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Neurodegeneration and Prion Disease pp 13–48Cite as

Neuropathology of Transmissible Spongiform Encephalopathies (Prion Diseases)

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References

  1. D. C. Gajdusek, C. J. Gibbs, M. P., and Alpers M. P., Experimental transmission of a kuru-like syndrome to chimpanzees. Nature 209, 794–796 (1966).

    PubMed  Google Scholar 

  2. C. J. Gibbs Jr, D. C. Gajdusek, D. M. Asher, M. P. Alpers, E. Beck, P. M. Daniel, and W. B. Matthews, Creutzfeldt-Jakob disease (spongiform encephalopathy): transmission to chimpanzee. Science 161, 388–389 (1968).

    PubMed  Google Scholar 

  3. C. L. Masters, D. C, Gajdusek, and C. J. Gibbs Jr, Creutzfeldt-Jakob disease virus isolations from the Gerstmann-Sträussler syndrome. With an analysis of the various forms of amyloid plaque deposition in the virus induced spongiform encephalopathies. Brain 104, 559–588 (1981).

    PubMed  Google Scholar 

  4. E. Lugaresi, R. Medori, P. Montagna, A. Baruzzi, P. Cortelli, A. Lugaresi, P. Tinuper, M. Zucconi, and P. Gambetti, Fatal familial insomnia and dysautonomia with selective degeneration of thalamic nuclei. New Engl. J. Med. 315, 997–1004 (1986).

    PubMed  Google Scholar 

  5. D. Burger and G. R. Hartsough, A scrapie-like disease of mink, in: Report of a Scrapie Seminar (United States Department of Agriculture, paper 27, 1964), pp. 225–227.

    Google Scholar 

  6. P. P. Liberski, D. C. Guiroy, E. S. Williams, A. Walis, and H. Budka, Deposition patterns of disease-associated prion protein in captive mule deer brains with chronic wasting disease. Acta Neuropathol. (Berl.) 102, 496–500 (2001).

    Google Scholar 

  7. G. A. H. Wells, A. C. Scott, C. T. Johnson, R. F. Gunning, R. D. Hancock, M. Jeffrey, M. Dawson, and R. Bradley, A novel progressive spongiform encephalopathy in cattle. Vet. Rec. 121, 419–420 (1987).

    PubMed  Google Scholar 

  8. C. Weissmann, A “unified theory” of prion propagation. Nature 352, 679–683 (1991).

    PubMed  Google Scholar 

  9. R. Gabizon, G. Telling, Z. Meiner, M. Halimi, I. Kahana, and S. B. Prusiner, Insoluble wild-type and protease-resistant mutant prion protein in brains of patients with inherited prion diseases. Nature Med., 2, 59–64 (1996).

    PubMed  Google Scholar 

  10. B. Oesch, D. Westaway, M. Walchlii, M. P. McKinley, S. B. H. Kent, R. Aebersold, R. A. Barry, P. Tempst, D. B. Teplow, L. E. Hood, S. B. Prusiner, and C. Weissmann, A cellular gene encodes scrapie PrP 27–30 protein. Cell 40, 735–746 (1985).

    PubMed  Google Scholar 

  11. S. B. Prusiner, Novel proteinaceous infection particles cause scrapie. Science 216, 136–144 (1982).

    PubMed  Google Scholar 

  12. P. P. Liberski, and M. Jaskolski, Prion diseases: a dual view of the prion hypothesis as seen from a distance. Acta Neurobiol Exp. 62, 197–226 (2002).

    Google Scholar 

  13. S. B. Prusiner, M. P. McKinley, K. A. Bowman, D. C. Bolton, P. E. Bendheim, D. C. Groth, and G. G. Glenner, Scrapie prions aggregate to form amyloid-like birefringent rods. Cell 35, 349–358 (1983).

    PubMed  Google Scholar 

  14. J. Collinge, K. C. L. Sidle, J. Meads, J. Ironside, and A. F. Hill, Molecular analysis of prion strain variation and the aetiology of “new variant” CJD. Nature 383, 685–690 (1996).

    PubMed  Google Scholar 

  15. A. F. Hill, M. Desbruslais, S. Joiner, K. C. L. Sidle, I. Gowland, J. Collinge, L. J. Doey, and P. Lantos, The same prion strain causes vCJD and BSE. Nature 389, 448–450 (1997).

    PubMed  Google Scholar 

  16. H. A. Kretzschmar, L. E. Stowring, D. Westaway, W. H. Stubblebine, S. B. Prusiner, and S. J. DeArmond, Molecular cloning of human prion protein cDNA. DNA, 4, 315–324 (1986).

