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Kuru: Introduction to Prion Diseases

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Prion Diseases

Part of the book series: Neuromethods ((NM,volume 129))

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Abstract

Kuru, the first human transmissible spongiform encephalopathy, was transmitted to chimpanzees in the D. Carleton Gajdusek (1923–2008) laboratory. In this review, we briefly summarize the history of this seminal discovery along with its epidemiology, clinical picture, neuropathology, and molecular genetics. The discovery of kuru opened new windows into the realms of human medicine, and was instrumental in the later transmission of Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker disease, as well as the relevance that bovine spongiform encephalopathy had for transmission to humans. The transmission of kuru was one of the greatest contributions to biomedical sciences of the twentieth century.

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References

  1. Asher DM, Gibbs CJ Jr, Sulima MP et al (1993) Transmission of human spongiform encephalopathies to experimental animals: comparison of chimpanzee and Squirrel monkey. In: Brown P (ed) Transmissible spongiform encephalopathies—impact on animal and human health, developments of biological standardization, vol 80. Karger, Basel, pp 9–13

    Google Scholar 

  2. Asher DM (2008) Kuru: memories of the NIH years. Philos Trans R Soc Lond Ser B Biol Sci 363(1510):3618–3625

    Article  Google Scholar 

  3. Gajdusek DC (1962) Kuru: an appraisal of five years of investigation. Eugen Q 9:69–74

    Article  CAS  PubMed  Google Scholar 

  4. Gibbs Jr CJ, Gajdusek DC (1969) Infection as the etiology of spongiform encephalopathy (Creutzfeldt-Jakob disease). Science 165(3897):1023–1025

    Article  CAS  Google Scholar 

  5. Hornabrook RW (1975) Kuru. P N G Med J 4:203–206

    Google Scholar 

  6. Hornabrook RW (1979) Kuru and clinical neurology. In: Prusiner SB, Hadlow WJ (eds) Slow transmissible diseases of the nervous system. Academic Press, New York

    Google Scholar 

  7. Liberski PP, Brown P (2007) Kuru—fifty years later. Neurol Neurochir Pol 41(6):548–556

    PubMed  Google Scholar 

  8. Liberski PP, Brown P (2004) Kuru: a half-opened window onto the landscape of neurodegenerative diseases. Folia Neuropathol 42(Suppl A):3–14

    PubMed  Google Scholar 

  9. Liberski PP, Brown P (2009) Kuru: its ramifications after fifty years. Exp Gerontol 44(1–2):63–69

    Article  CAS  PubMed  Google Scholar 

  10. Liberski PP, Brown P (2006) Prion disease: from ritualistic endocannibalism to cellular endocannibalism—from Kuru to autophagy. In: Karasek M (ed) Aging and age-related disease: the basics. Nova Science Publisher, Inc., New York

    Google Scholar 

  11. Liberski PP, Gajdusek DC (1997) Kuru: fourty years later, a historical note. Brain Pathol 7(1):555–560

    Article  CAS  PubMed  Google Scholar 

  12. Gajdusek DC, Zigas V (1959) Kuru. Clinical, pathological and epidemiological study of an acute progressive degenerative disease of the central nervous system among natives of the Eastern Highlands of New Papua. Am J Med 26(3):442–469

    Article  CAS  PubMed  Google Scholar 

  13. Gajdusek DC, Zigas V (1961) Studies of kuru. I. The ethnologic setting of kuru. Am J Trop Med Hyg 10:80–91

    Article  CAS  PubMed  Google Scholar 

  14. Gajdusek DC, Gibbs Jr CJ, Alpers PM (1967) Transmission and passage of experimental kuru to chimpanzees. Science 155(3759):212–214

    CAS  PubMed  Google Scholar 

  15. Gajdusek DC, Gibbs Jr CJ (1971) Transmission of two subacute spongiform encephalopathies of man (kuru and Creutzfeldt-Jakob disease) to New World monkeys. Nature 230(5296):588–591

    Article  CAS  PubMed  Google Scholar 

  16. Gajdusek DC, Gibbs CJ, Alpers PM (1966) Experimental transmission of a kuru-like syndrome to chimpanzees. Nature 209(5025):794–796

    Article  CAS  PubMed  Google Scholar 

  17. Gajdusek DC, Rogers NG, Basnight M et al (1969) Transmission experiments with kuru in chimpanzees and the isolation of latent viruses from the explanted tissues of affected animals. Ann N Y Acad Sci 162(1):529–550

    Article  CAS  PubMed  Google Scholar 

  18. Gajdusek DC, Zigas V (1957) Degenerative disease of the central nervous system in new Guinea. The endemic occurrence of “kuru” in the native population. N Engl J Med 257(20):974–978

    Article  CAS  PubMed  Google Scholar 

  19. Gibbs Jr CJ, Gajdusek DC, Asher DM et al (1968) Creutzfeldt-Jakob disease (spongiform encephalopathy): transmission to chimpanzee. Science 161(3839):388–389

    Article  CAS  Google Scholar 

  20. Jaskolski M, Liberski PP (2002) Kurt Wuthrich—co-winner of the Nobel Prize in Chemistry, 2002. Acta Neurobiol Exp 62:288–289

