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Creutzfeldt-Jakob disease

Henry G Brown, MD, PhD
John M Lee, MD, PhD
Section Editor
Steven T DeKosky, MD, FAAN, FACP, FANA
Deputy Editor
Janet L Wilterdink, MD


Prion diseases are neurodegenerative diseases that have long incubation periods and progress inexorably once clinical symptoms appear. Five human prion diseases are currently recognized: kuru, Creutzfeldt-Jakob disease (CJD), variant Creutzfeldt-Jakob disease (vCJD), Gerstmann-Sträussler-Scheinker syndrome (GSS), and fatal familial insomnia (FFI). CJD accounts for more than 90 percent of sporadic prion disease [1].

These human prion diseases share certain common neuropathologic features including neuronal loss, proliferation of glial cells, absence of an inflammatory response, and the presence of small vacuoles within the neuropil, which produces a spongiform appearance.

The clinical manifestations and diagnosis of Creutzfeldt-Jakob disease will be reviewed here. Variant Creutzfeldt-Jakob disease, Kuru, GSS, and FFI, and the biology of prions and the genetics of prion diseases are discussed separately. (See "Variant Creutzfeldt-Jakob disease" and "Diseases of the central nervous system caused by prions" and "Biology and genetics of prions".)


Creutzfeldt-Jakob disease (CJD) is the most frequent of the human prion diseases, although it is still rare. Sporadic (sCJD), familial (fCJD), iatrogenic (iCJD), and variant forms of CJD (vCJD) are all recognized. The vast majority of CJD cases are sporadic (85 to 95 percent), while 5 to 15 percent are due to fCJD; iCJD generally accounts for less than 1 percent [2,3].

Approximately one case of sporadic CJD occurs per 1,000,000 population per year with a worldwide distribution. The mean age for the onset of disease is between 57 and 62 years, although rare cases in young adults and those over 80 years of age have been described [3-7]. Patients with vCJD and iCJD tend to be much younger, which led to an early appreciation that the mode of transmission might be different. fCJD patients have only a slightly younger age of onset compared with sCJD [8]. There is no gender predilection for CJD. In the United States, the incidence of CJD appears to be less in African Americans, American Indians, and Alaska natives compared with the white population, however this observation may have resulted from ascertainment bias [9,10].


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  1. Puoti G, Bizzi A, Forloni G, et al. Sporadic human prion diseases: molecular insights and diagnosis. Lancet Neurol 2012; 11:618.
  2. Masters CL, Harris JO, Gajdusek DC, et al. Creutzfeldt-Jakob disease: patterns of worldwide occurrence and the significance of familial and sporadic clustering. Ann Neurol 1979; 5:177.
  3. Ladogana A, Puopolo M, Croes EA, et al. Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada. Neurology 2005; 64:1586.
  4. Brown P, Cathala F, Raubertas RF, et al. The epidemiology of Creutzfeldt-Jakob disease: conclusion of a 15-year investigation in France and review of the world literature. Neurology 1987; 37:895.
  5. Monreal J, Collins GH, Masters CL, et al. Creutzfeldt-Jakob disease in an adolescent. J Neurol Sci 1981; 52:341.
  6. de Silva R, Findlay C, Awad I, et al. Creutzfeldt-Jakob disease in the elderly. Postgrad Med J 1997; 73:557.
  7. Johnson RT, Gonzalez RG, Frosch MP. Case records of the Massachusetts General Hospital. Case 27-2005. An 80-year-old man with fatigue, unsteady gait, and confusion. N Engl J Med 2005; 353:1042.
  8. Ladogana A, Puopolo M, Poleggi A, et al. High incidence of genetic human transmissible spongiform encephalopathies in Italy. Neurology 2005; 64:1592.
  9. Maddox RA, Holman RC, Belay ED, et al. Creutzfeldt-Jakob disease among American Indians and Alaska Natives in the United States. Neurology 2006; 66:439.
  10. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA 2000; 284:2322.
  11. Wientjens DP, Davanipour Z, Hofman A, et al. Risk factors for Creutzfeldt-Jakob disease: a reanalysis of case-control studies. Neurology 1996; 46:1287.
  12. Collins S, Law MG, Fletcher A, et al. Surgical treatment and risk of sporadic Creutzfeldt-Jakob disease: a case-control study. Lancet 1999; 353:693.
