Kuru: The First Prion Disease

  • Paweł P. LiberskiEmail author
  • Beata Sikorska
  • Paul Brown
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 724)


Kuru disease is linked with the name of D. Carleton Gajdusek and he was the first to show that this human neurodegenerative disease can be transmitted to chimpanzees and subsequently classified as a transmissible spongiform encephalopathy (TSE), or slow unconventional virus disease. It was first reported to Western world in 1957 by Gajdusek and Vincent Zigas,1,2 and in 1975 a complete bibliography of kuru was published by Alpers et al.3 “Kuru” in the Fore language in Papua New Guinea means to shiver from fever and cold. The disease has been found to spread through ritualistic cannibalism and is an invariably fatal cerebellar ataxia accompanied by tremor, choreiform and athetoid movements. Neuropathologically, kuru is characterized by the presence of amyloid “kuru” plaques.


Neurodegenerative Disease Prion Disease Cerebellar Ataxia Transmissible Spongiform Encephalopathy Intracellular Vacuole 
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  1. 1.
    Gajdusek DC, Zigas V. 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 1959; 26:442–469.PubMedCrossRefGoogle Scholar
  2. 2.
    Gajdusek DC, Zigas V. Studies of kuru. I. The ethnologic setting of kuru. Am J Trop Med Hyg 1961; 10:80–91.PubMedGoogle Scholar
  3. 3.
    Alpers MP, Gajdusek DC, Gibbs CJ Jr. Bibliography of Kuru. Bethesda: Natl Inst Health, 1975.Google Scholar
  4. 4.
    Gibbs CJ Jr, Gajdusek DC, Asher DM et al. Creutzfeldt-Jakob disease (spongiform encephalopathy): transmission to chimpanzee. Science 1968; 161:388–389.PubMedCrossRefGoogle Scholar
  5. 5.
    Jaskolski M, Liberski PP. Kurt Wüthrich-cowinner of the Nobel Prize in Chemistry, Acta Neurobiol Exp 2002; 62:288–289.Google Scholar
  6. 6.
    Van Rossum A. Spastic pseudosclerosis (Creutzfeldt-jakob disease). In: PJ Vinken, GW Bruyn, eds. Handbook of Clinical Neurology. Vol 6. Diseases of Basal Ganglia. Amsterdam: North-Holland Publ Comp, 1968: 726–760.Google Scholar
  7. 7.
    Gajdusek DC. Kuru and its contribution to medicine. Phil Trans R Soc B 2008; 363:3697–3700.PubMedCrossRefGoogle Scholar
  8. 8.
    Mathews JD. The changing face of kuru: a personal perspective. Phil Trans R Soc 2008; 363:3679–3684.CrossRefGoogle Scholar
  9. 9.
    Mathews JD. The epidemiology of kuru. Papua new Guinea Med J 1967; 10:76–82.Google Scholar
  10. 10.
    Lindenbaum S. Kuru sorcery. Disease and danger in the New Guinea Highlands. Palo Alto: Mayfield Publishing Company, 1979.Google Scholar
  11. 11.
    Gajdusek DC. Unconventional viruses and the origin and disappearance of kuru. Science 1977; 197:943–960.PubMedCrossRefGoogle Scholar
  12. 12.
    Zigas V, Gajdusek DC. 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 1959; 3:1–31.Google Scholar
  13. 13.
    Zigas V. Origin of investigations on slow virus infections in man. In: B Prusiner, Hadlow WJ, eds. Slow Transmissible Diseases of the Nervous System, vol 1. New York: Academic Press, 1979:3–6.Google Scholar
  14. 14.
    Sorenson ER, Gajdusek DC. Nutrition in the kuru region. I. Gardening, Food handling and diet of the Fore people. Acta Tropica 1969; 26:281–330.PubMedGoogle Scholar
  15. 15.
