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Creutzfeldt-Jakob Disease and its Variants

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Multiple Sclerosis, Mad Cow Disease and Acinetobacter
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Abstract

Creutzfeldt-Jakob disease or spastic pseudosclerosis was a term applied by Jakob to a set of patients first described by Creutzfeldt (1920).

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References

  • Anderson RM, Donnelly CA, Ferguson NM, Woolhouse MEJ, Watt CJ, Udy HJ, Mawhinney S, Dunstan SP, Southwood TRE, Wilesmith JW, Ryan JBM, Hoinville LJ, Hillerton JE, Austin AR, Wells GAH. Transmission dynamics and epidemiology of BSE in British cattle. Nature. 1996;382:779–88.

    Article  CAS  PubMed  Google Scholar 

  • Aoki T, Gibbs CJ, Sotelo J, Gajdusek CD. Heterogeneic autoantibody against neurofilament protein in sera of animals with experimental kuru and Creutzfeldt-Jakob disease and natural scrapie infection. Infect Immun. 1982;38:316–24.

    CAS  PubMed Central  PubMed  Google Scholar 

  • Cousens S, Smith PG, Ward H, Everington D, Knight RS, Zeidler M, Stewart G, Smith-Bathgate EA, Macleod MA, Mackenzie J, Wills RG. Geographical distribution of variant-Creutzfeldt-Jakob disease in Great Britain 1994–2000. Lancet. 2001;357:1002–7.

    Article  CAS  PubMed  Google Scholar 

  • Gajdusek DC, Gibbs CJ, Alpers M. Transmission and passage of experimental kuru to chimpanzees. Science. 1967;155:212–4.

    CAS  PubMed  Google Scholar 

  • Galvez S, Farcas A, Monari M. Cerebrospinal fluid and serum immunoglobulins and C3 in Creutzfeldt-Jakob disease. Neurology. 1979;29:1610–2.

    Article  CAS  PubMed  Google Scholar 

  • Giese A, Brown DR, Groschup MH, Feldmann C, Haist I, Kretschmar HA. Role of microglia in neuronal cell death in prion disease. Brain Pathol. 1998;8:449–57.

    Article  CAS  PubMed  Google Scholar 

  • Jakob Uber eigenartige Erkrankungen des Zentralnervensystems mit Bemerkenswerten anatomischen Befunden. Spastische Pseudosklerose Encephalopathie mit disseminierte Degenerationsherden. Z ges Neurol Psychiater. 1920;64:146.

    Google Scholar 

  • Irani DN. The classic and variant forms of Creutzfeldt-Jakob disease. Semin Clin Neuropsychiatry. 2003;8:71–9.

    Article  PubMed  Google Scholar 

  • Klein MA, Frigg R, Flechsig E, Raeber AJ, Kalinke U, Bluethmann U, Bootz E, Suter M, Zinkernagel RM, Aguzzi A. A crucial role for B cells in neuroinvasive scrapie. Nature. 1987;390:687–90.

    Google Scholar 

  • Kohira I, Tsuji T, Ishizu H, Takao Y, Wake A, Abe K, Kuroda S. Elevation of neuron-specific enolase in serum and cerebrospinal fluid of early stage Creutzfeldt-Jaskob disease. Acta Neurol Scand. 2000;102:385–7.

    Article  CAS  PubMed  Google Scholar 

  • Kovacs GG, Gasque P, Strobel T, Lindeck-Pozza E, Strohschneider M, Ironside JW, Budka H, Guentchen M. Complement activation in human prion disease. Neurobiol Dis. 2004;15:21–8.

    Article  CAS  PubMed  Google Scholar 

  • Lewicki H, Tishon A, Homann D, Mazarquil H, Laval F, Asensio VC, Campbell IL, DeArmond S, Coon B, Teng C, Gairin JE, Oldstone MBA. T cells infiltrate the brain of murine and human transmissible spongiform encephalopathies. J Virol. 2003;77:3799–808.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • Mabbott NA, Bruce ME. The immunobiology of TSE diseases. J Gen Virol. 2001;82:2307–18.