  13. Farmer PM, Kane WC, Hollenberg-Sher J. Incidence of Creutzfeldt-Jakob disease in Brooklyn and Staten Island. N Engl J Med 1978; 298:283.
  14. Will RG, Matthews WB. Evidence for case-to-case transmission of Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry 1982; 45:235.
  15. Collins S, Boyd A, Fletcher A, et al. Creutzfeldt-Jakob disease cluster in an Australian rural city. Ann Neurol 2002; 52:115.
  16. Linsell L, Cousens SN, Smith PG, et al. A case-control study of sporadic Creutzfeldt-Jakob disease in the United Kingdom: analysis of clustering. Neurology 2004; 63:2077.
  17. Klug GM, Wand H, Boyd A, et al. Enhanced geographically restricted surveillance simulates sporadic Creutzfeldt-Jakob disease cluster. Brain 2009; 132:493.
  18. Brown P, Brandel JP, Sato T, et al. Iatrogenic Creutzfeldt-Jakob disease, final assessment. Emerg Infect Dis 2012; 18:901.
  19. Brown P, Preece MA, Will RG. "Friendly fire" in medicine: hormones, homografts, and Creutzfeldt-Jakob disease. Lancet 1992; 340:24.
  20. Frasier SD. The not-so-good old days: working with pituitary growth hormone in North America, 1956 to 1985. J Pediatr 1997; 131:S1.
  21. Lewis AM, Yu M, DeArmond SJ, et al. Human growth hormone-related iatrogenic Creutzfeldt-Jakob disease with abnormal imaging. Arch Neurol 2006; 63:288.
  22. Centers for Disease Control and Prevention (CDC). Creutzfeldt-Jakob disease associated with cadaveric dura mater grafts -- Japan, January 1979-May 1996. MMWR Morb Mortal Wkly Rep 1997; 46:1066.
  23. Centers for Disease Control and Prevention (CDC). Update: Creutzfeldt-Jakob disease associated with cadaveric dura mater grafts--Japan, 1979-2003. MMWR Morb Mortal Wkly Rep 2003; 52:1179.
  24. Preusser M, Ströbel T, Gelpi E, et al. Alzheimer-type neuropathology in a 28 year old patient with iatrogenic Creutzfeldt-Jakob disease after dural grafting. J Neurol Neurosurg Psychiatry 2006; 77:413.
  25. Defebvre L, Destée A, Caron J, et al. Creutzfeldt-Jakob disease after an embolization of intercostal arteries with cadaveric dura mater suggesting a systemic transmission of the prion agent. Neurology 1997; 48:1470.
  26. Allan B, Tuft S. Transmission of Creutzfeldt-Jakob disease in corneal grafts. BMJ 1997; 315:1553.
  27. Heckmann JG, Lang CJ, Petruch F, et al. Transmission of Creutzfeldt-Jakob disease via a corneal transplant. J Neurol Neurosurg Psychiatry 1997; 63:388.
  28. Créange A, Gray F, Cesaro P, et al. Creutzfeldt-Jakob disease after liver transplantation. Ann Neurol 1995; 38:269.
  29. Johnson RT, Gibbs CJ Jr. Creutzfeldt-Jakob disease and related transmissible spongiform encephalopathies. N Engl J Med 1998; 339:1994.
  30. Ward HJ, Everington D, Cousens SN, et al. Risk factors for sporadic Creutzfeldt-Jakob disease. Ann Neurol 2008; 63:347.
  31. Ward HJ, Everington D, Croes EA, et al. Sporadic Creutzfeldt-Jakob disease and surgery: a case-control study using community controls. Neurology 2002; 59:543.
  32. de Pedro-Cuesta J, Mahillo-Fernández I, Rábano A, et al. Nosocomial transmission of sporadic Creutzfeldt-Jakob disease: results from a risk-based assessment of surgical interventions. J Neurol Neurosurg Psychiatry 2011; 82:204.
  33. Rutala WA, Weber DJ, Society for Healthcare Epidemiology of America. Guideline for disinfection and sterilization of prion-contaminated medical instruments. Infect Control Hosp Epidemiol 2010; 31:107.
  34. Heath CA, Barker RA, Esmonde TF, et al. Dura mater-associated Creutzfeldt-Jakob disease: experience from surveillance in the UK. J Neurol Neurosurg Psychiatry 2006; 77:880.
  35. Brown P, Brandel JP, Preece M, Sato T. Iatrogenic Creutzfeldt-Jakob disease: the waning of an era. Neurology 2006; 67:389.