    Sorenson ER, Gajdusek DC. 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 1966; 37 (suppl):149–243.PubMedGoogle Scholar
  16. 16.
    Hadlow WJ. Kuru likened to scrapie: the story remembered. Phil Trans R Soc B 2008; 363:3644.PubMedCrossRefGoogle Scholar
  17. 17.
    Hadlow WJ. Neuropathology and the scrapie-kuru connection. Brain Pathol 1995; 5:27–31.PubMedCrossRefGoogle Scholar
  18. 18.
    Hadlow WJ. Scrapie and kuru. Lancet 1959; 2:289–290.CrossRefGoogle Scholar
  19. 19.
    Hadlow WJ. 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. New York: Ellis Horwood, 1993: 40–46.Google Scholar
  20. 20.
    Innes JRM, Saunders LZ. Comparative neuropathology, Academic Press, New York 1962; 839.Google Scholar
  21. 21.
    Goldfarb LG, Cervenakova L, Gajdusek DC. Genetic studies in relation to kuru: an overview. Curr Mol Med 2004; 4:375–384.PubMedCrossRefGoogle Scholar
  22. 22.
    Gajdusek, telephone conversation, 2008.Google Scholar
  23. 23.
    Zlotnik I. Significance of vacuolated neurones in the medulla of sheep infected with scrapie. Nature 1957a; 180,393–394.PubMedCrossRefGoogle Scholar
  24. 24.
    Zlotnik I. Vacuolated neurons in sheep affected with scrapie. Nature 1957b;179,737.PubMedCrossRefGoogle Scholar
  25. 25.
    Gibbs CJ Jr, Gajdusek DC. 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, 1965:39–48.Google Scholar
  26. 26.
    Gajdusek DC, Zigas V, Baker J. Studies on kuru. III. Patterns of kuru incidence: demographic and geographic epidemiological analysis. Am J Trop Med Hyg 1961; 10:599–627.PubMedGoogle Scholar
  27. 27.
    Mathews JD. A transmission model for kuru. Lancet 1967; 285:821–825.CrossRefGoogle Scholar
  28. 28.
    Matthews JD. Kuru as an epidemic disease. In: Hornabrook RW, ed. Essays on Kuru, Papua New Guinea Inst of Med Res Monographs no 3. Berkshire: Faringdon, 1976:83–104.Google Scholar
  29. 29.
    Klitzman RL, Alpers MP, Gajdusek DC. The natural incubation period of kuru and the episodes of transmission in three clusters of patients. Neuroepidemiology 1984; 3:3–20.CrossRefGoogle Scholar
  30. 30.
    Alpers MP. Kuru: a clinical study. Mimeographed. US Dept Health, Education, Welfare 1964; 1–38.Google Scholar
  31. 31.
    Alpers MP. Kuru: age and duration studies. Mimeographed. Dept Med, Univ Adelaide 1964; 12.Google Scholar
  32. 32.
    Zigas V, Gajdusek DC. Kuru. Clinical study of a new syndrome resembling paralysis agitans in natives of the eastern Highlands of Australian new Guinea. Med J Australia 1957; 44:745–754.PubMedGoogle Scholar
  33. 33.
    Klatzo I, Gajusek DC, Zigas V. Evaluation of pathological findings in twelve cases of kuru. In: Van Boagert L, Radermecker J, Hozay J, Lowenthal A, eds. Encephalitides. Amsterdam: Elsevier Publ. Comp, 1959:172–190.Google Scholar
  34. 34.
    Klatzo I, Gajusek DC. Pathology of kuru. Lab Invest 1959; 8:799–847.PubMedGoogle Scholar
  35. 35.
    Beck E, Daniel PM, Alpers MP et al. Experimental kuru in chimpanzees. A pathological report. Lancet 1966; 2:1056–1059.PubMedCrossRefGoogle Scholar
  36. 36.
    Beck E, Daniel PM, Asher DM et al. Experimental kuru in chimpanzees. A neuropathological study. Brain 1973; 96:441–462.PubMedCrossRefGoogle Scholar
  37. 37.
    Beck E, Daniel PM, Gajdusek DC. A comparison between the neuropathological changes in kuru and scrapie, system degeneration. Proc of the VIth Int Congress Neuropathol, Zurich 1965; 213–218.Google Scholar