    Google Scholar 

  17. S. Mead, M. P. Stumpf, J. Whitfield, J. A. Beck, M. Poulter, T. Campbell, J. B. Uphill, D. Goldstein, M. Alpers, F. M. Fisher, and J. Collinge, Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics. Science 300, 640–643 (2003).

    PubMed  Google Scholar 

  18. P. M. Daniel, Creutzfeldt-Jakob disease, J. Clin. Pathol. 25, 97–101 (1972).

    Google Scholar 

  19. B. Brownell and D. R. Oppenheimer, An ataxic form of subacute presenile polioencephalopathy (Creutzfeldt-Jakob disease), J. Neurol. Neurosurg. Psychiat. 28, 350–361 (1965).

    PubMed  Google Scholar 

  20. H. Siedler H and N. Malamud, Creutzfeldt-Jakob disease. Clinicopathological report of 15 cases and review of the literature (with special reference to a related disorder designated as subacute spongiform encephalopathy), J. Neuropathol. Exp. Neurol. 22, 381–402 (1963).

    PubMed  Google Scholar 

  21. H. Budka, M. W. Head, J. W. Ironside, P. Gambetti, P. Parchi, M. Zeidler, and F. Tagliavini, Prion Disorders: CJD: Sporadic Creutzfeldt-Jakob disease, in Neurodegeneration: The Molecular Pathology of Dementia and Movement Disorders, edited by D. Dickson (ISN Neuropath Press, Basel, 2003), pp. 287–297.

    Google Scholar 

  22. A. F. Hill, S. Joiner, J. D. F. Wadsworth, K. C. L. Sidle, J. E. Bell, H. Budka, J. W. Ironside, and J. Collinge, Molecular classification of sporadic Creutzfeldt-Jakob disease. Brain 126, 1333–1346 (2003).

    PubMed  Google Scholar 

  23. J. D. F. Wadsworth, A. F. Hill, S. Joiner, G. S. Jackson, A. R. Clarke, J. Collinge, Strain-specific prion-protein conformation determined by metal ions. Nature Cell Biol.1, 55–59 (1999).

    PubMed  Google Scholar 

  24. H. G. Creutzfeldt, Uber eine egenartige herdformige Erkrankung des Zentralnervensystems. Z. Ges. Neurol. Psychiat. 57, 1–18 (1920).

    Google Scholar 

  25. A. Jakob, Uber eigenartige Erkrankungen des Zentralnervensystems mit bemerkenswertem anatomischem Befunde (spastische Pseudosclerose-Encephalopathie mit disseminierten Degenerationsherden). Deutsch Z. Nervenheilk. 70, 132–146 (1921).

    Google Scholar 

  26. A. Jakob, Spastische Pseudosclerose, in: Monographien aus dem Gesamtgebiete der Neurologie und Psychiatrie, Die Ekstrapyramidalen Erkrankungen, Vol. 37, edited by O. Foerster, K. Wilmanns (Julius Springer, Berlin, 1923), pp. 215–245.

    Google Scholar 

  27. C. L. Masters and D. C. Gajdusek, The spectrum of Creutzfeldt-Jakob disease and the virus induced subacute spongiform encephalopathies, in: Recent Advances in Neuropathology, edited by T J. Smith and J. B. Cavanagh (Churchill Livingstone, Edinburgh, 1982), pp. 139–163.

    Google Scholar 

  28. A. M. Salazar, C. L. Masters, D. C. Gajdusek, C. J. Gibbs Jr, Syndromes of amyotrophic lateral sclerosis and dementia: relation to transmissible Creutzfeldt-Jakob disease. Ann. Neurol., 14, 17–26 (1983).

    PubMed  Google Scholar 

  29. H. Yamanouchi, H. Budka, and K. Vass, Unilateral Creutzfeldt-Jakob disease. Neurology 36, 1517–1520 (1986).

    PubMed  Google Scholar 

  30. R. Tinter, P. Brown, T. Hedley-Whyte, E. B. Raaport, C. P. Piccardo, and D. C. Gajdusek, Neuropathologic verification of Creutzfeldt-Jakob disease in the exhumed American recipientof human pituitary growth hormone: epidemiologic and pathogenetic implications. Neurology 36, 932–936 (1986).

    PubMed  Google Scholar 

  31. C. L. Masters and E. P. Richardson, Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease). The nature and progression of spongiform change. Brain 101, 333–344 (1978).