    Google Scholar 

  21. Gajdusek DC (2008) Kuru and its contribution to medicine. Philos Trans R Soc Lond Ser B Biol Sci 363(1510):3697–3700

    Article  Google Scholar 

  22. Beyreuther K, Masters CL (1997) βA4-amyloid domain is essential for axonal sorting of APP: implications for Alzheimer’s disease. In: Abstracts of the satellite meeting “Brain Tumors and Alzheimer’s Disease, From Neuropathology to Molecular Biology”, Bali, Indonesia, 3–5 Sept 1997

    Google Scholar 

  23. Gajdusek DC (1996) Infectious amyloids: subacute spongiform encephalopathies as transmissible cerebral amyloidosis. In: Fields BN, Knippe DM, Howley PM (eds) Fields virology, 3rd edn. Lippincott-Raven Publishers, Philadelphia

    Google Scholar 

  24. Gajdusek DC (2001) Molecular casting of infectious amyloids, inorganic and organic replication: nucleation, conformational change and self-assembly. In: Aggeli A, Boden B, Shuguang Z (eds) Self-assembling peptide systems in biology, medicine and engineering. Springer, New York

    Google Scholar 

  25. Ashe KH, Aguzzi A (2013) Prions, prionoids and pathogenic proteins in Alzheimer disease. Prion 7(1):55–59

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Gajdusek DC, Alpers MP, Gibbs CJ Jr (1976) Kuru: epidemiological and virological studies of unique New Guinean disease with wide significance to general medicine. In: Hornabrook RW (ed) Essays on Kuru. E.W. Classey Ltd., Faringdon, Berks

    Google Scholar 

  27. Gajdusek DC, Alpers MP (1972) Genetic studies in relation to kuru. I. Cultural, historical, and demographic background. Am J Hum Genet 24(Suppl):1–38

    Google Scholar 

  28. Gajdusek DC, Alpers MP (1966) Kuru in childhood: disappearance of the disease in the younger age group. 7th Annual Meeting of American Pediatric Society. J Pediatr 69:886–887

    Article  Google Scholar 

  29. Gajdusek DC, Farquhar J (eds) (1981) Kuru. Early letters and field-notes from the collection of D. Carleton Gajdusek. Raven Press, New York

    Google Scholar 

  30. Gajdusek DC, Gibbs CJ (1978) Unconventional viruses causing the spongiform virus encephalopathies. A fruitless search for the coat and core. In: Kurstak E, Maramosch K (eds) Viruses and environment. Academic Press, New York

    Google Scholar 

  31. Gajdusek DC, Reid HL (1961) Studies of kuru. IV: the kuru pattern in Moke, a representative Fore village. Am J Trop Med Hyg 10:628–638

    Article  CAS  PubMed  Google Scholar 

  32. Gajdusek DC (2008) Early images of kuru and the people of Okapa. Philos Trans R Soc Lond Ser B Biol Sci 363(1510):3636–3643

    Article  Google Scholar 

  33. Gajdusek DC (1973) Kuru in the new Guinea highlands. In: Spillane J (ed) Tropical neurology. Oxford University Press, London

    Google Scholar 

  34. Gajdusek DC (1979) Le kuru. Colloque sur les Viruses lents. Organize le 17 Septembre 1978, a Talloires, France. Collection Foundation Merieux. pp 25–57

    Google Scholar 

  35. Gajdusek DC (1979) Observations on the early history of kuru investigation. In: Prusiner SB, Hadlow WJ (eds) Slow transmissible diseases of the nervous system, vol 1. Academic Press, New York, pp 70–36

    Google Scholar 

  36. Gajdusek DC (1967) Slow virus infections of the nervous system. N Engl J Med 276(7):392–400

    Article  CAS  PubMed  Google Scholar 

  37. Gajdusek DC (1977) Unconventional viruses and the origin and disappearance of kuru. In: Les Prix Nobel en 1976. Nobel Foundation, PA Norstedt & Soner, Stockholm, pp 167–216

    Google Scholar 

  38. Gajdusek DC (1977) Unconventional viruses and the origin and disappearance of kuru. Science 197(4307):943–960

    Article  CAS  PubMed  Google Scholar 

  39. Gajdusek DC (1977) Urgent opportunistic observations: the study of changing, transient and disappearing phenomena of medical interest in disrupted primitive human communities. In: Health and disease in tribal societies. Elsevier, Amsterdam, pp 69–102

    Google Scholar 

  40. Zigas V, Gajdusek DC (1957) Kuru. Clinical study of a new syndrome resembling paralysis agitans in natives of the eastern Highlands of Australian New Guinea. Med J Aust 44(21):745–754

    CAS  PubMed  Google Scholar 

  41. Zigas V, Gajdusek DC (1959) Kuru. Clinical, pathological and epidemiological study of a recently discovered acute progressive degenerative disease of the central nervous system reaching “epidemic” proportions among natives of the Eastern Highlands of New Guinea. P N G Med J 3:1–31

    Google Scholar 

  42. Mathews JD (2008) The changing face of kuru: a personal perspective. Philos Trans R Soc Lond Ser B Biol Sci 363(1510):3679–3684

