:
3843:
3838:
3833:
3829:
3825:
3820:
3815:
3811:
3807:
3803:
3796:
3788:
3784:
3779:
3774:
3770:
3766:
3762:
3758:
3754:
3750:
3746:
3739:
3731:
3727:
3723:
3719:
3714:
3709:
3704:
3699:
3695:
3691:
3687:
3680:
3672:
3668:
3664:
3659:
3654:
3651:(5): 908–15.
3650:
3646:
3642:
3635:
3627:
3623:
3618:
3613:
3609:
3605:
3601:
3594:
3586:
3582:
3578:
3573:
3568:
3564:
3560:
3556:
3549:
3541:
3537:
3532:
3527:
3523:
3519:
3514:
3509:
3505:
3501:
3497:
3495:
3486:
3478:
3474:
3470:
3466:
3462:
3458:
3451:
3443:
3439:
3435:
3431:
3427:
3423:
3416:
3414:
3405:
3401:
3397:
3393:
3389:
3385:
3384:Radiographics
3377:
3369:
3365:
3361:
3357:
3353:
3349:
3345:
3341:
3334:
3327:
3319:
3315:
3311:
3307:
3303:
3299:
3296:(4): 637–43.
3295:
3291:
3283:
3275:
3271:
3267:
3263:
3259:
3255:
3251:
3247:
3239:
3231:
3227:
3222:
3217:
3213:
3209:
3205:
3201:
3197:
3190:
3182:
3178:
3173:
3168:
3163:
3158:
3154:
3150:
3147:(1): e54915.
3146:
3142:
3138:
3131:
3123:
3119:
3114:
3109:
3105:
3101:
3098:(3): 437–44.
3097:
3093:
3089:
3082:
3074:
3070:
3066:
3062:
3058:
3054:
3046:
3038:
3034:
3030:
3026:
3022:
3018:
3015:(4): 216–25.
3014:
3010:
3002:
2994:
2988:
2984:
2979:
2978:
2969:
2961:
2957:
2953:
2949:
2945:
2941:
2937:
2933:
2925:
2917:
2913:
2909:
2905:
2901:
2897:
2893:
2889:
2882:
2880:
2866:
2860:
2856:
2852:
2848:
2844:
2837:
2829:
2825:
2820:
2815:
2811:
2807:
2803:
2796:
2788:
2784:
2779:
2774:
2769:
2764:
2760:
2756:
2752:
2745:
2737:
2733:
2729:
2725:
2721:
2717:
2713:
2709:
2702:
2694:
2690:
2686:
2682:
2678:
2674:
2670:
2666:
2662:
2658:
2651:
2643:
2639:
2633:
2625:
2621:
2617:
2613:
2609:
2605:
2602:(5): 513–29.
2601:
2597:
2589:
2581:
2577:
2572:
2567:
2563:
2559:
2555:
2551:
2547:
2540:
2532:
2528:
2524:
2520:
2516:
2512:
2508:
2504:
2500:
2493:
2485:
2481:
2476:
2471:
2467:
2463:
2459:
2455:
2451:
2444:
2436:
2432:
2427:
2422:
2418:
2414:
2410:
2403:
2395:
2391:
2386:
2381:
2377:
2373:
2369:
2365:
2361:
2354:
2346:
2342:
2337:
2332:
2328:
2324:
2320:
2316:
2312:
2305:
2297:
2291:
2287:
2286:
2278:
2270:
2266:
2262:
2258:
2253:
2248:
2244:
2240:
2236:
2229:
2214:
2210:
2203:
2201:
2192:
2188:
2184:
2180:
2176:
2172:
2168:
2164:
2157:
2151:
2147:
2143:
2136:
2122:on 2017-07-06
2121:
2117:
2113:
2107:
2091:
2087:
2083:
2077:
2061:
2057:
2053:
2047:
2031:
2027:
2023:
2017:
2009:
2005:
2000:
1995:
1991:
1987:
1983:
1976:
1968:
1964:
1960:
1956:
1951:
1946:
1942:
1938:
1934:
1927:
1925:
1917:
1905:
1901:
1895:
1893:
1886:
1882:
1877:
1869:
1863:
1853:
1837:
1833:
1829:
1823:
1821:
1819:
1817:
1815:
1813:
1811:
1809:
1807:
1805:
1803:
1801:
1799:
1797:
1795:
1793:
1791:
1789:
1787:
1785:
1783:
1781:
1779:
1777:
1775:
1773:
1771:
1769:
1767:
1759:
1753:
1749:
1742:
1734:
1730:
1725:
1720:
1716:
1712:
1708:
1704:
1700:
1693:
1691:
1674:
1670:
1664:
1662:
1657:
1647:
1644:
1642:
1639:
1637:
1634:
1633:
1624:
1619:
1615:
1611:
1607:
1604:
1601:
1598:
1594:
1591:
1588:
1584:
1579:
1575:
1571:
1568:
1564:
1560:
1556:
1553:
1550:
1549:
1540:
1537:
1532:
1531:
1520:
1518:
1514:
1510:
1506:
1502:
1501:
1496:
1495:
1490:
1486:
1485:
1480:
1479:
1474:
1470:
1467:
1462:
1458:
1457:Michael Homer
1454:
1450:
1446:
1439:United States
1436:
1434:
1424:
1415:
1413:
1409:
1406:
1403:
1399:
1397:
1393:
1389:
1385:
1383:
1378:
1376:
1371:
1367:
1365:
1360:
1355:
1353:
1349:
1345:
1342:
1332:
1330:
1326:
1317:
1314:
1311:
1308:
1307:
1306:
1303:
1296:
1293:
1290:
1287:
1286:
1285:
1279:
1276:
1273:
1269:
1266:
1262:
1258:
1255:
1254:
1253:
1251:
1242:
1240:
1229:
1227:
1223:
1208:
1205:
1202:
1201:
1197:
1195:Not reported
1194:
1191:
1187:
1183:
1182:
1178:
1175:
1172:
1168:
1167:
1163:
1160:
1157:
1154:
1153:
1149:
1147:Not reported
1146:
1143:
1139:
1136:
1135:
1132:Often absent
1131:
1129:Often present
1128:
1126:
1122:
1118:
1114:
1113:
1110:Often absent
1109:
1107:Often present
1106:
1104:
1100:
1099:
1095:
1091:
1088:
1085:
1084:
1081:13–14 months
1080:
1077:
1074:
1073:
1069:
1066:
1063:
1062:
1058:
1055:
1052:
1051:
1042:
1038:
1034:
1030:
1027:
1023:
1018:
1014:
1011:
1008:
1007:
1006:
998:
996:
992:
988:
984:
980:
979:vacuolization
975:
973:
969:
964:
961:
957:
953:
949:
944:
937:
925:
924:
923:
917:
914:
910:
906:
905:basal ganglia
902:
898:
897:
892:
888:
885:
876:
874:
866:
863:
860:
856:
852:
848:
845:
842:
839:
838:
837:
835:
831:
827:
823:
814:
809:
800:
798:
793:
792:chromosome 20
783:
780:
775:
771:
767:
766:sterilization
764:
760:
755:
753:
749:
745:
741:
737:
733:
731:
727:
722:
720:
719:
714:
710:
705:
703:
699:
695:
686:
677:
675:
674:
669:
668:feedback loop
665:
661:
660:exponentially
657:
652:
650:
646:
636:
634:
630:
626:
621:
619:
615:
611:
607:
603:
599:
595:
591:
587:
583:
579:
575:
571:
568:changes, and
567:
563:
560:, leading to
559:
549:
547:
543:
538:
534:
530:
525:
523:
519:
515:
511:
507:
503:
499:
495:
491:
486:
484:
480:
476:
472:
468:
464:
461:, is a fatal
460:
456:
452:
448:
439:
435:
431:
427:
423:
419:
417:
413:
410:
406:
403:
399:
397:
393:
389:
385:
381:
377:
373:
370:
366:
362:
358:
354:
351:
349:
345:
341:
339:
335:
331:
329:
325:
322:
319:
315:
311:
307:
303:
299:
296:), Familial (
295:
291:
287:
283:
279:
275:
271:
268:
264:
261:
259:
258:Complications
255:
249:
245:
241:
238:
235:
232:
231:
229:
227:
223:
220:
217:
215:
211:
203:
202:
191:
164:
158:
157:
144:
85:
80:
79:
77:
75:Pronunciation
73:
69:
65:
61:
56:
52:
48:
43:
35:
30:
19:
7915:Folie à deux
7840:Intoxication
7821:substances,
7819:Psychoactive
7738:Night terror
7555:Dissociative
7519:Panic attack
7308:
7071:Tic disorder
6758:
6657:
6630:
6591:Degenerative
6328:Inflammatory
6276:
6275:
6208:Stiff-person
6046:Parkinsonism
6019:Degenerative
5916:Encephalitis
5899:Inflammation
5889:, primarily
5781:
5753:
5717:
5637:
5626:
5615:
5591:
5580:
5569:
5554:
5535:
5512:
5454:
5450:
5439:
5394:
5390:
5380:
5345:
5341:
5327:
5317:25 September
5315:. Retrieved
5310:
5301:
5266:
5262:
5252:
5241:. Retrieved
5237:
5228:
5195:
5189:
5183:
5172:. Retrieved
5158:
5147:. Retrieved
5132:
5097:
5093:
5083:
5056:
5052:
5042:
5031:. Retrieved
5024:the original
5019:
5007:
4982:
4978:
4968:
4960:statnews.com
4959:
4950:
4939:. Retrieved
4925:
4912:
4902:
4891:. Retrieved
4877:
4865:. Retrieved
4861:the original
4857:Boston Globe
4856:
4846:
4834:. Retrieved
4820:
4810:20 September
4808:. Retrieved
4794:
4784:
4774:
4769:
4760:
4751:
4732:
4687:
4683:
4673:
4662:. Retrieved
4648:
4613:
4609:
4599:
4564:
4560:
4512:
4508:
4498:
4473:
4469:
4463:
4436:
4430:
4390:
4386:
4358:
4340:
4333:
4322:. Retrieved
4318:the original
4308:
4297:. Retrieved
4293:the original
4283:
4274:
4265:
4230:
4226:
4216:
4181:
4177:
4167:
4156:. Retrieved
4154:. 2017-10-23
4151:
4142:
4115:
4111:
4073:
4069:
4063:
4028:
4024:
4014:
4006:the original
3964:
3960:
3921:
3917:
3907:
3898:
3889:
3872:
3866:
3809:
3805:
3795:
3752:
3748:
3738:
3693:
3689:
3679:
3648:
3644:
3634:
3610:(2): 281–3.
3607:
3603:
3593:
3562:
3558:
3548:
3503:
3499:
3493:
3485:
3463:(2): 560–6.
3460:
3456:
3450:
3428:(3): 793–8.
3425:
3421:
3387:
3383:
3376:
3343:
3339:
3326:
3293:
3289:
3282:
3249:
3245:
3238:
3206:(1): 79–92.
3203:
3199:
3189:
3144:
3140:
3130:
3095:
3091:
3081:
3059:(6): 813–6.
3056:
3052:
3045:
3012:
3008:
3001:
2976:
2968:
2938:(6): 763–7.
2935:
2931:
2924:
2891:
2887:
2868:, retrieved
2846:
2836:
2809:
2805:
2795:
2758:
2754:
2744:
2711:
2707:
2701:
2660:
2656:
2650:
2641:
2632:
2599:
2595:
2588:
2556:(6): 901–7.
2553:
2549:
2539:
2506:
2502:
2492:
2457:
2453:
2443:
2416:
2412:
2402:
2367:
2363:
2353:
2318:
2314:
2304:
2284:
2277:
2242:
2238:
2228:
2217:. Retrieved
2207:Gambetti P.
2166:
2162:
2156:
2135:
2124:. Retrieved
2120:the original
2115:
2106:
2094:. Retrieved
2085:
2076:
2064:. Retrieved
2055:
2046:
2034:. Retrieved
2025:
2016:
1992:(1): 83–85.