  38. 38.
    Beck E, Daniel PM. 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. Washington, DC: US Dept Health, Education, Welfare, 1965:85–93.Google Scholar
  39. 39.
    Beck E, Daniel PM. Prion diseases from a neuropathologist’s perspective. In: Prusiner SB, Collinge J, Powell J, Anderton B, eds. Prion Diseases of Humans and Animals. New York, London, Toronto, Sydney: Singapore Ellis Horwood, 1993:63–65.Google Scholar
  40. 40.
    Seitelberger F. Eigenartige familiar-hereditare Krankheit des Zetralnervensystems in einer niederosterreichischen Sippe. Wien Klein Wochen 1962; 74:687–691.Google Scholar
  41. 41.
    Neuman MA, Gajdusek DC, Zigas V. Neuropathologic findings in exotic neurologic disorder among natives of the Highlands of New Guinea. J Neuropathol Exp Neurol 1964; 23:486–507.CrossRefGoogle Scholar
  42. 42.
    Masters CL, Gajdusek DC, Gibbs CJ 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 1981; 104:559–588.PubMedCrossRefGoogle Scholar
  43. 43.
    Sikorska B, Liberski PP, Sobów T et al. 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 2009; 35:46–59.PubMedCrossRefGoogle Scholar
  44. 44.
    Hainfellner J, Liberski PP, Guiroy DC et al. Pathology and immunohistochemistry of a kuru brain. Brain Pathol 1997; 7:547–554.PubMedCrossRefGoogle Scholar
  45. 45.
    McLean CA, Ironside JW, Alpers MP et al. Comparative neuropathology of kuru with the new variant of Creutzfeldt-Jakob disease: evidence for strain of agent predominating over genotype of host. Brain Pathol 1998; 8:428–437.Google Scholar
  46. 46.
    Peat A, Field EJ. An unusual structure in kuru brain. Acta Neuropathol (Berl) 1970; 15:288–292.CrossRefGoogle Scholar
  47. 47.
    Liberski PP. The occurrence of cytoplasmic lamellar bodies in scrapie infected and normal hamster brains. Neuropatol. Polska 1988,26:79–85.Google Scholar
  48. 48.
    Field EJ, Mathews JD, Raine CS. Electron microscopic observations on the cerebellar cortex in kuru. J Neurol Sci 1969; 8:209–224PubMedCrossRefGoogle Scholar
  49. 49.
    Liberski PP, Yanagihara R, Gibbs CJ, Jr et al. Re-evaluation of experimental Creutzfeldt-Jakob disease: serial studies of the Fujisaki strain of Creutzfeldt-Jakob disease virus in mice. Brain 1990; 113:121–137.PubMedCrossRefGoogle Scholar
  50. 50.
    Goodbrand IA, Ironside JW, Nicolson D et al. Prion protein accumulations in the spinal cords of patients with sporadic and growth hormone-associated Creutzfeldt-Jakob disease. Neurosci Lett 1995; 183:127–130.PubMedCrossRefGoogle Scholar
  51. 51.
    Brandner S, Whitfield J, Boone K et al. Central and peripheral pathology of kuru: pathological analysis of a recent case and comparison with other forms of human prion diseases. Phil Trans R Soc B 2008; 363:3755–3763.PubMedCrossRefGoogle Scholar
  52. 52.
    Lee H-S, Brown P, Cervenakova L et al. Increased susceptibility to kuru of carriers of the PRNP 129 Methionine/ Methionine genotype. J Inf Dis 2001; 183:192–196.CrossRefGoogle Scholar
  53. 53.
    Cervenakova L, Goldfarb LG, Garruto R et al. Phenotype-genotype studies in kuru: implications for new variant Creutzfeldt-Jakob disease. Proc Natl Acad Sci USA 1998; 95:13239–13241.PubMedCrossRefGoogle Scholar
  54. 54.