  36. Centers for Disease Control and Prevention (CDC). Update: Creutzfeldt-Jakob disease associated with cadaveric dura mater grafts--Japan, 1978-2008. MMWR Morb Mortal Wkly Rep 2008; 57:1152.
  37. Huillard d'Aignaux J, Costagliola D, Maccario J, et al. Incubation period of Creutzfeldt-Jakob disease in human growth hormone recipients in France. Neurology 1999; 53:1197.
  38. Furtner M, Gelpi E, Kiechl S, et al. Iatrogenic Creutzfeldt-Jakob disease 22 years after human growth hormone therapy: clinical and radiological features. J Neurol Neurosurg Psychiatry 2008; 79:229.
  39. Swerdlow AJ, Higgins CD, Adlard P, et al. Creutzfeldt-Jakob disease in United Kingdom patients treated with human pituitary growth hormone. Neurology 2003; 61:783.
  40. Ricketts MN, Cashman NR, Stratton EE, ElSaadany S. Is Creutzfeldt-Jakob disease transmitted in blood? Emerg Infect Dis 1997; 3:155.
  41. Brown P. Can Creutzfeldt-Jakob disease be transmitted by transfusion? Curr Opin Hematol 1995; 2:472.
  42. Dorsey K, Zou S, Schonberger LB, et al. Lack of evidence of transfusion transmission of Creutzfeldt-Jakob disease in a US surveillance study. Transfusion 2009; 49:977.
  43. Patry D, Curry B, Easton D, et al. Creutzfeldt-Jakob disease (CJD) after blood product transfusion from a donor with CJD. Neurology 1998; 50:1872.
  44. Williamson LM. Leucocyte depletion of the blood supply - how will patients benefit? Br J Haematol 2000; 110:256.
  45. Ludlam CA, Turner ML. Managing the risk of transmission of variant Creutzfeldt Jakob disease by blood products. Br J Haematol 2006; 132:13.
  46. Gregori L, Gurgel PV, Lathrop JT, et al. Reduction in infectivity of endogenous transmissible spongiform encephalopathies present in blood by adsorption to selective affinity resins. Lancet 2006; 368:2226.
  47. Wiltshire M, Thomas S, Scott J, et al. Prion reduction of red blood cells: impact on component quality. Transfusion 2010; 50:970.
  48. Sowemimo-Coker SO, Demczyk CA, Andrade F, Baker CA. Evaluation of removal of prion infectivity from red blood cells with prion reduction filters using a new rapid and highly sensitive cell culture-based infectivity assay. Transfusion 2010; 50:980.
  49. Guntz P, Walter C, Schosseler P, et al. Feasibility study of a screening assay that identifies the abnormal prion protein PrPTSE in plasma: initial results with 20,000 samples. Transfusion 2010; 50:989.
  50. Tarantola A, Abiteboul D, Rachline A. Infection risks following accidental exposure to blood or body fluids in health care workers: a review of pathogens transmitted in published cases. Am J Infect Control 2006; 34:367.
  51. Berger JR, David NJ. Creutzfeldt-Jakob disease in a physician: a review of the disorder in health care workers. Neurology 1993; 43:205.
  52. Matthews WB. CJD in health care workers. Neurology 1993; 43:2421.
  53. Mitrová E, Belay G. Creutzfeldt-Jakob disease in health professionals in Slovakia. Eur J Epidemiol 2000; 16:353.
  54. Hillier CE, Salmon RL. Is there evidence for exogenous risk factors in the aetiology and spread of Creutzfeldt-Jakob disease? QJM 2000; 93:617.
  55. van Duijn CM, Delasnerie-Lauprêtre N, Masullo C, et al. Case-control study of risk factors of Creutzfeldt-Jakob disease in Europe during 1993-95. European Union (EU) Collaborative Study Group of Creutzfeldt-Jakob disease (CJD). Lancet 1998; 351:1081.
  56. Harries-Jones R, Knight R, Will RG, et al. Creutzfeldt-Jakob disease in England and Wales, 1980-1984: a case-control study of potential risk factors. J Neurol Neurosurg Psychiatry 1988; 51:1113.
  57. Ena J. Prions: who should worry about them? Arch Med Res 2005; 36:622.
  58. Taylor DM. Preventing accidental transmission of human transmissible spongifom encephalopathies. Br Med Bull 2003; 66:293.