    PubMed  Google Scholar 

  32. M. Jeffrey, J. R. Scott, A. Williams, and H. Fraser, Ultrastructural features of spongiform encephalopathy transmitted to mice from three species of bovidae. Acta Neuropathol. (Berl.) 84, 559–569 (1992).

    Google Scholar 

  33. S. M. Chou, W. N. Payne, C. J. Gibbs Jr., and D. C. Gajdusek, Transmission and scanning electron microscopy of spongiform change in Creutzfeldt-Jakob disease. Brain 103, 885–904 (1980).

    PubMed  Google Scholar 

  34. P. P. Liberski, R. Yanagihara, C. J. Gibbs Jr, and D. C. Gajdusek, White matter ultrastructural pathology of experimental Creutzfeldt-Jakob disease in mice. Acta Neuropathol (Berl.) 79, 1–9 (1989).

    Google Scholar 

  35. P. P. Liberski, R. Kordek, P. Brown, and D. C. Gajdusek, Astrocytes in transmissible spongiform encephalopathies (prion diseases), in: Astrocytes in Brain Aging and Neurodegeneration, edited by H. M. Schipper (R. G. Landes Company, Austin, Texas, USA, 1998) pp. 127–164.

    Google Scholar 

  36. P. P. Liberski, P. Brown, L. Cervenakova, and D. C. Gajdusek, Interactions between astrocytes and oligodendroglia in human and experimental Creutzfeldt-Jakob disease and scrapie. Exp. Neurol. 144, 227–234 (1997).

    PubMed  Google Scholar 

  37. M. Lafarga, M. T. Berciano, I. Suarez, C. F. Viadero, M. A. Andres, and J. Berciano, Cytology and organization of reactive astroglia in human cerebellar cortex with severe loss of granule cells: a study on the ataxicform of Creutzfeldt-Jakob disease. Neuroscience 40, 337–352 (1991).

    PubMed  Google Scholar 

  38. M. Lafarga, M. T. Berciano, I. Suarez, M. A. Andres, and J. Berciano, Reactive astroglia-neuron relationships in the human cerebellar cortex: a quantitative, morphological and immunohistochemical study in Creutzfeldt-Jakob disease. Int. J. Dev. Neurosci. 11, 199–213 (1993).

    PubMed  Google Scholar 

  39. M. Dubois-Dalcq, M. Rodriguez, T. S. Reese, C. J. Gibbs Jr, and D. C. Gajdusek, Search for a specific marker in the neural membranes of scrapie mice. Lab. Invest. 36, 547–553 (1977).

    PubMed  Google Scholar 

  40. H. Budka, A. Aguzzi, P. Brown, J. M. Brucher, O. Bugiani, F. Gullotta, M. Haltia, J. J. Hauw, J. W, Ironside, K. Jellinger, et al., Neuropathological diagnostic criteria for Creutzfeldt-Jakob disease (CJD) and other human spongiform encephalopathies. Brain Pathol., 4, 459–466 (1995).

    Google Scholar 

  41. M. Jeffrey, C. M. Goodsir, N. Fowler, J. Hope, M. E. Bruce, and P. A. McBride, Ultrastructural immuno-localization of synthetic prion protein peptide antibodies in 87V muine scrapie. Neurodegeneration 5, 101–109 (1996).

    PubMed  Google Scholar 

  42. M. Barcikowska, P. P. Liberski, J. Boellaard, P. Brown, D. C. Gajdusek, and H. Budka, Microglia is a component of the prion protein amyloid plaque in the Gerstmann-Sträussler-Scheinkersyndrome. Acta Neuropathol. (Berl.), 85, 623–627 (1993).

    Google Scholar 

  43. M. Jeffrey, C. Goodsir, M. E. Bruce, P. A. McBride, and C. Farquhar, Morhogenesis of amyloid plaque in 87V murine scrapie. Neuropathol. Appl. Neurobiol. 20, 535–542 (1994).

    PubMed  Google Scholar 

  44. J. A. Hainfellner, P. P. Liberski, D. C. Guiroy, L. Cervenakova, P. Brown, D. C. Gajdusek, and H. Budka, Pathology and immunocytochemistry of a kuru brain. Brain Pathol. 7, 547–554 (1997).

    PubMed  Google Scholar 

  45. J. Hainfellner, S. Brantner-Inhaler, L. Cervenakova, P. Brown, T. Kitamoto, J. Tateishi, H. Diringer, P. P. Liberski, H. Regele, M. Feucht, N. Mayr, K. Wessely, K. Summer, F. Seitelberger, and H. Budka, The original Gerstmann-Sträussler-Scheinkerfamily of Austria: divergent clinicopathological phenotypes but constant PrP genotype. Brain Pathol. 5, 201–213 (1995).

