Creutzfeldt–Jakob disease

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%

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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

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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

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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,

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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

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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

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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

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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

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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

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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

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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

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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

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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

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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.

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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.

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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

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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

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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).

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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

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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".

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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".

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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.

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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".

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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,

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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.

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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".

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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

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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

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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?".

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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:

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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".

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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

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Brown P, Cathala F, Castaigne P, Gajdusek DC (November 1986). "Creutzfeldt–Jakob disease: clinical analysis of a consecutive series of 230 neuropathologically verified cases".

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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".

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who conducted the study see these early-stage results as encouraging and suggest to conduct a larger study, ideally at the earliest possible intervention.

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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.

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has challenged the prion protein (PrP) explanation for the disease. In January 2007, she and her colleagues reported that they had found a

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Orrú CD, Bongianni M, Tonoli G, Ferrari S, Hughson AG, Groveman BR, Fiorini M, Pocchiari M, Monaco S, Caughey B, Zanusso G (August 2014).

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In 2014, a human study showed a nasal brushing method that can accurately detect PrP in the olfactory epithelial cells of people with CJD.

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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).

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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.

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implants and pituitary human growth hormone, which has been replaced by recombinant human growth hormone that poses no such risk.

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Karapetyan YE, Sferrazza GF, Zhou M, Ottenberg G, Spicer T, Chase P, Fallahi M, Hodder P, Weissmann C, Lasmézas CI (April 2013).

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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.).

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Brown P, Brandel JP, Sato T, Nakamura Y, MacKenzie J, Will RG, Ladogana A, Pocchiari M, Leschek EW, Schonberger LB (June 2012).

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monitors the occurrence of CJD in the United States through periodic reviews of national mortality data. According to the CDC:

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Sporadic (sCJD), caused by the spontaneous misfolding of prion-protein in an individual. This accounts for 85% of cases of CJD.

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Approximately 85% of CJD cases are sporadic and 10–15% of CJD cases are due to inherited mutations of the prion protein gene.

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Bonda DJ, Manjila S, Mehndiratta P, Khan F, Miller BR, Onwuzulike K, Puoti G, Cohen ML, Schonberger LB, Cali I (July 2016).

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Meggendorfer F (1930). "Klinische und genealogische Beobachtungen bei einem Fall von spastischer Pseudokosklerose Jakobs".

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Surveillance studies from 2005 and later show the estimated global incidence is 1–2 cases per million population per year.

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In the United States, CJD deaths among people younger than 30 years of age are extremely rare (fewer than five deaths per

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appearance is spongiform change in the gray matter: the presence of many round vacuoles from one to 50 micrometers in the

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People can also develop CJD because they carry a mutation of the gene that codes for the prion protein (PRNP), located on

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Acquired CJD, caused by contamination with tissue from an infected person, usually as the result of a medical procedure (

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In more than 85% of cases, the duration of CJD is less than one year (median: four months) after the onset of symptoms.

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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

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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

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in CSF samples of people with CJD. It is therefore suggested as a high-value diagnostic method for the disease.

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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.

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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.

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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).

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CJD incidence is greatest in those over the age of 55 years old, with an average age of 67 years old.

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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.

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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).

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CJD incidence was 3.5 cases per million among those over 50 years of age between 1979 and 2017.

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due to impaired coughing reflexes. About 15% of people with CJD survive for two or more years.

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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)

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Manix M, Kalakoti P, Henry M, Thakur J, Menger R, Guthikonda B, Nanda A (November 2015).

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The defective protein can be transmitted by contaminated harvested human brain products,

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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: 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: 1668: 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:. 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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: 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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: 3624: 3579: 3569: 3538: 3528: 3520: 3475: 3440: 3402: 3366: 3358: 3316: 3308: 3272: 3264: 3228: 3218: 3179: 3169: 3120: 3110: 3071: 3035: 3027: 2989: 2958: 2950: 2914: 2906: 2861: 2826: 2785: 2775: 2734: 2726: 2708:Lancet 2691: 2683: 2657:Nature 2642:CIDRAP 2622: 2614: 2578: 2568: 2529: 2521: 2503:Lancet 2482: 2475:489736 2472: 2433: 2392: 2382: 2343: 2333: 2292: 2267: 2259: 2189: 2181: 2144: 2006: 1965: 1957: 1754: 1731: 1721: 1041:prions 960:biopsy 956:spleen 649:prions 602:ataxia 598:speech 500:in an 440:(2020) 433:Deaths 317:Causes 205: 152:-felt 150:KROYTS 7986:Other 7715:sleep 7502:Other 7358:Other 7224:Mania 6896:Other 6759:both: 6691:DSMA1 6686:SMAX2 6681:SMAX1 6661:only: 6634:only: 6538:Other 6460:Other 6372:Focal 6005:Brain 5908:Brain 5565:046.1 5550:F02.1 5546:A81.0 5477:S2CID 5216:S2CID 5027:(PDF) 5016:(PDF) 4761:Stuff 4486:S2CID 4422:(PDF) 4411:S2CID 4383:(PDF) 3957:(PDF) 3726:S2CID 3364:S2CID 3336:(PDF) 3314:S2CID 3270:S2CID 3033:S2CID 2956:S2CID 2912:S2CID 2732:S2CID 2689:S2CID 2620:S2CID 2527:S2CID 2523:65575 2187:S2CID 1832:NINDS 1585:or a 1572:Both 1412:virus 822:signs 698:dural 664:cells 639:Cause 625:death 520:, or 490:prion 321:Prion 289:Types 240:Later 234:Early 201:-kohb 7890:and 7539:PTSD 7153:Mood 6993:ADHD 6362:and 6073:PKAN 6068:NBIA 5792:Kuru 5787:vCJD 5782:iCJD 5754:sCJD 5718:fCJD 5709:PRNP 5689:and 5598:3166 5582:MeSH 5571:OMIM 5560:9-CM 5523:8E00 5469:PMID 5427:PMID 5368:PMID 5319:2022 5289:PMID 5208:PMID 5120:PMID 5071:PMID 4995:ISSN 4869:2013 4838:2013 4812:2013 4773:via 4720:PMID 4636:PMID 4587:PMID 4527:PMID 4449:ISBN 4403:PMID 4253:PMID 4204:PMID 4130:PMID 4086:PMID 4051:PMID 3979:PMID 3922:2013 3877:PMID 3842:PMID 3824:ISSN 3783:PMID 3765:ISSN 3718:PMID 3663:PMID 3622:PMID 3577:PMID 3536:PMID 3518:ISSN 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