    Article  Google Scholar 

  43. Mathews JD (1967) The epidemiology of kuru. P N G Med J 10:76–82

    Google Scholar 

  44. Lindenbaum S (2009) Cannibalism, kuru and anthropology. Folia Neuropathol 47(2):138–144

    PubMed  Google Scholar 

  45. Mathews JD (1971) Kuru. A puzzle in cultural and environmental medicine. Thesis submitted to the University of Melbourne towards the Degree of Doctor of Medicine. Melbourne

    Google Scholar 

  46. Bennett JH, Rhodes FA, Robson HN (1959) A possible genetic basis for kuru. Am J Hum Genet 11(2, part 1):169–187

    CAS  PubMed  PubMed Central  Google Scholar 

  47. Bennett JH, Rhodes FA, Robson HN (1958) Observations on kuru. I. A possible genetic basis. Australas Ann Med 7(4):269–275

    CAS  PubMed  Google Scholar 

  48. Alpers MP (1968) Kuru: implications of its transmissibility for the interpretation of its changing epidemiologic pattern. In: Bailey OT, Smith DE (eds) The central nervous system, International Academy of Pathology Monograph No. 9. Williams & Wilkins Company, Baltimore, pp 234–250

    Google Scholar 

  49. Lindenbaum S (2013) Kuru Sorcery. Disease and danger in the New Guinea Highlands, 2nd edn. Paradigm Publishers, Boulder, p 224

    Google Scholar 

  50. Lindenbaum S (2008) Understanding kuru: the contribution of anthropology and medicine. Philos Trans R Soc Lond Ser B Biol Sci 363(1510):3715–3720

    Article  Google Scholar 

  51. Lindenbaum S (1998) Images of catastrophe: the making of an epidemic. In: Singer M (ed) The political economy of AIDS. Baywood Publishing Co Inc, Amityville

    Google Scholar 

  52. Lindenbaum S (2001) Kuru, prions, and human affairs. Annu Rev Anthropol 30:363–385

    Article  Google Scholar 

  53. Lindenbaum S (2004) Thinking about cannibalism. Annu Rev Anthropol 33:475–498

    Article  Google Scholar 

  54. Matthews JD, Glasse RM, Lindenbaum S (1968) Kuru and cannibalism. Lancet 2(7565):449–452

    Article  Google Scholar 

  55. Glasse R, Lindenbaum S (1993) Fieldwork in the South Fore: the process of ethnographic inquiry. In: Prusiner SB, Collinge J, Powell J, Anderton B (eds) Prion diseases of humans and animals. Ellis Horwood, New York

    Google Scholar 

  56. Glasse R (1967) Cannibalism in the Kuru region of New Guinea. Trans N Y Acad Sci 29(6):748–754

    Article  CAS  PubMed  Google Scholar 

  57. Glasse, R. (1962) Fieldwork report to the Department of Public Health. South Fore cannibalism and kuru. Reprinted by the National Institutes of Health

    Google Scholar 

  58. Glasse, R. (1963) Fieldwork Report to the Department of Public Health. The spread of kuru in the kuru region. Reprinted by the National Institutes of Health

    Google Scholar 

  59. Arens W (1979) The man-eating myth: anthropology and anthropophagy. Oxford University Press, Oxford

    Google Scholar 

  60. Zigas V (1979) Origin of investigations on slow virus infections in man. In: Prusiner SB, Hadlow WJ (eds) Slow transmissible diseases of the nervous system, vol 1. Academic Press, New York, pp 3–6

    Google Scholar 

  61. Hamilton L, Gajdusek DC (1969) Nutrition in the kuru region. II. A nutritional evaluation of tradition Fore diet in Moke village in 1957. Acta Trop 26(4):331–345

    Google Scholar 

  62. Sorenson ER, Gajdusek DC (1969) Nutrition in the kuru region. I. Gardening, food handling, and diet of the fore people. Acta Trop 26:281–330

    CAS  PubMed  Google Scholar 

  63. Sorenson ER, Gajdusek DC (1966) The study of child growth and development in primitive cultures. A research archive for ethnopediatric film investigations of styles in the patterning of the nervous system. Pediatrics 37(suppl):149–243

    Google Scholar 

  64. Curtain CC, Gajdusek DC, Zigas V (1961) Studies on kuru. II. Serum proteins in natives from the kuru region of New Guinea. Am J Trop Med Hyg 10:92–109

    Article  CAS  PubMed  Google Scholar 

  65. Kitchin D, Bearn AG, Alpers MP, Gajdusek DC (1972) Genetic studies in relation to kuru. III. Distribution of the inherited serum group-specific protein (Gc) phenotypes in New Guineans: an association of kuru and the Gc Ab phenotype. Am J Hum Genet 24(6 Pt 2):S72–S85

    PubMed Central  Google Scholar 

  66. Mbaginta’o IG (1976) Medical practices and funeral ceremony of the Dunkwi Anga. J Soc Océan 32(53):299–305

    Article  Google Scholar 

  67. Plato CC, Gajdusek DC (1972) Genetic studies in relation to kuru. IV. Dermatoglyphics of the Fore and Anga populations of the Eastern Highlands of New Guinea. Am J Hum Genet 24:S86–S93