    Matthews JD, Glasse RM, Lindenbaum S. Kuru and cannibalism. Lancet 1968; 292:449–452.CrossRefGoogle Scholar
  55. 55.
    Mead S, Poulter M, Uphill J et al. Genetic risk factors for variant Creutzfeldt-Jakob disease: a genome-wide association study. Lancet Neurology 2009; 8:57–66.PubMedCrossRefGoogle Scholar
  56. 56.
    Mead S, Stumpf MP, Whitfield J et al. Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics. Science 2003; 300:640–643.PubMedCrossRefGoogle Scholar
  57. 57.
    Mead S, Whitfield J, Poulter M et al. Genetic susceptibility, evolution and the kuru epdemic. Phil Trans R Soc B 2008; 363:3741–3746.PubMedCrossRefGoogle Scholar
  58. 58.
    Mead S. Prion disease genetics. Eur J Hum Genet 2006; 14:273–81.PubMedCrossRefGoogle Scholar
  59. 59.
    Lantos B, Bhata K, Doey LJ et al. Is the neuropathology of new variant Creutzfeldt-Jakob disease and kuru similar? Lancet 1997; 350:187–188.PubMedCrossRefGoogle Scholar
  60. 60.
    Aguzzi A, Heikenwalder M. Prion diseases: cannibals and garbage piles. Nature 2003; 423:127–129.PubMedCrossRefGoogle Scholar
  61. 61.
    Brookfield JF. Human evolution: a legacy of cannibalism in our genes? Curr Biol 2003; 13:R592–593.PubMedCrossRefGoogle Scholar
  62. 62.
    Marlar RA, Leonard BL, Billman BR et al. Biochemical evidence of cannibalism at a prehistoric Puebloan site in southwestern Colorado. Nature 2000; 407:25–26.CrossRefGoogle Scholar
  63. 63.
    Collinge CJ, Whitfield J, McKintosch E et al. Kuru in the 21st century-an acquired human prion disease with very long incubation periods. Lancet 2006; 367:2068–2074.PubMedCrossRefGoogle Scholar
  64. 64.
    Wadsworth JDF, Joiner S, Linehan JM et al. The origin of the prion agent of kuru: molecular and biological strain typing. Phil Trans R Soc B 2008; 363:3747–3753.PubMedCrossRefGoogle Scholar
  65. 65.
    Wadsworth JDF, Joiner S, Linehan JM et al. Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice. Proc Natl ACad SCi USA 2007; 105:3885–3890.CrossRefGoogle Scholar
  66. 66.
    Collinge J, Sidle KCL, Meads J et al. Molecular analysis of prion strain variation and the etiology of “new variant” CJD. Nature 1996; 383:685–670.PubMedCrossRefGoogle Scholar
  67. 67.
    Hill AF, Desbruslais M, Joiner S et al. The same prion strain causes vCJD and BSE. Nature 1997; 389:448–450.PubMedCrossRefGoogle Scholar
  68. 68.
    Gibbs CJ. Spongiform encephalopathies—slow, latent and temperate virus infections—in retrospect. In: Prusiner SB, Collinge J, Powell J, Anderon B, eds. Prion Diseases of Humans and Animals. New York, London, Toronto, Sydney, Singapore: Ellis Horwood, 1993:77–91.Google Scholar
  69. 69.
    Gajdusek DC. Infectious amyloids: subacute spongiform encephalopathies as transmissible cerebral amyloidoses. In: BN Fields, DM Knippe, PM Howley eds. Fields Virology, 3rd ed. Philadelphia: Lippincott-Raven, 1996:2851–2900.Google Scholar

Copyright information

© Landes Bioscience and Springer Science+Business Media 2012

Authors and Affiliations

  • Paweł P. Liberski
    • 1
    Email author
  • Beata Sikorska
    • 1
  • Paul Brown
    • 2
  1. 1.Laboratory of Electron Microscopy and Neuropathology, Department of Molecular Pathology and NeuropathologyMedical University LodzLodzPoland
  2. 2.CEA/DSV/iMETI/SEPIAFontenay-aux-RosesFrance

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