eMedicine Specialties > Infectious Diseases > Viral Infections

Kuru: Follow-up

Author: Paul A Janson, MD, Instructor, Tufts University School of Medicine; Director, EMT/RN Consultants; Consulting Staff, Department of Emergency Medicine, Lawrence General Hospital
Coauthor(s): Rachel H Chung, MD, Consulting Staff, Department of Family Practice, North Clinic, North Memorial Hospital; Mary Buechler, MD, Per Diem Staff, Department of Emergency Medicine, Caritas Holy Family Medical Center
Contributor Information and Disclosures

Updated: Apr 13, 2009

Follow-up

Deterrence/Prevention

  • The disease has essentially vanished because cannibalism is no longer practiced by the Fore.

Complications

  • Most patients die of complications such as decubiti, infections, and pneumonia.

Prognosis

  • The disease is uniformly fatal.

Patient Education

Miscellaneous

Special Concerns

  • Kuru has been transmitted to monkeys and mice under laboratory conditions, but it has no real veterinary implications. Other prion diseases are of major veterinary importance, and an understanding of kuru is helpful in understanding these other prion diseases. All prion diseases have similar proteins as their causative agents, and, although species barriers exist, transmission between species is well documented.
  • The oldest known prion disease is scrapie, a disease found in sheep that was described as early as the 17th century. In England, during the 18th century, recommended measures for control of the disease included the slaughtering of infected sheep away from the flock. Giving the meat to the servants to eat was considered safe. In 1936, the disease was shown to be transmissible when sheep were kept for an 18-month incubation for the first time. Also at that time, the disease was transmitted when sheep were accidentally inoculated with a preparation that had been derived from sheep infected with scrapie. Earlier efforts at transmission had probably been unsuccessful because the animals had been observed for only a few months.
    • Scrapie has also been proposed as possibly being transmitted to humans in areas where large quantities of sheep brain are eaten, such as northern Africa. Here, an unusually high incidence of a particular form of CJD exists.
    • Scrapie is thought to be the source of the prion that causes BSE and the resultant new vCJD in humans that is now seen in Great Britain.
  • CJD occurs in 3 patterns.
    • A familial form and a sporadic form of CJD exist; both have been described for many years, and both are thought to be caused by prions. In the case of the familial disorder, an abnormal gene on chromosome 20 has been documented. The sporadic form is thought to be caused by a spontaneous mutation at the same site.
    • A new form of this disease, termed vCJD, presents in younger patients and has been associated with BSE. As noted above, the clinical features of vCJD and kuru are similar, and both differ from classic CJD.
  • Prion spongiform encephalopathy also is present in mink on mink farms, where epidemics have occurred.
  • Chronic wasting disease may be present in as many as 2.5-10% of wild deer and elk in northern Colorado and southern Wyoming in the United States. Although cases have been investigated by the Centers for Disease Control and Prevention (CDC), no transmission to humans has been documented.
  • Other prion diseases may be present in animals and humans, but the difficulties encountered in demonstrating the prion have hampered investigation.
 


More on Kuru

Overview: Kuru
Differential Diagnoses & Workup: Kuru
Treatment & Medication: Kuru
Follow-up: Kuru
References
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References

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Further Reading

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Keywords

kuru, prion, prion-related disease, prion disease, human prion disease, spongiform encephalopathy, slow virus, infectious proteins, infectious amyloids, crystal protein, bovine spongiform encephalopathy, BSE, mad cow disease, scrapie, endocannibalism, Creutzfeldt-Jakob disease, CJD, variant Creutzfeldt-Jakob disease, vCJD, Gerstmann-Strãussler-Scheinker syndrome, fatal familial insomnia, Fore, New Guinea, prion protein, PrP, PRNP

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Contributor Information and Disclosures

Author

Paul A Janson, MD, Instructor, Tufts University School of Medicine; Director, EMT/RN Consultants; Consulting Staff, Department of Emergency Medicine, Lawrence General Hospital
Paul A Janson, MD is a member of the following medical societies: American Academy of Emergency Medicine and American College of Emergency Physicians
Disclosure: Nothing to disclose.

Coauthor(s)

Rachel H Chung, MD, Consulting Staff, Department of Family Practice, North Clinic, North Memorial Hospital
Disclosure: Nothing to disclose.

Mary Buechler, MD, Per Diem Staff, Department of Emergency Medicine, Caritas Holy Family Medical Center
Mary Buechler, MD is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, and American College of Occupational and Environmental Medicine
Disclosure: Nothing to disclose.

Medical Editor

Martin J Wood, MD †, Former Consulting Staff, Department of Infection and Tropical Medicine, Birmingham Heartlands Hospital, UK
Martin J Wood, MD † is a member of the following medical societies: Alliance for the Prudent Use of Antibiotics, American Society for Microbiology, Infectious Diseases Society of America, International Society for Infectious Diseases, and Royal College of Physicians
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Thomas M Kerkering, MD, Chief of Infectious Diseases, Virginia Tech, Carilion School of Medicine, Roanoke, Virginia
Thomas M Kerkering, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American Public Health Association, American Society for Microbiology, American Society of Tropical Medicine and Hygiene, Infectious Diseases Society of America, Medical Society of Virginia, and Wilderness Medical Society
Disclosure: Nothing to disclose.

CME Editor

Eleftherios Mylonakis, MD, Clinical and Research Fellow, Department of Internal Medicine, Division of Infectious Diseases, Massachusetts General Hospital
Eleftherios Mylonakis, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Physicians, American Society for Microbiology, and Infectious Diseases Society of America
Disclosure: Nothing to disclose.

Chief Editor

Burke A Cunha, MD, Professor of Medicine, State University of New York School of Medicine at Stony Brook; Chief, Infectious Disease Division, Winthrop-University Hospital
Burke A Cunha, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, and Infectious Diseases Society of America
Disclosure: Nothing to disclose.

 
 
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