    PubMed Central  Google Scholar 

  68. Simmons RT, Gajdusek DC (1960) Blood group genetical studies on kuru-afflicted natives of the eastern Highlands of New Guimea, and comparison with unaffected neighboring tribes in Papua New Guinea. In: Proceedings of the 8th Congress of International Society of Blood Transfusions, Tokyo, pp 255–259

    Google Scholar 

  69. Simmons RT, Graydon JJ, Gajdusek DC et al (1972) Genetic studies in relation to kuru. II. Blood-group genetic patterns and populations of the eastern Highlands of New Guinea. Am J Hum Genet 24:S39–S71

    PubMed Central  Google Scholar 

  70. Simmons RT, Graydon JJ, Zigas V et al (1961) Studies on kuru. V. A blood group genetical survey of the kuru region and other parts of Papua New Guinea. Am J Trop Med Hyg 10:639–664

    Article  Google Scholar 

  71. Simmons RT, Graydon JJ, Zigas V et al (1961) Studies on kuru. VI. Blood groups in kuru. Am J Trop Med Hyg 10:665–668

    Article  Google Scholar 

  72. Wiesenfeld SL, Gajdusek DC (1975) Genetic studies in relation to kuru. VI. Evaluation of increased liability to kuru in Gc, Ab-Ab indyviduals. Am J Hum Genet 27:498–504

    CAS  PubMed  PubMed Central  Google Scholar 

  73. Gajdusek DC (1993) Kuru and scrapie. In: Prusiner SB, Collinge J, Powell J, Anderton B (eds) Prion diseases of humans and animals. Ellis Horwood, New York

    Google Scholar 

  74. Hadlow WJ (2008) Kuru likened to scrapie: the story remembered. Philos Trans R Soc Lond Ser B Biol Sci 363(1510):3644

    Article  Google Scholar 

  75. Hadlow WJ (1995) Neuropathology and the scrapie-kuru connection. Brain Pathol 5(1):27–31

    Article  CAS  PubMed  Google Scholar 

  76. Hadlow WJ (1959) Scrapie and kuru. Lancet 2:289–290

    Article  Google Scholar 

  77. Hadlow WJ (1993) The Scrapie-kuru connection: recollections of how it came about. In: Prusiner SB, Collinge J, Powell J, Anderton B (eds) Prion diseases of humans and animals. Ellis Horwood, New York

    Google Scholar 

  78. Collinge CJ, Whitfield J, McKintosch E et al (2008) A clinical study of kuru patients with long incubation periods at the end of the epidemic in Papua New Guinea. Philos Trans R Soc Lond Ser B Biol Sci 363(1510):3725–3739

    Article  Google Scholar 

  79. Besnoit C (1899) La tremblante ou nevrite peripherique enzootique du mouton. Rev Vet (Tolouse) 24:265–277

    Google Scholar 

  80. M’Gowan JP (1914) Investigation into the disease of sheep called “scrapie” (Traberkrankheit, La tremblante). With especial reference to its association with Sarcosporidiosis. William Blackwood and Sons, Edinburgh

    Google Scholar 

  81. Innes JMR, Saunders LZ (1962) Comparative neuropathology. Academic Press, New York

    Google Scholar 

  82. Goldfarb LG, Cervenakova L, Gajdusek DC (2004) Genetic studies in relation to kuru: an overview. Curr Mol Med 4(4):375–384

    Article  CAS  PubMed  Google Scholar 

  83. Zlotnik I (1957) Significance of vacuolated neurones in the medulla of sheep infected with scrapie. Nature 180(4582):393–394

    Article  CAS  PubMed  Google Scholar 

  84. Zlotnik I (1957) Vacuolated neurons in sheep affected with scrapie. Nature 179(4562):737

    Article  CAS  PubMed  Google Scholar 

  85. Gajdusek DC, Gibbs CJ (1964) Attempts to demonstrate a transmissible agent in kuru, amyotrophic lateral sclerosis, and other sub-acute and chronic system degenerations of man. Nature 204:257–259

    Article  CAS  PubMed  Google Scholar 

  86. Carrell RW, Lomas DA (1997) Conformational disease. Lancet 350(9071):134–138

    Article  CAS  PubMed  Google Scholar 

  87. Gajdusek DC (1988) Transmissible and nontransmissible dementias: distinction between primary cause and pathogenetic mechanisms in Alzheimer’s disease and aging. Mt Sinai J Med 55(1):3–5

    CAS  PubMed  Google Scholar 

  88. Gibbs CJ Jr, Gajdusek DC (1965) Attempts to demonstrate a transmissible agent in kuru, amyotrophic lateral sclerosis, and other subacute and chronic progressive nervous system degenerations of man. In: Gajdusek DC, Gibbs CJ Jr, Alpers M (eds) Slow, latent, and temperate virus infections, NINDB Monograph No. 2. US Department of Health, Education, and Welfare, Washington, DC

    Google Scholar 

  89. Alpers MP, Gajdusek DC (1965) Changing pattern of kuru: epidemiological changes in the period of increasing contact of the Fore people with western civilization. Am J Trop Med Hyg 14(5):852–879

    Article  CAS  PubMed  Google Scholar 

  90. Alpers MP (1979) Epidemiology and ecology of kuru. In: Prusiner SB, Hadlow WJ (eds) Slow transmissible diseases of the nervous system, vol 1. Academic Press, New York, pp 67–90