    CAS  PubMed  Google Scholar 

  • Manuelidis L, Yu ZX, Barquero N, Banquero N, Mullins B. Cells infected with scrapie and Creutzfeldt-Jakob disease agents produce intracellular 25 nm virus like particles. Proc Natl Acad Sci USA. 2007;104:1965–70.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • Peden AH, Head MW, Ritchie DL, Bell JE, Ironside JW. Pre-clinical v-CJD after blood transfusion in a PRNP codon heterozygous patient. Lancet. 2004;364:527–9.

    Article  PubMed  Google Scholar 

  • Pugliatti M, Sotgiu S, Rosati G. The worldwide prevalence of multiple sclerosis. Clin Neurol Neurosurg. 2002;104:182–91.

    Article  PubMed  Google Scholar 

  • Scholz R, Lorenzen S. Phantom BSE-Gefahr. Innsbruck: Berenkamp; 2005.

    Google Scholar 

  • Sotelo J, Gibbs CJ, Gajdusek DC. Autoantibodies against axonal neurofilaments in patients with kuru and Creutzfeldt-Jakob disease. Science. 1980;210:190–3.

    Article  CAS  PubMed  Google Scholar 

  • Toh DH, Gibbs CJ, Gajdusek DC, Tutdill DD, Dahl D. The 200 and 150-kDa neurofilaments protein react with IgG autoantibodies from chimpanzees with kuru or Creutzfeldt-Jakob disease; a 62 kDa neurofilament associated protein reacts with sera from sheep with natural scrapie. Proc Natl Acad Sci U S A. 1985;82:3894–6.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • Van Everbroeck B, Dewulf E, Pals P, Lübke U, Martin JJ, Cras P. The role of cytokines, astrocytes, microglia and apoptosis in Creutzfeldt-Jakob disease. Neurobiol Aging. 2002;23:59–64.

    Article  PubMed  Google Scholar 

  • Venters GA. New variant Creutzfeldt-Jakob dfisease: the epidemic that never was. Bit Med J. 2001;323:858–61.

    Article  CAS  Google Scholar 

  • Volkel D, Zimmermann K, Zerr I, Lindner T, Bodemer M, Poser S, Schwarz HS. C-reactive protein and Il-6: new marker proteins for the diagnosis of CJD in plasma? Transfusion. 2001;41:1509–14.

    Article  CAS  PubMed  Google Scholar 

  • Ward HJT, Everington D, Cousens SN, Smith-Bathgate B, Leitch M, Cooper S, et al. Risk factors for variant Creutzfeldt-Jakob disease: a case-control study. Ann Neurol. 2006;59:111–20.

    Article  PubMed  Google Scholar 

  • Wientjens DP, Davanipour Z, Hofman A, Kondo K, Mattlews WB, Will RG, Van Duijn CM. Risk factors for Creutzfeldt-Jakob disease: a re-analysis of case-control studies. Neurology. 1996;46:1287–91.

    Article  CAS  PubMed  Google Scholar 

  • Will RG, Ironside JW, Zeidler M, Cousens SN, Estibeiro K, Alperovitch A, Poser S, Pocchiari M, Hofman A, Smith PG. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet. 1996;347:921–5.

    Article  CAS  PubMed  Google Scholar 

  • Will RG, Alperovitch A, Poser S, Pocchiari M, Hoiffman A, Mitrova E, et al. Descriptiver epidemiology of Creutzfeldt-Jakob disease in six European countries. Ann Neurol. 1998;43:763–7

    Article  CAS  PubMed  Google Scholar 

  • Zerr I, Poser S. Clinical diagnosis and differential diagnosis of CJD and variant-CJD. APMIS. 2002;110:88–98.

    Article  PubMed  Google Scholar 

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Ebringer, A. (2015). Creutzfeldt-Jakob Disease and its Variants. In: Multiple Sclerosis, Mad Cow Disease and Acinetobacter. Springer, Cham. https://doi.org/10.1007/978-3-319-02735-7_16

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