  59. Blättler T. Implications of prion diseases for neurosurgery. Neurosurg Rev 2002; 25:195.
  60. Yamakawa Y, Hagiwara K, Nohtomi K, et al. Atypical proteinase K-resistant prion protein (PrPres) observed in an apparently healthy 23-month-old Holstein steer. Jpn J Infect Dis 2003; 56:221.
  61. Biacabe AG, Laplanche JL, Ryder S, Baron T. Distinct molecular phenotypes in bovine prion diseases. EMBO Rep 2004; 5:110.
  62. Casalone C, Zanusso G, Acutis P, et al. Identification of a second bovine amyloidotic spongiform encephalopathy: molecular similarities with sporadic Creutzfeldt-Jakob disease. Proc Natl Acad Sci U S A 2004; 101:3065.
  63. Priola SA, Vorberg I. Identification of possible animal origins of prion disease in human beings. Lancet 2004; 363:2013.
  64. Gambetti P, Kong Q, Zou W, et al. Sporadic and familial CJD: classification and characterisation. Br Med Bull 2003; 66:213.
  65. Noguchi-Shinohara M, Hamaguchi T, Kitamoto T, et al. Clinical features and diagnosis of dura mater graft associated Creutzfeldt Jakob disease. Neurology 2007; 69:360.
  66. Haywood AM. Transmissible spongiform encephalopathies. N Engl J Med 1997; 337:1821.
  67. Rabinovici GD, Wang PN, Levin J, et al. First symptom in sporadic Creutzfeldt-Jakob disease. Neurology 2006; 66:286.
  68. Meissner B, Körtner K, Bartl M, et al. Sporadic Creutzfeldt-Jakob disease: magnetic resonance imaging and clinical findings. Neurology 2004; 63:450.
  69. Landolt HP, Glatzel M, Blättler T, et al. Sleep-wake disturbances in sporadic Creutzfeldt-Jakob disease. Neurology 2006; 66:1418.
  70. Will RG. Acquired prion disease: iatrogenic CJD, variant CJD, kuru. Br Med Bull 2003; 66:255.
  71. Boesenberg C, Schulz-Schaeffer WJ, Meissner B, et al. Clinical course in young patients with sporadic Creutzfeldt-Jakob disease. Ann Neurol 2005; 58:533.
  72. Appleby BS, Appleby KK, Crain BJ, et al. Characteristics of established and proposed sporadic Creutzfeldt-Jakob disease variants. Arch Neurol 2009; 66:208.
  73. Kirschbaum WR. Jakob-Creutzfeldt disease. Elsevier, New York 1968.
  74. Parchi P, Castellani R, Capellari S, et al. Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol 1996; 39:767.
  75. Parchi P, Giese A, Capellari S, et al. Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 1999; 46:224.
  76. Lewis V, Hill AF, Klug GM, et al. Australian sporadic CJD analysis supports endogenous determinants of molecular-clinical profiles. Neurology 2005; 65:113.
  77. Collins SJ, Sanchez-Juan P, Masters CL, et al. Determinants of diagnostic investigation sensitivities across the clinical spectrum of sporadic Creutzfeldt-Jakob disease. Brain 2006; 129:2278.
  78. Cooper SA, Murray KL, Heath CA, et al. Sporadic Creutzfeldt-Jakob disease with cerebellar ataxia at onset in the UK. J Neurol Neurosurg Psychiatry 2006; 77:1273.
  79. Krasnianski A, Schulz-Schaeffer WJ, Kallenberg K, et al. Clinical findings and diagnostic tests in the MV2 subtype of sporadic CJD. Brain 2006; 129:2288.
  80. Castellani RJ, Colucci M, Xie Z, et al. Sensitivity of 14-3-3 protein test varies in subtypes of sporadic Creutzfeldt-Jakob disease. Neurology 2004; 63:436.
  81. Sanchez-Juan P, Green A, Ladogana A, et al. CSF tests in the differential diagnosis of Creutzfeldt-Jakob disease. Neurology 2006; 67:637.
  82. Krasnianski A, Meissner B, Schulz-Schaeffer W, et al. Clinical features and diagnosis of the MM2 cortical subtype of sporadic Creutzfeldt-Jakob disease. Arch Neurol 2006; 63:876.
  83. Parchi P, Capellari S, Chin S, et al. A subtype of sporadic prion disease mimicking fatal familial insomnia. Neurology 1999; 52:1757.