    Google Scholar 

  91. Alpers MP (2008) The epidemiology of kuru: monitoring the epidemic from its peak to the end. Philos Trans R Soc Lond Ser B Biol Sci 363(1510):3707–3713

    Article  Google Scholar 

  92. Gajdusek DC (1996) Kuru in childhood: implications for the problem of whether bovine spongiform encephalopathy affects humans. In: Court L, Dodet B (eds) Transmissible subacute spongiform encephalopathies: prion diseases. Elsevier, Amsterdam

    Google Scholar 

  93. Goldfarb LG (2002) Kuru: the old epidemic in a new mirror. Microbes Infect 4(8):875–882

    Article  PubMed  Google Scholar 

  94. Gajdusek DC, Zigas V, Baker J (1961) Studies on kuru. III. Patterns of kuru incidence: demographic and geographic epidemiological analysis. Am J Trop Med Hyg 10:599–627

    Article  CAS  PubMed  Google Scholar 

  95. Mathews JD (1967) A transmission model for kuru. Lancet 1(7494):821–825

    Article  CAS  PubMed  Google Scholar 

  96. Matthews JD (1976) Kuru as an epidemic disease. In: Hornabrook RW (ed) Essays on kuru, Monographs no 3. Papua New Guinea Institute of Medical Research, E. W. Glassey, Faringdon

    Google Scholar 

  97. Alpers MP (2008) Some tributes to research colleagues and other contributors to our knowledge about kuru. Philos Trans R Soc Lond Ser B Biol Sci 363(1510):3614–3617

    Article  Google Scholar 

  98. Klitzman RL, Alpers MP, Gajdusek DC (1984) The natural incubation period of kuru and the episodes of transmission in three clusters of patients. Neuroepidemiology 3:3–20

    Article  Google Scholar 

  99. Cervenakova L, Goldfarb LG, Garruto R et al (1998) Phenotype-genotype studies in kuru: implications for new variant Creutzfeldt-Jakob disease. Proc Natl Acad Sci U S A 95(22):13239–13241

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  100. Brown P, Gibbs CJ, Rodgers-Johnson P et al (1994) Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease. Ann Neurol 35(5):513–529

    Article  CAS  PubMed  Google Scholar 

  101. Gibbs Jr CJ, Amyx HL, Bacote A et al (1980) Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates. J Infect Dis 142(2):205–208

    Article  CAS  Google Scholar 

  102. Gibbs Jr CJ, Gajdusek DC, Amyx H (1979) Strain variation in the viruses of Creutzfeldt-Jakob disease and kuru. In: Prusiner SB, Hadlow WJ (eds) Slow transmissible diseases of the nervous system, vol 1. Academic Press, New York, pp 87–110

    Google Scholar 

  103. Gibbs CJ Jr, Gajdusek DC (1979) Transmission and characterization of the agents of spongiform virus encephalopathies: kuru, Creutzfeldt-Jakob disease, scrapie and mink encephalopathy. In: Immunological disorders of the nervous system, Res Publ ARNMD, vol 49, pp 383–410

    Google Scholar 

  104. Gibbs CJ (1993) Spongiform encephalopathies—slow, latent, and temperate virus infections—in retrospect. In: Prusiner SB, Collinge J, Poqwell J, Anderon B (eds) Prion diseases of humans and animals. Ellis Horwood, New York

    Google Scholar 

  105. Liberski PP (1981) Wirusy powolne układu nerwowego człowieka i zwierząt. Część I. Kuru. (Slow viruses of the nervous system of man and animals. part I. Kuru). Postepy Hig Med Dosw 35:471–493

    CAS  PubMed  Google Scholar 

  106. Gajdusek DC, Gibbs Jr CJ (1972) Transmission of kuru from man to Rhesus monkeys (Macaca mulatto) 8.1/2 years after inoculation. Nature 240(5380):351

    Article  CAS  PubMed  Google Scholar 

  107. Peterson DA, Wolfe LG, Deinhardt F et al (1974) Transmission of kuru and Creutzfeldt-Jakob disease to Marmoset monkeys. Intervirology 2(1):14–19

    Article  CAS  PubMed  Google Scholar 

  108. Masters CL, Alpers MP, Gajdusek DC et al (1976) Experimental kuru in the gibbon and Sooty mangabey and Creutzfeldt-Jakob disease in the pigtailed macaque. J Med Primatol 5(4):205–209

    CAS  PubMed  Google Scholar 

  109. Beck E, Daniel MP (1979) Kuru and Creutzfeldt-Jakob disease: neuropathological lesions and their significance. In: Prusiner SB, Hadlow WJ (eds) Slow transmissible diseases of the nervous system, vol 1. Academic Press, New York, pp 253–270

    Google Scholar 

  110. Beck E, Daniel MP, Alpers MP et al (1969) Neuropathological comparisons of experimental kuru in Chimpanzees with human kuru. With a note on its relation to scrapie and spongiform encephalopathy. In Burdzy K, Kallos P (eds) Pathogenesis and etiology of demyelinating diseases. Proceedings of the Workshop on Contributions to the Pathogenesis and Etiology of Demylinating Conditions, Locarno, Switzerland, May 31–June 3, 1967. Additamentum to Int Arch Allergy Appl Immunol, vol 36. pp 553–562

