Kuru

Gajdusek, D. Carleton; Zigas, Vincent

doi:10.1016/0002-9343(59)90251-7

Cited by 161 publications

(18 citation statements)

References 26 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…62 At the time of Gajdusek’s initial report, a full epidemic had been recognized, with more than 1000 cases identified in the first 10 months of the opening of a native hospital for the treatment and study of the disease. 34 Kuru, as the disease was known among the Fore, is a word used to designate the trembling associated with fear or cold. Gajdusek and his colleague V. Zigas, a medical officer in the Fore tribe region of New Guinea, lived among the Fore and investigated this major medical problem.…”

Section: Human Prion Diseases: Historical Perspectivementioning

confidence: 99%

“…Gajdusek later noted the similarity of kuru to “heterofamilial degenerative disorders of the central nervous system,” 34 whereas Igor Klatzo, neuropathologist at NIH, compared kuru to Creutzfeldt-Jakob disease (CJD), the latter being documented only 20 times and never observed in children. 59 In 1957, Klatzo wrote: “[kuru] seems to be definitely a new condition without anything similar described in the literature.…”

Section: Human Prion Diseases: Historical Perspectivementioning

confidence: 99%

“…According to their 1959 report, 34 kuru had the following properties. 1) It was responsible for the death of approximately 1% of the population per year (up to 50% in certain areas).…”

Section: Kuru and Cannabalismmentioning

confidence: 99%

See 2 more Smart Citations

Human prion diseases: surgical lessons learned from iatrogenic prion transmission

Bonda

Manjila

Mehndiratta

et al. 2016

FOC

105

View full text Add to dashboard Cite

The human prion diseases, or transmissible spongiform encephalopathies, have captivated our imaginations since their discovery in the Fore linguistic group in Papua New Guinea in the 1950s. The mysterious and poorly understood “infectious protein” has become somewhat of a household name in many regions across the globe. From bovine spongiform encephalopathy (BSE), commonly identified as mad cow disease, to endocannibalism, media outlets have capitalized on these devastatingly fatal neurological conditions. Interestingly, since their discovery, there have been more than 492 incidents of iatrogenic transmission of prion diseases, largely resulting from prion-contaminated growth hormone and dura mater grafts. Although fewer than 9 cases of probable iatrogenic neurosurgical cases of Creutzfeldt-Jakob disease (CJD) have been reported worldwide, the likelihood of some missed cases and the potential for prion transmission by neurosurgery create considerable concern. Laboratory studies indicate that standard decontamination and sterilization procedures may be insufficient to completely remove infectivity from prion-contaminated instruments. In this unfortunate event, the instruments may transmit the prion disease to others. Much caution therefore should be taken in the absence of strong evidence against the presence of a prion disease in a neurosurgical patient. While the Centers for Disease Control and Prevention (CDC) and World Health Organization (WHO) have devised risk assessment and decontamination protocols for the prevention of iatrogenic transmission of the prion diseases, incidents of possible exposure to prions have unfortunately occurred in the United States. In this article, the authors outline the historical discoveries that led from kuru to the identification and isolation of the pathological prion proteins in addition to providing a brief description of human prion diseases and iatrogenic forms of CJD, a brief history of prion disease nosocomial transmission, and a summary of the CDC and WHO guidelines for prevention of prion disease transmission and decontamination of prion-contaminated neurosurgical instruments.

show abstract

Section: Human Prion Diseases: Historical Perspectivementioning

confidence: 99%

Section: Human Prion Diseases: Historical Perspectivementioning

confidence: 99%

See 1 more Smart Citation

Human prion diseases: surgical lessons learned from iatrogenic prion transmission

Bonda

Manjila

Mehndiratta

et al. 2016

FOC

105

View full text Add to dashboard Cite

show abstract

“…The recent reports of production of a clinically and pathologically similar CJD in macaques by intracerebral injection of brain homogenate from afflicted cows [12], and of biochemical properties shared between the human cases and Bovine Spongiform Encephalopathy (BSE) [13], suggest that BSE is transmissible to man. [1,3,4,18,19] Transmissible Mink Encephalopathy (TME) Mink [1] Chronic Wasting Disease (CWD) Muledeer, elk [1] Bovine Spongiform Encephalopathy (BSE) Cows [11][12][13] Creutzfeld-Jacob Disease (CJD) Human [8][9][10] Fatal Familial Insomnia (FFI) Human [7] Gerstmann-Straussler-Scheinker Syndrome (GSS) Human [7] Alpers Syndrome Human [7] Kuru Human [2,3] Fig. (1).…”

Section: Transmission Of Prion Diseasesmentioning

confidence: 99%

“…After two years, nearly 10% of the flock developed Scrapie [1]. Some years later, a clinically and pathologically similar human disease, Kuru, meaning "trembling, " was identified in highlanders of New Guinea [2,3]. Again, ataxia predominated, proceeding to death usually within 9 months.…”

Section: Introductionmentioning

confidence: 99%

Prion Disease: A Deadly Disease for Protein Misfolding

Chakraborty¹,

Nandi²,

Jana

2005

CPB

View full text Add to dashboard Cite

An infectious particle, termed prion, composed largely and perhaps solely of a single protein, is the likely causative agent of prion disease. It produces lethal decline of cognitive and motor function. The responsible protein arrives at a pathogenic state by misfolding from a normal form that has ubiquitous tissue distribution. Prion diseases are often called spongiform encephalopathies. Probably most mammalian species develop these diseases. Specific examples in various animals are -Scrapie, Transmissible Mink Encephalopathy (TME ), Chronic Wasting Disease(CWD) and bovine spongiform encephalopathy (BSE). Humans are also susceptible to several prion diseases: Creutzfeld-Jacob Disease (CJD), Gerstmann-Straussler-Scheinker Syndrome (GSS), Fatal Familial Insomnia (FFI), Kuru and Alpers Syndrome. This paper reviews transmission of this diseases, protein involvement, nature of protein, the conversion process from PrP(c) to PrP(Sc), conversion of prion protein in vitro, the different proposed models for the conversion of PrP(c) to PrP(Sc), prion and other amyloid diseases, prion strains, structure of PrP(c) the particular process that may induce prion disease, and immunization against these diseases.

show abstract

D. Carleton Gajdusek, MD-- Nobel Laureate in Medicine for 1976

Tower¹

1977

Archives of Neurology

View full text Add to dashboard Cite

Kuru

Cited by 161 publications

References 26 publications

Human prion diseases: surgical lessons learned from iatrogenic prion transmission

Human prion diseases: surgical lessons learned from iatrogenic prion transmission

Prion Disease: A Deadly Disease for Protein Misfolding

D. Carleton Gajdusek, MD-- Nobel Laureate in Medicine for 1976

Contact Info

Product

Resources

About