  84. Nozaki I, Hamaguchi T, Noguchi-Shinohara M, et al. The MM2-cortical form of sporadic Creutzfeldt-Jakob disease presenting with visual disturbance. Neurology 2006; 67:531.
  85. Meissner B, Westner IM, Kallenberg K, et al. Sporadic Creutzfeldt-Jakob disease: clinical and diagnostic characteristics of the rare VV1 type. Neurology 2005; 65:1544.
  86. Collinge J, Sidle KC, Meads J, et al. Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature 1996; 383:685.
  87. Wadsworth JD, Jackson GS, Hill AF, Collinge J. Molecular biology of prion propagation. Curr Opin Genet Dev 1999; 9:338.
  88. Hill AF, Joiner S, Wadsworth JD, et al. Molecular classification of sporadic Creutzfeldt-Jakob disease. Brain 2003; 126:1333.
  89. Head MW, Bunn TJ, Bishop MT, et al. Prion protein heterogeneity in sporadic but not variant Creutzfeldt-Jakob disease: UK cases 1991-2002. Ann Neurol 2004; 55:851.
  90. Xiang W, Windl O, Westner IM, et al. Cerebral gene expression profiles in sporadic Creutzfeldt-Jakob disease. Ann Neurol 2005; 58:242.
  91. Schoch G, Seeger H, Bogousslavsky J, et al. Analysis of prion strains by PrPSc profiling in sporadic Creutzfeldt-Jakob disease. PLoS Med 2006; 3:e14.
  92. Giaccone G, Di Fede G, Mangieri M, et al. A novel phenotype of sporadic Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry 2007; 78:1379.
  93. Head MW, Ironside JW. Sporadic Creutzfeldt-Jakob disease: discrete subtypes or a spectrum of disease? Brain 2009; 132:2627.
  94. Cali I, Castellani R, Alshekhlee A, et al. Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics. Brain 2009; 132:2643.
  95. Tschampa HJ, Neumann M, Zerr I, et al. Patients with Alzheimer's disease and dementia with Lewy bodies mistaken for Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry 2001; 71:33.
  96. Johnson DY, Dunkelberger DL, Henry M, et al. Sporadic Jakob-Creutzfeldt disease presenting as primary progressive aphasia. JAMA Neurol 2013; 70:254.
  97. Josephs KA, Tsuboi Y, Dickson DW. Creutzfeldt-Jakob disease presenting as progressive supranuclear palsy. Eur J Neurol 2004; 11:343.
  98. Heinemann U, Krasnianski A, Meissner B, et al. Creutzfeldt-Jakob disease in Germany: a prospective 12-year surveillance. Brain 2007; 130:1350.
  99. Josephs KA, Ahlskog JE, Parisi JE, et al. Rapidly progressive neurodegenerative dementias. Arch Neurol 2009; 66:201.
  100. Seipelt M, Zerr I, Nau R, et al. Hashimoto's encephalitis as a differential diagnosis of Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry 1999; 66:172.
  101. Poser S, Mollenhauer B, Kraubeta A, et al. How to improve the clinical diagnosis of Creutzfeldt-Jakob disease. Brain 1999; 122 ( Pt 12):2345.
  102. Slee M, Pretorius P, Ansorge O, et al. Parkinsonism and dementia due to gliomatosis cerebri mimicking sporadic Creutzfeldt-Jakob disease (CJD). J Neurol Neurosurg Psychiatry 2006; 77:283.
  103. Geschwind MD, Shu H, Haman A, et al. Rapidly progressive dementia. Ann Neurol 2008; 64:97.
  104. Geschwind MD, Tan KM, Lennon VA, et al. Voltage-gated potassium channel autoimmunity mimicking creutzfeldt-jakob disease. Arch Neurol 2008; 65:1341.
  105. Chitravas N, Jung RS, Kofskey DM, et al. Treatable neurological disorders misdiagnosed as Creutzfeldt-Jakob disease. Ann Neurol 2011; 70:437.
  106. Weiss D, Brockmann K, Nägele T, et al. Rapid emergence of temporal and pulvinar lesions in MELAS mimicking Creutzfeldt-Jakob disease. Neurology 2011; 77:914.
  107. Paterson RW, Torres-Chae CC, Kuo AL, et al. Differential diagnosis of Jakob-Creutzfeldt disease. Arch Neurol 2012; 69:1578.
  108. Cathala F, Brown P, Castaigne P, Gajdusek DC. [Creutzfeldt-Jacob disease in continental France. Retrospective study from 1968 to 1977]. Rev Neurol (Paris) 1979; 135:439.