    Google Scholar 

  111. Beck E, Daniel MP, Alpers MP et al (1966) Experimental kuru in chimpanzees. A pathological report. Lancet 2(7472):1056–1059

    Article  CAS  PubMed  Google Scholar 

  112. Beck E, Daniel MP, Asher MP et al (1973) Experimental kuru in chimpanzees. A neuropathological study. Brain 96(3):441–462

    Article  CAS  PubMed  Google Scholar 

  113. Beck E, Daniel MP, Gajdusek DC (1965) A comparison between the neuropathological changes in kuru and scrapie, system degeneration. In: Proceedings of the VIth International Congress Neuropathological, Zurich, pp 213–218

    Google Scholar 

  114. Beck E, Daniel MP (1965) Kuru and scrapie compared: are they examples of system degeneration? In: Gajdusek DC, Gibbs CJ Jr, Alpers MP (eds) Slow, latent, and temperate virus infections. US Department of Health, Education, Welfare, Washington, DC

    Google Scholar 

  115. Beck E, Daniel MP (1993) Prion diseases from a neuropathologist’s perspective. In: Prusienr SB, Collinge J, Powell J, Anderton B (eds) Prion diseases of humans and animals. Ellis Horwood, New York

    Google Scholar 

  116. Beck E, Bak J, Christ JF et al (1975) Experimental kuru in the Spider monkey. Histopathological and Ultrastructural studies of the brain during early stages of incubation. Brain 98(4):595–612

    Article  CAS  PubMed  Google Scholar 

  117. Beasley A (2006) The promised medicine: fore reflections on the scientific investigation of kuru. Oceania 76:186–202

    Article  Google Scholar 

  118. Alpers MP (1964) Kuru: age and duration studies. Mimeographed. Department of Medicine, University of Adelaide, Adelaide

    Google Scholar 

  119. Beasley A (2009) Frontier journals. Fore experiences on the kuru patrols. Oceania 79:34–52

    Article  Google Scholar 

  120. Beasley A (2004) Frontier science: the early investigation of kuru in Papua New Guinea. In: Dew K, Fitzgerald R (eds) Challenging science: issues in New Zealand. Dunmore Press, Palmerston North

    Google Scholar 

  121. Beasley A (2006) Kuru truths: obtaining Fore narratives. Field Methods 18:21–42

    Article  Google Scholar 

  122. Beasley A (2008) Richard Hornabrook’s first impressions of kuru and Okapa. Philos Trans R Soc Lond Ser B Biol Sci 363(1510):3626–3627

    Article  Google Scholar 

  123. Collinge CJ, Whitfield J, McKintosch E et al (2006) Kuru in the 21st century—an acquired human prion disease with very long incubation periods. Lancet 367(9528):2068–2074

    Article  PubMed  Google Scholar 

  124. Collinge J (2008) Lessons of kuru research: background to recent studies with some personal reflections. Philos Trans R Soc Lond Ser B Biol Sci 363(1510):3689–3696

    Article  Google Scholar 

  125. Gajdusek DC, Sorenson ER, Meyer J (1970) A comprehensive cinema record of disappearing kuru. Brain 93(1):65–76

    Article  CAS  PubMed  Google Scholar 

  126. Prusiner SB, Gajdusek DC, Alpers MP (1982) Kuru with incubation periods exceeding two decades. Ann Neurol 12(1):1–9

    Article  CAS  PubMed  Google Scholar 

  127. Alpers MP (1964) Kuru: a clinical study, Mimeographed. US Department of Health, Education, Welfare, Washington, DC

    Google Scholar 

  128. Klatzo I, Gajusek DC, Zigas V (1959) Evaluation of pathological findings in twelve cases of kuru. In: Van Boagert L, Radermecker J, Hozay J, Lowenthal A (eds) Encephalitides. Elsevier, Amsterdam

    Google Scholar 

  129. Klatzo I, Gajdusek DC (1959) Pathology of kuru. Lab Investig 8(4):799–847

    CAS  PubMed  Google Scholar 

  130. Biernat W, Liberski PP, Guiroy DC et al (1995) Proliferating cell nuclear antigen immunohistochemistry in astrocytes in experimental Creutzfeldt-Jakob disease and in human kuru, Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker syndrome. Neurodegeneration 4(2):195–201

    Article  CAS  PubMed  Google Scholar 

  131. Fowler M, Robertson EG (1959) Observations on kuru. III: pathological features in five cases. Australas Ann Med 8(1):16–26

    CAS  PubMed  Google Scholar 

  132. Kakulas BA, Lecours A-R, Gajdusek DC (1967) Further observations on the pathology of kuru. J Neuropathol Exp Neurol 26(1):85–97

    Article  CAS  PubMed  Google Scholar 

  133. Piccardo P, Safar J, Ceroni M et al (1990) Immunohistochemical localization of prion protein in spongiform encephalopathies and normal brain tissue. Neurology 40(3):518–522

    Article  CAS  PubMed  Google Scholar 

  134. Scrimgeour EM, Masters LC, Alpers MP et al (1983) A clinico-pathological study of case of kuru. J Neurol Sci 59(2):265–275