    PubMed  Google Scholar 

  46. P. P. Liberski and H. Budka, Ultrastructural pathology of Gerstmann-Sträussler-Scheinkerdisease. Ultrastr. Pathol. 19, 23–36 (1995).

    Google Scholar 

  47. R. G. Will, J. W. Ironside, M. Zeidler, S. N. Cousens, K. Esstibeiro, A. Alperovitch, S. Poser, M. Pocchiari, A. Hofman, and P. G. Smith PG, A newvariantof Creutzfeldt-Jakob disease in the UK. Lancet 347, 921–925 (1996).

    PubMed  Google Scholar 

  48. P. P. Liberski, R. Yanagihara, C. J. Gibbs Jr, and D. C. Gajdusek, Scrapie as a model for neuroaxonal dystrophy: ultrastructural studies. Exp. Neurol. 106, 133–144 (1989).

    PubMed  Google Scholar 

  49. P. P. Liberski, H. Budka, E. Sluga, M. Barcikowska, and H. Kwiecinski, Tubulovesicular structures in Creutzfeldt-Jakob disease. Acta Neuropathol. (Berl.) 84, 238–243 (1992).

    Google Scholar 

  50. P. P. Liberski and H. Budka, Neuroaxonal pathology in Creutzfeldt-Jakob disease. Acta Neuropathol. 97, 329–334 (1999).

    PubMed  Google Scholar 

  51. J. R. Fraser, What is the basis of transmissible spongiform encephalopathy induced neurodegeneration and can it be repaired? Neuropathol. Appl. Neurobiol. 28, 1–11 (2002).

    PubMed  Google Scholar 

  52. M. Jeffrey, I. A. Goodbrand, and A. Goodsir, Pathology of the transmissible spongiform encephalopathies with special emphasis on ultrastructure. Micron 26, 277–298 (1995).

    PubMed  Google Scholar 

  53. D. Jesionek-Kupnicka, J. Buczynski, R. Kordek, T. Sobow, I. Kloszewska, W. Papierz, and P. P. Liberski, Programmed cell death (apoptosis) in Alzheimer’s disease and Creutzfeldt-Jakob disease. Folia Neuropathol. 35, 233–235 (1997).

    PubMed  Google Scholar 

  54. D. Jesionek-Kupnicka, R. Kordek, J. Buczynski, and P. P. Liberski, Apoptosis in relation to neuronal loss in experimental Creutzfeldt-Jakob disease in mice. Acta Neurobiol. Exp. 61, 13–19 (2001).

    Google Scholar 

  55. A. Dorandeu, L. Wingertsmann, F. Chretien, M-B. Delisle, C. Vital, P. Parchi, P. Montagna, E. Lugaresi, J. W. Ironside, H. Budka, P. Gambetti, and F. Gray, Neuronal apoptosis in fatal familial insomnia. Brain Pathol. 8, 531–537 (1998).

    PubMed  Google Scholar 

  56. P. P. Liberski, R. Yanagihara, C. J. Gibbs Jr, and D.C. Gajdusek, Neuronal autophagic vacuoles in experimental scrapie and Creutzfeldt-Jakob disease. Acta Neuropathol. 83, 134–139 (1992).

    PubMed  Google Scholar 

  57. J. W. Boellaard, M. Kao, W. Schlote, and H. Diringer, Neuronal autophagy in experimental scrapie. Acta Neuropathol. 82, 225–228 (1991).

    PubMed  Google Scholar 

  58. J. W. Boellaard, W. Schlote, and J. Tateishi, Neuronal autophagy in experimental Creutzfeldt-Jakob disease. Acta Neuropathol. 78, 410–418 (1989).

    PubMed  Google Scholar 

  59. B. Sikorska, P. P. Liberski, P. Giraud, N. Kopp, and P. Brown, Autophagy is a part of ultrastructural synaptic pathology in Creutzfeldt-Jakob disease: a brain biopsy study. Int. J. Biochem Cell Biol., in press (2004).

    Google Scholar 

  60. S. J. DeArmond, S.-L. Yang, A. Lee, R. Bowler, A. Taraboulos, D. Groth, and S. B. Prusiner, Three scrapie prion isolates exhibit different accumulation patterns of the prion protein scrapie isoform. Proc. Natl. Acad. Sci., USA 90, 6449–6453 (1993).