  109. Kretzschmar HA, Ironside JW, DeArmond SJ, Tateishi J. Diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Arch Neurol 1996; 53:913.
  110. Brandel JP, Delasnerie-Lauprêtre N, Laplanche JL, et al. Diagnosis of Creutzfeldt-Jakob disease: effect of clinical criteria on incidence estimates. Neurology 2000; 54:1095.
  111. CDC's Diagnostic Criteria for Creutzfeldt-Jakob Disease (CJD), 2010 http://www.cdc.gov/ncidod/dvrd/cjd/diagnostic_criteria.html (Accessed on June 07, 2011).
  112. Safar JG, Geschwind MD, Deering C, et al. Diagnosis of human prion disease. Proc Natl Acad Sci U S A 2005; 102:3501.
  113. Macfarlane RG, Wroe SJ, Collinge J, et al. Neuroimaging findings in human prion disease. J Neurol Neurosurg Psychiatry 2007; 78:664.
  114. Collie DA, Sellar RJ, Zeidler M, et al. MRI of Creutzfeldt-Jakob disease: imaging features and recommended MRI protocol. Clin Radiol 2001; 56:726.
  115. Schröter A, Zerr I, Henkel K, et al. Magnetic resonance imaging in the clinical diagnosis of Creutzfeldt-Jakob disease. Arch Neurol 2000; 57:1751.
  116. de Priester JA, Jansen GH, de Kruijk JR, Wilmink JT. New MRI findings in Creutzfeldt-Jakob disease: high signal in the globus pallidus on T1-weighted images. Neuroradiology 1999; 41:265.
  117. Zeidler M, Sellar RJ, Collie DA, et al. The pulvinar sign on magnetic resonance imaging in variant Creutzfeldt-Jakob disease. Lancet 2000; 355:1412.
  118. Finkenstaedt M, Szudra A, Zerr I, et al. MR imaging of Creutzfeldt-Jakob disease. Radiology 1996; 199:793.
  119. Demaerel P, Heiner L, Robberecht W, et al. Diffusion-weighted MRI in sporadic Creutzfeldt-Jakob disease. Neurology 1999; 52:205.
  120. Shiga Y, Miyazawa K, Sato S, et al. Diffusion-weighted MRI abnormalities as an early diagnostic marker for Creutzfeldt-Jakob disease. Neurology 2004; 63:443.
  121. Young GS, Geschwind MD, Fischbein NJ, et al. Diffusion-weighted and fluid-attenuated inversion recovery imaging in Creutzfeldt-Jakob disease: high sensitivity and specificity for diagnosis. AJNR Am J Neuroradiol 2005; 26:1551.
  122. Kallenberg K, Schulz-Schaeffer WJ, Jastrow U, et al. Creutzfeldt-Jakob disease: comparative analysis of MR imaging sequences. AJNR Am J Neuroradiol 2006; 27:1459.
  123. Ukisu R, Kushihashi T, Kitanosono T, et al. Serial diffusion-weighted MRI of Creutzfeldt-Jakob disease. AJR Am J Roentgenol 2005; 184:560.
  124. Manners DN, Parchi P, Tonon C, et al. Pathologic correlates of diffusion MRI changes in Creutzfeldt-Jakob disease. Neurology 2009; 72:1425.
  125. Bavis J, Reynolds P, Tegeler C, Clark P. Asymmetric neuroimaging in Creutzfeldt-Jakob disease: a ruse. J Neuroimaging 2003; 13:376.
  126. Sibon I, Foubert A, Menegon P, et al. Creutzfeldt-Jakob disease mimicking radiologic posterior reversible leukoencephalopathy. Neurology 2005; 65:329.
  127. Vitali P, Maccagnano E, Caverzasi E, et al. Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other rapid dementias. Neurology 2011; 76:1711.
  128. Meissner B, Kallenberg K, Sanchez-Juan P, et al. MRI lesion profiles in sporadic Creutzfeldt-Jakob disease. Neurology 2009; 72:1994.
  129. Hamaguchi T, Kitamoto T, Sato T, et al. Clinical diagnosis of MM2-type sporadic Creutzfeldt-Jakob disease. Neurology 2005; 64:643.
  130. Tschampa HJ, Kallenberg K, Urbach H, et al. MRI in the diagnosis of sporadic Creutzfeldt-Jakob disease: a study on inter-observer agreement. Brain 2005; 128:2026.