    Article  CAS  PubMed  Google Scholar 

  135. Scrimgeou EM (2008) Some recollections about kuru in a patient at Rabaul in 1978, and subsequent experiences with prion diseases. Philos Trans R Soc Lond Ser B Biol Sci 363(1510):3663–3664

    Article  Google Scholar 

  136. Sikorska B, Liberski PP, Sobów T et al (2009) Ultrastructural study of florid plaques in variant Creutzfeldt-Jakob disease: a comparison with amyloid plaques in kuru, sporadic Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker disease. Neuropathol Appl Neurobiol 35(1):46–59

    Article  CAS  PubMed  Google Scholar 

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

    CAS  Google Scholar 

  138. Neuman MA, Gajdusek DC, Zigas V (1964) Neuropathologic findings in exotic neurologic disorder among natives of the Highlands of New Guinea. J Neuropathol Exp Neurol 23:486–507

    Article  Google Scholar 

  139. Masters CL, Gajdusek DC, Gibbs Jr CJ (1981) 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(3):559–588

    Article  CAS  PubMed  Google Scholar 

  140. Liberski PP, Sikorska B, Lindenbaum S et al (2012) Kuru: genes, cannibals and neuropathology. J Neuropathol Exp Neurol 71(2):92–103

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  141. Hainfellner J, Liberski PP, Guiroy DC et al (1997) Pathology and immunohistochemistry of a kuru brain. Brain Pathol 7(1):547–554

    Article  CAS  PubMed  Google Scholar 

  142. McLean CA (2008) The neuropathology of kuru and variant Creutzfeldt-Jakob disease. Philos Trans R Soc Lond Ser B Biol Sci 363(1510):3685–3687

    Article  Google Scholar 

  143. McLean CA, Ironside JW, Alpers MP et al (1998) 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(3):428–437

    Google Scholar 

  144. Peat A, Field EJ (1970) An unusual structure in kuru brain. Acta Neuropathol (Berl) 15(3):288–292

    Article  CAS  Google Scholar 

  145. Liberski PP (1988) The occurrence of cytoplasmic lamellar bodies in scrapie infected and normal hamster brains. Neuropatol Pol 26:79–85

    CAS  PubMed  Google Scholar 

  146. Field EJ, Mathews JD, Raine CS (1969) Electron microscopic observations on the cerebellar cortex in kuru. J Neurol Sci 8(2):209–224

    Article  CAS  PubMed  Google Scholar 

  147. Liberski PP, Yanagihara R, Gibbs Jr CJ, Gajdusek DC (1990) Re-evaluation of experimental Creutzfeldt-Jakob disease: serial studies of the Fujisaki strain of Creutzfeldt-Jakob disease virus in mice. Brain 113:121–137

    Article  PubMed  Google Scholar 

  148. Lampert PW, Earle KM, Gibbs Jr CJ, Gajdusek DC (1969) Experimental kuru encephalopathy in chimpanzees and spider monkey. J Neuropathol Exp Neurol 28(3):353–370

    Article  CAS  PubMed  Google Scholar 

  149. Goodbrand IA, Ironside JW, Nicolson D, Bell JE (1995) Prion protein accumulations in the spinal cords of patients with sporadic and growth hormone-associated Creutzfeldt-Jakob disease. Neurosci Lett 183(1–2):127–130

    Article  CAS  PubMed  Google Scholar 

  150. Brandner S, Whitfield J, Boone K et al (2008) Central and peripheral pathology of kuru: pathological analysis of a recent case and comparison with other forms of human prion diseases. Philos Trans R Soc Lond Ser B Biol Sci 363(1510):3755–3763

    Article  Google Scholar 

  151. Collinge J, Sidle KCL, Meads J et al (1996) Molecular analysis of prion strain variation and the etiology of “new variant” CJD. Nature 383(6602):685–670

    Article  CAS  PubMed  Google Scholar 

  152. Parchi P, Castellani R, Capellari S et al (1996) Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol 39(6):767–778

    Article  CAS  PubMed  Google Scholar 

  153. Wadsworth JD, Joiner S, Hill AF et al (2001) Tissue distribution of protease resistant prion protein in variant Creutzfeldt-Jakob disease using a highly sensitive immunoblotting assay. Lancet 358(9277):171–180

    Article  CAS  PubMed  Google Scholar 

  154. Lee H-S, Brown P, Cervenakova L et al (2001) Increased susceptibility to kuru of carriers of the PRNP 129 methionine/methionine genotype. J Infect Dis 183(2):192–196

    Article  CAS  PubMed  Google Scholar 

  155. Mead S, Poulter M, Uphill J et al (2009) Genetic risk factors for variant Creutzfeldt-Jakob disease: a genome-wide association study. Lancet Neurol 8(1):57–66

    Article  PubMed  PubMed Central  Google Scholar 

  156. Mead S, Stumpf MP, Whitfield J et al (2003) Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics. Science 300(5619):640–643

    Article  CAS  PubMed  Google Scholar 

  157. Mead S, Whitfield J, Poulter M et al (2008) Genetic susceptibility, evolution and the kuru epidemic. Philos Trans R Soc Lond Ser B Biol Sci 363(1510):3741–3746