    Google Scholar 

  61. P. P. Liberski, B. Sikorska, J. Bratosiewicz-Wasik, D. Carleton Gajdusek, and P. Brown, Neuronal cell death in transmissible spongiform encephalopathies (prion diseases) revisited: from apoptosis to autophagy—a review. Int. J. Biochem Cell Biol., in press (2004).

    Google Scholar 

  62. P. P. Liberski, R. Yanagihara, C. J. Gibbs Jr, and D. C. Gajdusek, Tubulovesicular structures in experimental Creutzfeldt-Jakob disease and scrapie. Intervirology 29, 115–120 (1988).

    PubMed  Google Scholar 

  63. P. P. Liberski and H. Budka, Tubulovesicular structures in Gerstmann-Sträussler-Scheinker disease. Acta Neuropathol. (Berl.) 88, 491–492 (1994).

    Google Scholar 

  64. M. Jeffrey, J. R. Fraser, Tubulovesicular particles occur early in the incubation period of murine scrapie. Acta Neuropathol. (Berl.) 99, 525–528 (2000).

    Google Scholar 

  65. P. H. Gibson and L. A. Doughty, An electron microscopic study of inclusion bodies in synaptic terminals of scrapie-infected animals. Acta Neuropathol. (Berl.) 77, 420–425 (1989).

    Google Scholar 

  66. J. F. David-Ferreira, K. L. David-Ferreira, C. J. Gibbs Jr., and J. A. Morris, Scrapie in mice: ultrastructural observations in the cerebral cortex. Proc. Soc. Exp. Biol. Med. 127, 313–320 (1968).

    PubMed  Google Scholar 

  67. A. Bignami and H. B. Parry, Aggregations of 35-nanometer particle associated with neuronal cytopathic changes in natural scrapie. Science 171, 389–390 (1971).

    PubMed  Google Scholar 

  68. P. P. Liberski, N. Streichenberger, P. Giraud, M. Soutrenon, D. Meyronnet, B. Sikorska, and N. Kopp, Ultrastructural pathology of prion diseases revisited: brain biopsy studies. Neuropathol. Appl. Neurobiol. in press (2005).

    Google Scholar 

  69. P. P. Liberski, R. Yanagihara, C. J. Gibbs Jr, and D. C. Gajdusek, Appearance of tubulovesicular structures in Creutzfeldt-Jakob disease and scrapie precedes the onset of clinical disease. Acta Neuropathol. (Berl.) 79, 1–9 (1989).

    Google Scholar 

  70. P. P. Liberski, M. Jeffrey and C. Goodsir, Tubulovesicular structures are not labeled using antibodies to prion protein (PrP) with the immunogold electron microscopy techniques. Acta Neuropathol. (Berl.) 93, 260–264 (1997).

    Google Scholar 

  71. P. P. Liberski, M. Jeffrey, and C. Goodsir, Electron microscopy in prion research: tubulovesicular structures are not composed of prion protein (PrP) but may be intimately associated with PrP amyloid fibrils, in: Prions and brain diseases in animals and humans, edited by D. R. O. Morrison (Plenum Press, New York and London, 1998), pp. 77–86.

    Google Scholar 

  72. A. Tarabulos, K. Jendroska, D. Serban, S.-L. Yang, S. J. DeArmond, and S. B. Prusiner, Regional mapping of prion protein in brain. Proc. Natl. Acad. Sci., USA 89, 7620–7624 (1992).

    Google Scholar 

  73. W. J. Schulz-Schaeffer, S. Tschoke, N. Kranefuss, W. Drose, D. Hause-Reitner, A. Giese, M. H. Groschup, and H. A. Kretzschmar, The paraffin-embedded tissue blot detects PrPSc early in the incubation time in prion diseases. Am. J. Pathol. 156, 51–56 (2000).

    PubMed  Google Scholar 

  74. C. Jarius, G. G. Kovacs, G. Belay, J. A. Hainfellner, E. Mitrova, and H. Budka, Distinctive cerebellar immunoreactivity for the prion protein in familial (E200K) Creutzfedt-Jakob disease. Acta Neuropathol. 105, 449–454 (2003).

    PubMed  Google Scholar 

  75. G. Kovacs, T. Voigtlander, J. A. Hainfellner, and H. Budka, Distribution of intra-neuronal immunoreactivity for the prion protein in human prion diseases. Acta Neuropathol 104, 320–326 (2002).

    PubMed  Google Scholar 

  76. G. G. Kovacs, E. Lindeck-Pozza, L. Chimelli, A. Q. C. Araujo, A. A. Gabbai, T. Strobel, M. Glatzel, A. Aguzzi, and H. Budka, Creutzfeldt-Jakob disease and inclusion body myisitis: abundant disease-associated prion protein in muscle. Ann. Neurol. 55, 121–125 (2004).