  131. Zerr I, Kallenberg K, Summers DM, et al. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain 2009; 132:2659.
  132. Lee H, Rosenmann H, Chapman J, et al. Thalamo-striatal diffusion reductions precede disease onset in prion mutation carriers. Brain 2009; 132:2680.
  133. Zeidler M, Green A. Advances in diagnosing Creutzfeldt-Jakob disease with MRI and CSF 14-3-3 protein analysis. Neurology 2004; 63:410.
  134. Murata T, Shiga Y, Higano S, et al. Conspicuity and evolution of lesions in Creutzfeldt-Jakob disease at diffusion-weighted imaging. AJNR Am J Neuroradiol 2002; 23:1164.
  135. Schwaninger M, Winter R, Hacke W, et al. Magnetic resonance imaging in Creutzfeldt-Jakob disease: evidence of focal involvement of the cortex. J Neurol Neurosurg Psychiatry 1997; 63:408.
  136. Hayashi R, Hanyu N, Kuwabara T, Moriyama S. Serial computed tomographic and electroencephalographic studies in Creutzfeldt-Jakob disease. Acta Neurol Scand 1992; 85:161.
  137. Itoh Y, Amano T, Shimizu T, et al. Single-photon emission computed tomography image of benzodiazepine receptors in a patient with Creutzfeldt-Jakob disease. Intern Med 1998; 37:896.
  138. Villemagne VL, McLean CA, Reardon K, et al. 11C-PiB PET studies in typical sporadic Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry 2009; 80:998.
  139. Lodi R, Parchi P, Tonon C, et al. Magnetic resonance diagnostic markers in clinically sporadic prion disease: a combined brain magnetic resonance imaging and spectroscopy study. Brain 2009; 132:2669.
  140. Steinhoff BJ, Räcker S, Herrendorf G, et al. Accuracy and reliability of periodic sharp wave complexes in Creutzfeldt-Jakob disease. Arch Neurol 1996; 53:162.
  141. Steinhoff BJ, Zerr I, Glatting M, et al. Diagnostic value of periodic complexes in Creutzfeldt-Jakob disease. Ann Neurol 2004; 56:702.
  142. Bortone E, Bettoni L, Giorgi C, et al. Reliability of EEG in the diagnosis of Creutzfeldt-Jakob disease. Electroencephalogr Clin Neurophysiol 1994; 90:323.
  143. Goto K, Umezaki H, Suetsugu M. Electroencephalographic and clinicopathological studies on Creutzfeldt-Jakob syndrome. J Neurol Neurosurg Psychiatry 1976; 39:931.
  144. NELSON JR, LEFFMAN H. The human diffusely projecting system. Evoked potentials and interactions. Arch Neurol 1963; 8:544.
  145. Brown P, Gibbs CJ Jr, Rodgers-Johnson P, et al. Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease. Ann Neurol 1994; 35:513.
  146. Zerr I, Schulz-Schaeffer WJ, Giese A, et al. Current clinical diagnosis in Creutzfeldt-Jakob disease: identification of uncommon variants. Ann Neurol 2000; 48:323.
  147. Lee RG, Blair RD. Evolution of EEG and visual evoked response changes in Jakob-Creutzfeldt disease. Electroencephalogr Clin Neurophysiol 1973; 35:133.
  148. Hsich G, Kenney K, Gibbs CJ, et al. The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies. N Engl J Med 1996; 335:924.
  149. Zerr I, Bodemer M, Gefeller O, et al. Detection of 14-3-3 protein in the cerebrospinal fluid supports the diagnosis of Creutzfeldt-Jakob disease. Ann Neurol 1998; 43:32.
  150. Kenney, K, Hsich, G, Brechtel, C, Gibbs, CJ,. The cerebrospinal fluid 14-3-3- assay's diagnostic utility among clinically suspected Creutzfeldt-Jakob disease patients: the NIH experience. Neurol 2002; 58(Suppl 3); A250 (abstract).
  151. Geschwind MD, Martindale J, Miller D, et al. Challenging the clinical utility of the 14-3-3 protein for the diagnosis of sporadic Creutzfeldt-Jakob disease. Arch Neurol 2003; 60:813.
  152. Chohan G, Pennington C, Mackenzie JM, et al. The role of cerebrospinal fluid 14-3-3 and other proteins in the diagnosis of sporadic Creutzfeldt-Jakob disease in the UK: a 10-year review. J Neurol Neurosurg Psychiatry 2010; 81:1243.