    Article  Google Scholar 

  158. Mead S (2006) Prion disease genetics. Eur J Hum Genet 14(3):1–9

    Article  CAS  Google Scholar 

  159. Lantos B, Bhata K, Doey LJ et al (1997) Is the neuropathology of new variant Creutzfeldt-Jakob disease and kuru similar? Lancet 350(9072):187–188

    Article  CAS  PubMed  Google Scholar 

  160. Aguzzi A, Heikenwalder M (2003) Prion diseases: cannibals and garbage piles. Nature 423(6936):127–129

    Article  CAS  PubMed  Google Scholar 

  161. Brookfield JF (2003) Human evolution: a legacy of cannibalism in our genes? Curr Biol 13(15):R592–R593

    Article  CAS  PubMed  Google Scholar 

  162. Mead S, Whitfield J, Poulter M et al (2009) A novel protective prion protein variant that colocalizes with kuru exposure. N Engl J Med 361(21):2056–2065

    Article  CAS  PubMed  Google Scholar 

  163. Mead S, Uphill J, Beck J et al (2012) Genome-wide association study in multiple human prion diseases suggests genetic risk factors additional to PRNP. Hum Mol Genet 21(8):1897–1906

    Article  CAS  PubMed  Google Scholar 

  164. Marlar RA, Leonard BL, Billman BR et al (2000) Biochemical evidence of cannibalism at a prehistoric Puebloan site in southwestern Colorado. Nature 407(6800):25–26

    Article  CAS  Google Scholar 

  165. Riel-Salvatore J (2008) Mad Neanderthal disease? Some comments on “A potential role for Transmissible Spongiform Encephalopathies in Neanderthal extinction”. Med Hypotheses 71(3):473–474

    Article  PubMed  Google Scholar 

  166. Underdown S (2008) A potential role for transmissible spongiform encephalopathies in Neanderthal extinction. Med Hypotheses 71(1):4–7

    Article  PubMed  Google Scholar 

  167. Wadsworth JDF, Joiner S, Linehan JM et al (2008) The origin of the prion agent of kuru: molecular and biological strain typing. Philos Trans R Soc Lond Ser B Biol Sci 363(1510):3747–3753

    Article  CAS  Google Scholar 

  168. Wadsworth JDF, Joiner S, Linehan JM et al (2007) Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice. Proc Natl Acad Sci U S A 105(10):3885–3890

    Article  Google Scholar 

  169. Hill AF, Desbruslais M, Joiner S et al (1997) The same prion strain causes vCJD and BSE. Nature 389(6650):448–450

    Article  CAS  PubMed  Google Scholar 

  170. Parchi P, Cescatti M, Notari S et al (2010) Agent strain variation in human prion disease: insights from a molecular and pathological review of the National Institutes of Health series of experimentally transmitted disease. Brain 133(10):3030–3042

    Article  PubMed  PubMed Central  Google Scholar 

  171. Parchi P, Saverioni D (2012) Molecular pathology, classification, and diagnosis of sporadic human prion disease variants. Folia Neuropathol 50(1):20–45

    CAS  PubMed  Google Scholar 

  172. Manuelidis L, Chakrabarty T, Miyazawa K et al (2009) The kuru infectious agent is a unique geographic isolate distinct from Creutzfeldt-Jakob disease and scrapie agents. Proc Natl Acad Sci U S A 106(32):13529–13534

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  173. Jaunmuktane Z, Mead S, Ellis M et al (2015) Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy. Nature 525(7568):247–250

    Article  CAS  PubMed  Google Scholar 

  174. Kovacs GG, Lutz MI, Ricken G et al (2016) Dura mater is a potential source of Aβ seeds. Acta Neuropathol 131(6):911–923

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  175. Frontzek K, Lutz MI, Aguzzi A et al (2016) Amyloid-β pathology and cerebral amyloid angiopathy are frequent in iatrogenic Creutzfeldt-Jakob disease after dural grafting. Swiss Med Wkly 146:w14287. doi:10.4414/smw.2016.14287. eCollection 2016

    PubMed  Google Scholar 

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Acknowledgements

I am immensely indebted to late Dr. D. Carleton Gajdusek and Dr. Clarence J. Gibbs Jr. for generously providing me unique illustrations I have used through the text. I thank Prof. Shirley Lindenbaum and Prof. Michael Alpers for exciting discussion and helpful criticism, and Prof. James W. Ironside, the National CJD Surveillance Unit, for reading and correcting the MS in totally impossibly express tempo.

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  1. Laboratory of Electron Microscopy and Neuropathology, Department of Molecular Pathology and Neuropathology, Medical University Lodz, Czechoslowacka st 8/10, 92-216, Lodz, Poland

    Paweł P. Liberski

  2. Department of Extrapyramidal Diseases, Medical University of Lodz, Pomorska 251 st, 92-213, Lodz, Poland

    Agata Gajos

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  1. Paweł P. Liberski
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Correspondence to Paweł P. Liberski .

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  1. Department of Molecular Pathology, Medical University of Lodz, Lodz, Poland

    Pawel P. Liberski

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Liberski, P.P., Gajos, A. (2017). Kuru: Introduction to Prion Diseases. In: Liberski, P. (eds) Prion Diseases. Neuromethods, vol 129. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4939-7211-1_1

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  • DOI: https://doi.org/10.1007/978-1-4939-7211-1_1

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