    PubMed  Google Scholar 

  77. S. Haik, B. A. Faucheux, V. Sazdovitch, N. Privat, J.-L. Kemeny, A. Perret-Liaudet, and J.-J. Hauw, The sympathetic nervous system is involved in variant Creutzfeldt-Jakob disease. Nature Med. 9, 1121–1123 (2003).

    PubMed  Google Scholar 

  78. H. Budka, Histopathology and immunohistochemistry of human transmissible spongiform encephalopathies (TSEs). Arch. Virol. 16, 135–142 (2000).

    Google Scholar 

  79. D. C. Guiroy, S. K. Shankar, C. J. Gibbs Jr, J. A. Messenheimer, S. Das, and D. C. Gajdusek, Neuronal degeneration and neurfilament accumulation in the trigeminal ganglia in Creutzfeldt-Jakob disease. Ann. Neurol. 25, 102–106 (1989).

    PubMed  Google Scholar 

  80. J. A. Hainfellner and H. Budka, Disease associated prion protein may deposit inthe peripheral nervous system in human transmissible spongiform encephalopathies. Acta Neuropathol. 98, 458–460 (1999).

    PubMed  Google Scholar 

  81. M. H. Groschup, M. Beekes, P. A. McBride, M. Hardt, J. A. Hainfellner, and H. Budka, Deposition of disease-associated prion protein involves the peripheral nervous system in experimental scrapie. Acta Neuropathol. 98, 453–457 (1999).

    PubMed  Google Scholar 

  82. M. Glatzel, E. Abela, M. Maissen, and A. Aguzzi, Extraneural pathologic prion protein in sporadic Creutzfeldt-Jakob disease. N. Engl. J. Med. 349, 1812–1820 (2003).

    PubMed  Google Scholar 

  83. D. C. Gajdusek and V. Zigas, Degenerative disease of the central nervous system in New Guinea. The endemic occurrence of “kuru” in the native population. New Engl. J. Med. 257. 974–978 (1957).

    PubMed  Google Scholar 

  84. I. Klatzo, D. C. Gajusek and V. Zigas, Evaluation of pathological findings in twelve cases of kuru, in: Encephalitides, edited by L. Van Boagert, J. Radermecker, J. Hozay, and A. Lowenthal (Elsevier Publ. Comp, Amsterdam, 1959), pp 172–190.

    Google Scholar 

  85. E. Beck and P. M. Daniel, Kuru and Creutzfeldt-Jakob disease: neuropathological lesions and their significance, in: Slow Transmissible Diseases of the Nervous System, edited by S. B. Prusiner and W. J. Hadlow (Academic Press, New York, 1979), Vol 1, pp. 253–270.

    Google Scholar 

  86. F. Seitelberger, Eigenartige familiar-hereditare Krankheit des Zetralnervensystems in einer niederosterreichischen Sippe. Wien Klin Wochen 74, 687–691 (1962).

    Google Scholar 

  87. M. A. Neuman, D. C. Gajdusek and V. Zigas, Neuropathologic findings in exotic neurologic disorder among natives of the Highlands of New Guinea. J. Neuropathol. Exp. Neurol. 23. 486–507 (1964).

    PubMed  Google Scholar 

  88. C. A. McLean, J. W. Ironside, M. P. Alpers, P. W. Brown, L. Cervenakova, R. M. D. Anderson, and C. L. Masters, Comparative neuropathology of kuru with the new variant of Creutzfeldt-Jakob disease: evidence for strain of agent predominating over genotype of host. Brain Pathol. 8, 428–437 (1998).

    Google Scholar 

  89. P. Piccardo, J. Safar, M. Ceroni, D. C. Gajdusek, C. J. Gibbs Jr, Immunohistochemical localization of prion protein in spongiform encephalopathies and normal brain tissue. Neurology 40, 518–522 (1990).

    PubMed  Google Scholar 

  90. I. A. Goodbrand, J. W. Ironside, D. Nicolson, and J. E. Bell, Prion protein accumulations in the spinal cords of patients with sporadic and growth hormone-associated Creutzfeldt-Jakob disease. Neurosci. Lett. 183, 127–130 (1995).

    PubMed  Google Scholar 

  91. A. von Braunmühl, Uber eine eigenartige hereditaer-familiaere Erkrankung des Zentralnervensystems. Arch. Psychiatr. Z. Neurol. 191, 419–449 (1954).

