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Kuru: The First Prion Disease

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Neurodegenerative Diseases

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 724))

Abstract

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.

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References

  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.

    Article  PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  3. Alpers MP, Gajdusek DC, Gibbs CJ Jr. Bibliography of Kuru. Bethesda: Natl Inst Health, 1975.

    Google Scholar 

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

    Article  PubMed  Google Scholar 

  5. Jaskolski M, Liberski PP. Kurt Wüthrich-cowinner of the Nobel Prize in Chemistry, Acta Neurobiol Exp 2002; 62:288–289.

    Google Scholar 

  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. Gajdusek DC. Kuru and its contribution to medicine. Phil Trans R Soc B 2008; 363:3697–3700.

    Article  PubMed  Google Scholar 

  8. Mathews JD. The changing face of kuru: a personal perspective. Phil Trans R Soc 2008; 363:3679–3684.

    Article  Google Scholar 

  9. Mathews JD. The epidemiology of kuru. Papua new Guinea Med J 1967; 10:76–82.

    Google Scholar 

  10. Lindenbaum S. Kuru sorcery. Disease and danger in the New Guinea Highlands. Palo Alto: Mayfield Publishing Company, 1979.

    Google Scholar 

  11. Gajdusek DC. Unconventional viruses and the origin and disappearance of kuru. Science 1977; 197:943–960.

    Article  PubMed  CAS  Google Scholar 

  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. 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. 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.

    PubMed  CAS  Google Scholar 

  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.

    PubMed  Google Scholar 

  16. Hadlow WJ. Kuru likened to scrapie: the story remembered. Phil Trans R Soc B 2008; 363:3644.

    Article  PubMed  Google Scholar 

  17. Hadlow WJ. Neuropathology and the scrapie-kuru connection. Brain Pathol 1995; 5:27–31.

    Article  PubMed  CAS  Google Scholar 

  18. Hadlow WJ. Scrapie and kuru. Lancet 1959; 2:289–290.

    Article  Google Scholar 

  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. Innes JRM, Saunders LZ. Comparative neuropathology, Academic Press, New York 1962; 839.

    Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  22. Gajdusek, telephone conversation, 2008.

    Google Scholar 

  23. Zlotnik I. Significance of vacuolated neurones in the medulla of sheep infected with scrapie. Nature 1957a; 180,393–394.

    Article  PubMed  CAS  Google Scholar 

  24. Zlotnik I. Vacuolated neurons in sheep affected with scrapie. Nature 1957b;179,737.

    Article  PubMed  CAS  Google Scholar 

  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. 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.

    PubMed  CAS  Google Scholar 

  27. Mathews JD. A transmission model for kuru. Lancet 1967; 285:821–825.

    Article  Google Scholar 

  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. 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.

    Article  Google Scholar 

  30. Alpers MP. Kuru: a clinical study. Mimeographed. US Dept Health, Education, Welfare 1964; 1–38.

    Google Scholar 

  31. Alpers MP. Kuru: age and duration studies. Mimeographed. Dept Med, Univ Adelaide 1964; 12.

    Google Scholar 

  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.

    PubMed  CAS  Google Scholar 

  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. Klatzo I, Gajusek DC. Pathology of kuru. Lab Invest 1959; 8:799–847.

    PubMed  CAS  Google Scholar 

  35. Beck E, Daniel PM, Alpers MP et al. Experimental kuru in chimpanzees. A pathological report. Lancet 1966; 2:1056–1059.

    Article  PubMed  CAS  Google Scholar 

  36. Beck E, Daniel PM, Asher DM et al. Experimental kuru in chimpanzees. A neuropathological study. Brain 1973; 96:441–462.

    Article  PubMed  CAS  Google Scholar 

  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. 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. 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. Seitelberger F. Eigenartige familiar-hereditare Krankheit des Zetralnervensystems in einer niederosterreichischen Sippe. Wien Klein Wochen 1962; 74:687–691.

    CAS  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

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

    Article  CAS  Google Scholar 

  47. Liberski PP. The occurrence of cytoplasmic lamellar bodies in scrapie infected and normal hamster brains. Neuropatol. Polska 1988,26:79–85.

    CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  54. Matthews JD, Glasse RM, Lindenbaum S. Kuru and cannibalism. Lancet 1968; 292:449–452.

    Article  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  58. Mead S. Prion disease genetics. Eur J Hum Genet 2006; 14:273–81.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  60. Aguzzi A, Heikenwalder M. Prion diseases: cannibals and garbage piles. Nature 2003; 423:127–129.

    Article  PubMed  CAS  Google Scholar 

  61. Brookfield JF. Human evolution: a legacy of cannibalism in our genes? Curr Biol 2003; 13:R592–593.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  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. 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 

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Author information

Authors and Affiliations

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

    Paweł P. Liberski & Beata Sikorska

  2. CEA/DSV/iMETI/SEPIA, Fontenay-aux-Roses, France

    Paul Brown

Authors
  1. Paweł P. Liberski
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  2. Beata Sikorska
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  3. Paul Brown
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Correspondence to Paweł P. Liberski .

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Editors and Affiliations

  1. School of Science and Technology, Nottingham Trent University, Nottingham, UK

    Shamim I. Ahmad BSc, MSc, PhD

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Liberski, P.P., Sikorska, B., Brown, P. (2012). Kuru: The First Prion Disease. In: Ahmad, S.I. (eds) Neurodegenerative Diseases. Advances in Experimental Medicine and Biology, vol 724. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-0653-2_12

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