1989:
1985:
1975:
1940:
1936:
1915:
1908:. Retrieved
1903:
1885:Who Named It
1876:
1862:
1852:
1840:. Retrieved
1831:
1747:
1741:
1706:
1702:
1677:. Retrieved
1672:
1505:José Baselga
1498:
1492:
1482:
1476:
1473:John Carroll
1442:
1430:
1421:
1400:
1386:
1382:gonadotropin
1379:
1372:
1368:
1356:
1338:
1321:
1304:
1300:
1283:
1248:
1245:Epidemiology
1235:
1219:
1094:dysesthesias
1059:Variant CJD
1004:
976:
966:The classic
965:
945:
942:
921:
886:
882:
870:
818:
789:
757:Prions, the
756:
734:
729:
723:
716:
706:
691:
680:Transmission
671:
653:
642:
622:
608:, and rigid
555:
526:
487:
458:
454:
450:
446:
445:
401:
353:Encephalitis
328:Risk factors
304:(acquired),
239:
233:
29:
7888:schizotypal
7723:Hypersomnia
7713:Nonorganic
7454:Agoraphobia
7185:Cyclothymia
7180:Bipolar NOS
7155:(affective)
7027:Stereotypic
6935:Kleptomania
6743:Fazio–Londe
6583:Both/either
6377:Generalised
6236:Early-onset
6231:Alzheimer's
5966:spinal cord
5617:MedlinePlus
4867:25 November
4836:5 September
4341:www.cdc.gov
4148:"Treatment"
3340:Transfusion
1904:www.cdc.gov
1679:21 November
1578:doxorubicin
1509:AstraZeneca
1427:New Zealand
1341:neurologist
1325:nephropathy
1078:4–5 months
1056:Classic CJD
1029:transplants
954:and/or the
772:and the US
744:Fore people
736:Cannibalism
700:grafts, or
629:nerve cells
566:personality
562:memory loss
436:131 in the
386:None, only
273:Usual onset
50:Other names
8059:Categories
8040:Stereotypy
7903:Delusional
7892:delusional
7865:Withdrawal
7733:Parasomnia
7638:Nosophobia
7418:Adjustment
7406:somatoform
7339:Sundowning
7192:Depression
7175:Bipolar II
7064:Stuttering
7059:Cluttering
6855:Paraphilia
6351:paroxysmal
6323:Autoimmune
6116:Dyskinesia
5986:Meningitis
5964:Brain and
5707:inherited/
5639:Patient UK
5593:DiseasesDB
5243:2022-05-09
5174:2007-10-30
5149:2012-11-09
5033:2011-08-21
4941:2016-12-30
4893:2015-01-18
4664:2011-02-21
4515:: 213–39.
4324:2009-06-20
4299:2009-06-20
4158:2018-04-17
4031:(1): E10.
3967:: w14212.
2870:2024-03-15
2219:2011-06-04
2146:1437704344
2126:2017-07-09
1652:References
1603:Astemizole
1593:Quinacrine
1453:podiatrist
1433:dura mater
1359:iatrogenic
1274:per year).
1190:immunoblot
1184:Increased
1017:iatrogenic
968:histologic
797:iatrogenic
633:microscope
582:depression
533:clonazepam
518:spinal tap
396:Medication
375:Prevention
302:Iatrogenic
292:Sporadic (
267:swallowing
7993:Catatonia
7925:Psychosis
7760:Postnatal
7743:Nightmare
7349:Wandering
7202:Dysthymia
7170:Bipolar I
7125:Pervasive
6940:Pyromania
6865:Voyeurism
6860:Fetishism
6348:Episodic/
6226:Tauopathy
6177:Akathisia
6165:Myoclonus
6148:Athetosis
6080:Tauopathy
5773:acquired/
5747:sporadic:
5700:in humans
5633:neuro/725
5628:eMedicine
5481:247479825
4999:0028-3878
4979:Neurology
4490:178922541
4415:209483271
4118:(5): E2.
3828:2076-0817
3806:Pathogens
3769:0095-1137
3730:244465297
3522:0976-3147
3422:Radiology
3290:Neurology
2755:Pathogens
2364:Continuum
2261:1018-5615
1959:1092-0684
1943:(5): E2.
1857:35761497.