    Google Scholar 

  92. J. W. Boellaard and W. Schlote, Subakute spongiforme Encephalopathie mit multiformer Plaquebildung. Eigenartige familiar-hereditare Kranknheit des Zentralnervensystems [spino-cerebellare Atrophie mit Demenz, Plaques and plaqueahn-lichen im Klein-and Grosshirn (Gerstmann, Sträussler, Scheinker). Acta Neuropathol. (Berl.) 49, 205–212 (1980).

    Google Scholar 

  93. K. Hsiao, H. F. Baker, T. J. Crow, M. Poulter, F. Owen, J. D. Terwilliger, D. Westaway, J. Ott, and S. B. Prusiner, Linkage of a prion protein missense variantto Gerstmann-Sträusslersyndrome. Nature 338, 342–345 (1989).

    PubMed  Google Scholar 

  94. B. Ghetti, O. Bugiani, F. Tagliavini, and P. Piccardo, Prion disorders: Gerstmann-Sträussler-Scheinkerdisease, in Neurodegeneration: The Molecular Pathology of Dementia and Movement Disorders, edited by D. Dickson (ISN Neuropath Press, Basel, 2003), pp. 318–325.

    Google Scholar 

  95. S. J. DeArmond, J. W. Ironside, E. Bouzamondo-Berstein, D. Peretz, and J. R. Fraser, Neuropathology of prion diseases, in: Prion Biology and Diseases, edited by S. B. Prusiner—2nd ed. (Cold Spring Harbor, New York, 2004), pp. 777–856.

    Google Scholar 

  96. P. P. Liberski, I. Kloszewska, I. Boellaard, W. Papierz, A. Omulecka, and H. Budka, Dystrophic neurites of Alzheimer’s disease and Gerstmann-Sträussler-Scheinker’s disease dissociate from the formation of paired helical filaments. Alzheimer’s Res. 1, 89–93 (1995).

    Google Scholar 

  97. B. Ghetti, S. R. Dlouhy, G. Giaccone, O. Bugiani, B. Frangione, M. R. Farlow, and F. Tagliavini, Gerstmann-Sträussler-Scheinker diseases and the Indiana kindred. Brain Pathol. 5, 61–95 (1995).

    PubMed  Google Scholar 

  98. J. W. Ironside, L. McCardle, A. Horsburgh, Z. Lim, and M. W. Head, Pathological diagnosis of variant Creutzfeldt-Jakob disease. APMIS 11, 79–87 (2002).

    Google Scholar 

  99. P. P. Liberski, J. Ironside, L. McCardle, A. Sherring, Ultrastructural analysis of the florid plaque in variant Creutzfeldt-Jakob disease. Folia Neuropathol. 38, 167–170 (2000).

    PubMed  Google Scholar 

  100. J. G. Fournier, N. Kopp, N. Streichenberger, F. Escaig-Haye, J. Langeveld, and P. Brown, Electron microscopy of brain amyloid plaques from a patient with new variant Creutzfeldt-Jakob disease. Acta Neuropathol. 99, 637–642 (2000).

    PubMed  Google Scholar 

  101. M. Zeidler, R. J. Sellar, D. A. Collie, R. Knight, G. Stewart, M. A. Macleod, J. W. Ironside, S. Cousens, A. C. Colchester, D. M. Hadley, R. G. Will, and A. F. Colchester, The pulvinar sign on magnetic resonance imaging in variant Creutzfeldt-Jakob disease. Lancet 355, 1412–1418 (2000).

    PubMed  Google Scholar 

  102. J. W. Ironside, K. Sutherland, J. E. Bell, L. McCardle, C. Barrie, K. Estebeiro, M. Zeidler, and R. G. Will, A new variant of Creutzfeldt-Jakob disease: neuropathological and clinical features. Cold Spring Harb. Symp. Quant. Biol. 61, 523–530 (1996).

    PubMed  Google Scholar 

  103. R. A. Armstrong, N. J. Cairns, J. W. Ironside, and P. L. Lantos, Quantification of vacuolation (“spongiform change”), surviving neurones and prion protein deposition in eleven cases of variant Creutzfeldt-Jakob disease. Neuropathol. Appl. Neurobiol. 28, 129–135 (2002).

    PubMed  Google Scholar 

  104. R. A. Armstrong, N. J. Cairns, J. W. Ironside, and P. L. Lantos, Quantitative variations in the pathology of 11 cases of variant Creutzfeldt-Jakob disease (vCJD). Pathophysiology B, 235–241 (2002).

    Google Scholar 

  105. R. A. Armstrong, N. J. Cairns, J. W. Ironside, and P. L. Lantos, The spatial patterns of prion protein deposits in cases of variant Creutzfeldt-Jakob disease. Acta Neuropathol. 104, 665–669 (2002).