  153. Muayqil T, Gronseth G, Camicioli R. Evidence-based guideline: diagnostic accuracy of CSF 14-3-3 protein in sporadic Creutzfeldt-Jakob disease: report of the guideline development subcommittee of the American Academy of Neurology. Neurology 2012; 79:1499.
  154. Chapman T, McKeel DW Jr, Morris JC. Misleading results with the 14-3-3 assay for the diagnosis of Creutzfeldt-Jakob disease. Neurology 2000; 55:1396.
  155. Satoh J, Kurohara K, Yukitake M, Kuroda Y. 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. Eur Neurol 1999; 41:216.
  156. Van Everbroeck BR, Boons J, Cras P. 14-3-3 {gamma}-isoform detection distinguishes sporadic Creutzfeldt-Jakob disease from other dementias. J Neurol Neurosurg Psychiatry 2005; 76:100.
  157. Zanusso G, Fiorini M, Farinazzo A, et al. Phosphorylated 14-3-3zeta protein in the CSF of neuroleptic-treated patients. Neurology 2005; 64:1618.
  158. Zerr I, Bodemer M, Otto M, et al. Diagnosis of Creutzfeldt-Jakob disease by two-dimensional gel electrophoresis of cerebrospinal fluid. Lancet 1996; 348:846.
  159. Le Pera M, Urso E, Sprovieri T, et al. Contribution of cerebrospinal fluid thymosin β4 levels to the clinical differentiation of Creutzfeldt-Jakob disease. Arch Neurol 2012; 69:868.
  160. Otto M, Wiltfang J, Cepek L, et al. Tau protein and 14-3-3 protein in the differential diagnosis of Creutzfeldt-Jakob disease. Neurology 2002; 58:192.
  161. Hamlin C, Puoti G, Berri S, et al. A comparison of tau and 14-3-3 protein in the diagnosis of Creutzfeldt-Jakob disease. Neurology 2012; 79:547.
  162. Nilsson-Helander K, Silbernagel KG, Thomeé R, et al. Acute achilles tendon rupture: a randomized, controlled study comparing surgical and nonsurgical treatments using validated outcome measures. Am J Sports Med 2010; 38:2186.
  163. Pan T, Sethi J, Nelsen C, et al. Detection of misfolded prion protein in blood with conformationally sensitive peptides. Transfusion 2007; 47:1418.
  164. McGuire LI, Peden AH, Orrú CD, et al. Real time quaking-induced conversion analysis of cerebrospinal fluid in sporadic Creutzfeldt-Jakob disease. Ann Neurol 2012; 72:278.
  165. Fratini F, Principe S, Puopolo M, et al. Increased levels of acute-phase inflammatory proteins in plasma of patients with sporadic CJD. Neurology 2012; 79:1012.
  166. Tanaka M, Iizuka O, Yuasa T. Hepatic dysfunction in Creutzfeldt-Jakob disease. Neurology 1992; 42:1249.
  167. Shaked GM, Shaked Y, Kariv-Inbal Z, et al. A protease-resistant prion protein isoform is present in urine of animals and humans affected with prion diseases. J Biol Chem 2001; 276:31479.
  168. Head MW, Kouverianou E, Taylor L, et al. Evaluation of urinary PrPSc as a diagnostic test for sporadic, variant, and familial CJD. Neurology 2005; 64:1794.
  169. Zanusso G, Ferrari S, Cardone F, et al. Detection of pathologic prion protein in the olfactory epithelium in sporadic Creutzfeldt-Jakob disease. N Engl J Med 2003; 348:711.
  170. Glatzel M, Abela E, Maissen M, Aguzzi A. Extraneural pathologic prion protein in sporadic Creutzfeldt-Jakob disease. N Engl J Med 2003; 349:1812.
  171. Favereaux A, Quadrio I, Vital C, et al. Pathologic prion protein spreading in the peripheral nervous system of a patient with sporadic Creutzfeldt-Jakob disease. Arch Neurol 2004; 61:747.
  172. Quadrio I, Ugnon-Café S, Dupin M, et al. Rapid diagnosis of human prion disease using streptomycin with tonsil and brain tissues. Lab Invest 2009; 89:406.
  173. Pocchiari M, Puopolo M, Croes EA, et al. Predictors of survival in sporadic Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies. Brain 2004; 127:2348.