1583:valproate
1546:Treatment
1528:Diagnosis
1418:Australia
1261:incidence
1232:Prognosis
1222:twitching
1216:Treatment
1188:ratio on
1186:glycoform
1142:FLAIR MRI
1125:FLAIR MRI
1070:28 years
1026:meningeal
803:Diagnosis
715:for PrP,
702:electrode
656:refolding
618:pneumonia
594:psychosis
574:Myoclonus
498:inherited
494:misfolded
425:Frequency
416:Prognosis
409:Methadone
383:Treatment
310:infection
276:Around 60
219:Neurology
214:Specialty
197:-
8075:Dementia
7728:Insomnia
7398:Neurotic
7365:Delirium
7291:Dementia
7130:Specific
6714:SMALED2B
6709:SMALED2A
6406:Migraine
6398:Headache
6364:epilepsy
6360:Seizures
6218:Dementia
6121:Dystonia
5473:35305340
5431:23576755
5372:19278902
5334:Rossor M
5293:17143329
5220:12725001
5212:17477808
5168:Archived
5143:Archived
5124:25099576
5075:20357038
4935:Archived
4917:Archived
4887:Archived
4830:Archived
4804:Archived
4741:Archived
4724:17267596
4658:Archived
4640:29518068
4531:14522861
4419:Archived
4407:31876504
4275:BBC News
4208:25667875
4184:: 75–9.
4134:26646926
4090:12489284
4055:27364252
3983:26715203
3924:: 1–11.
3881:16903142
3846:29301257
3812:(1): 5.
3787:31366689
3722:35018165
3671:Archived
3667:12748093
3626:26238299
3585:Archived
3581:15956529
3540:23189017
3477:15671380
3404:28076012
3368:33032755
3360:20561299
3318:23306766
3310:16924018
3274:40578363
3266:27942718
3230:27893164
3181:23372790
3141:PLOS ONE
3122:21674591
3073:12810484
3037:21795464
3029:10343153
2960:13562215
2916:38449324
2847:Demenzen
2828:15722550
2787:25437203
2736:11506094
2728:16798390
2624:22496358
2580:22607808
2531:26804024
2435:19136921
2394:26633779
2345:11260799
2269:24272930
2213:Archived
2090:Archived
2060:Archived
2030:Archived
2008:32609989
1967:26646926
1836:Archived
1733:28153848
1630:See also
1523:Research
1513:Cerdanya
1171:lymphoid
1138:Pulvinar
1067:68 years
987:ischemia
972:neuropil
828:such as
826:symptoms
786:Genetics
709:mutation
696:grafts,
586:paranoia
558:dementia
467:dementia
405:Morphine
298:heredity
294:mutation
281:Duration
244:dementia
226:Symptoms
7437:Anxiety
7276:PDD-NOS
7165:Bipolar
6818: (
6726:SMA-PME
6721:SMA-PCH
6704:SMALED1
6416:Tension
6411:Cluster
6136:Meige's
5953:Amoebic
5824:Scrapie
5587:D007562
5422:3637718
5399:Bibcode
5363:2660392
5284:1679709
5115:4186748
4715:1794316
4692:Bibcode
4631:5844283
4591:9204296
4582:2627622
4257:7012278
4199:4308028
4046:5082740
3837:5874731
3778:6760952
3713:8686746
3658:7975779
3572:8149066
3531:3505356
3442:8638007
3221:5266667
3172:3556051
3149:Bibcode
3113:3170496
2952:9629846
2908:3961045
2778:4235695
2693:4355186
2685:8878476
2665:Bibcode
2616:8179297
2571:3358170
2499:Regli F
2484:8410042
2385:4879966
2336:1088424
2191:7995631
2183:3539001
2096:16 July
2066:16 July
2036:29 July
1910:17 June
1842:16 July
1724:5520355
1597:malaria
1563:scrapie
1557:Use of
1390:of the
1364:Lyodura
1335:History
1272:billion
1265:million
1173:tissue
1121:putamen
1022:corneal
879:Imaging
694:corneal
610:posture
578:anxiety
529:Opioids
306:Variant
7681:Eating
7529:Stress
7446:Phobia
7402:stress
7261:Autism
7054:Speech
6843:Sexual
6445:Stroke
6259:Pick's
6186:Tremor
6153:Chorea
5622:000788
5576:123400
5531:6D85.5
5527:8E02.0
5479:
5471:
5429:
5419:
5370:
5360:
5291:
5281:
5218:
5210:
5122:
5112:
5073:
4997:
4828:. AP.
4722:
4712:
4638:
4628:
4589:
4579:
4529:
4488:
4451:
4413:
4405:
4255:
4248:490840
4245:
4206:
4196:
4152:nhs.uk
4132:
4088:
4053:
4043:
3981:
3879:
3844:
3834:
3826:
3785:
3775:
3767:
3728:
3720:
3710:
3665:
3655:
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