    PubMed  Google Scholar 

  106. W. H. Nailon and J. W. Ironside, Variant Creutzfeldt-Jakob disease: immunocytochemical studies and image analysis. Microsc. Res. Tech. 50, 2–9 (2000).

    PubMed  Google Scholar 

  107. M. W. Head, V. Northcott, K. Rennison, D. Ritchie, L. McCardle, T. J. Bunn, N. F. McLennan, J. W. Ironside, A. B. Tullo, and R. E. Bonshek, Prion protein accumulation in eyes of patients with sporadic and variant Creutzfeldt-Jakob disease. Invest. Ophthalmol. Vis. Sci. 44, 342–346 (2003).

    PubMed  Google Scholar 

  108. J. W. Ironside, M. W. Head, J. E. Bell, L. McCardle, and R. G. Will, Laboratory diagnosis of variant Creutzfeldt-Jakob disease. Histopathology 37, 1–9 (2000).

    PubMed  Google Scholar 

  109. A. F. Hill, R. J. Butterworth, S. Joiner, G. Jackson, M. N. Rossor, D. J. Thomas, A. Frosh, N. Tolley J. E. Bell, M. Spencer, A. King, S. Al-Sarraj, J. W. Ironside, P. L. Lantos, and J. Collinge, Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples. Lancet 353, 183–189 (1999).

    PubMed  Google Scholar 

  110. S. Shimizu, K. Hoshi, T. Muramoto, M. Homma, J. W. Ironside, S. Kuzuhara, T. Sato, T. Yamamoto, and T. Kitamoto, Creutzfeldt-Jakob disease with florid-type plaques after cadaveric dura mater grafting. Arch. Neurol. 56, 357–363 (1999).

    PubMed  Google Scholar 

  111. P. Parchi, A. Giese, S. Capellari, P. Brown, W. Schulz-Schaeffer, O. Windl, I. Zerr, H. Budka, N. Kopp, P. Piccardo, S. Poser, A. Rojiani, N. Streichemberger, J. Julien, C. Vital, B. Ghetti, P. Gambetti, and H. Kretzschmar, Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann. Neurol. 46, 224–233 (1999).

    PubMed  Google Scholar 

  112. G. Puoti, G. Giaccone, G. Rossi, B. Canciani, O. Bugiani, and F. Tagliavini, Sporadic Creutzfeldt-Jakob disease: co-occurrence of differenttypes of PrP(Sc) in the same brain. Neurology 53, 2173–2176 (1999).

    PubMed  Google Scholar 

  113. C. A. Llewlyn, P. E. Hewitt, R. S. Knight, K. Amar, S. Cousens, J. Mackenzie, R. G. Will. Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion. Lancet 363, 417–421 (2004).

    PubMed  Google Scholar 

  114. M. E. Bruce, R. G. Will, J. W. Ironside, I. McConnell, D. Drummond, A. Suttie, L. McCardle, A. Chree, J. Hope, C. Birkett, S. Cousens, H. Fraser, and C. J. Bostock, Transmissions to mice indicate that “new variant” CJD iscaused by the BSE agent. Nature 389, 498–501 (1997).

    Article  PubMed  Google Scholar 

  115. M. R. Scott, R. Will, J. Ironside, H. O. Nguyen, P. Tremblay, S. J. De Armond, and S. B. Prusiner, Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans. Proc. Natl. Acad. Sci. USA 96, 15137–15142 (1999).

    PubMed  Google Scholar 

  116. S. Notari, S. Capellari, A. Giese, I. Westner, A. Baruzzi, B. Ghetti, P. Gambetti, H. A. Kretzschmar, and P. Parchi. Effects of different experimental conditions on the PrPSc core generated by protease digestion: implications for strain typing and molecular classification of CJD. J. Biol. Chem. 279, 16797–16804 (2004).

    PubMed  Google Scholar 

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  1. Laboratory of Electron Microscopy and Neuropathology, Department of Molecular Pathology and Neuropathology, Medical University, Lodz, Poland

    Pawel P. Liberski

  2. National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Edinburgh, EH4 2XU, UK

    James W. Ironside

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    David R. Brown

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Liberski, P.P., Ironside, J.W. (2005). Neuropathology of Transmissible Spongiform Encephalopathies (Prion Diseases). In: Brown, D.R. (eds) Neurodegeneration and Prion Disease. Springer, Boston, MA. https://doi.org/10.1007/0-387-23923-5_2

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