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Kuru

ACKNOWLEDGEMENT
We are most grateful to Dr. Anthony Radford who kindly allowed us to include his PowerPoint presentation among the Internet series of Talks given to the Society by its members and invited speakers.

Dr Radford first visited New Guinea (then TPNG) in 1959 as a senior medical student and later returned to establish a successful rural medical practice. He recorded fifty years of his experiences in a book called "Singsings, Sutures and Sorcery". He was one of the many South Australians who contributed to the advances in public health in the area. His experience with the solving of the Kuru mystery is revealed in paper which he and Dr. Roy Scragg published in November 2013 entitled "Discovery of Kuru Revisited: How Anthropology Hindered Then Enhanced Kuru Research".

His slides deal with the early theories and the possible causes of Kuru, an incurable transmissible spongiform encephalopathy, formerly endemic in the Fore tribe of the Okapa District, Papua New Guinea. In his talk he mentioned Daniel Carlton Gajdusek, who used monkeys to confirm the transmission of the disease, and drew parallels with Creutzfeldt-Jakob Disease, and prions as a possible causal protein. In 1976, Gajdusek was a co-recipient of the Nobel Prize in Physiology or Medicine for his work on kuru, along with Baruch Samuel Blumberg (for his research on Hepatitis B).



Who first described the signs and symptoms of kuru?
&
the role of social science in the elucidation of its aetiology?

Anthony J Radford
23 May 2013

Eastern PNG map

Table 1: New Guinea has had many sentinel medical world highlights:

  • Enteritis necroticans (Clostridium perfringens) by Tim Murrell
  • Iodised oil to prevent goitre and endemic cretinism by McCullagh, Pharoah and Hetzel
  • Use of tetanus toxoid in pregnant women to prevent neonatal tetanus by Frank Schofield
  • Use of pneumococcal vaccine by Douglas and Riley

* Murrell, Hetzel, Douglas were all from Adelaide.

 

... and then there is KURU.


Medical student examines kuru patient at Okapa, 1968. Photo AJR.
Medical student examines a kuru patient at Okapa, 1968.
Photo AJR.

Table 2: Kuru signs and symptoms

  • Initially the patient noticed mild intermittent unsteadiness when crossing a narrow bridge into a garden.
  • Instability then increased, followed by tremors and, finally, quite violent shaking developed.
  • Eventually the patient was unable to walk, and then even to stand.
  • The victims became totally dependent on their care by others.
  • They developed facial grimaces. Some developed emotional lability.
  • At the end they were unable to swallow and progressive emaciation occurred.
  • Finally, intercurrent infection – usually pneumonia, and large decubitus ulcers developed which caused immense distress to both victims and relatives.
  • In a few the infection extended into the medulla of the brain, up into the areas controlling breathing and death occurred from respiratory failure.
Kuru patient and family, in a village near Okapa, 1968. Photo AJR.
Kuru patient and family, in a village near Okapa, 1968.
Photo AJR.
Kuru patient.
Medical review of kuru sufferer in a village near Okapa, 1968. Photo AJR.
Medical review of kuru sufferer in a village near Okapa, 1968.
Photo AJR.

This paper:

  • describes the sequence of events which led to the earliest descriptions of kuru, and
  • the role of social science,
    • first in delaying any medical assessment and investigation due to a belief of a sorcery origin, and
    • then in the development of the theory that its transmission was related to cannibalism,
  • illustrating the value of multidisciplinary approaches to many epidemiological problems.

Diseases which don’t fit into standard medical reference are often ascribed:

  • to sorcery or the supernatural in the animist world, or
  • to a psychosomatic origin in our own; such as epilepsy, gastric ulcers, and
  • Unexplained community infertility in more primitive communities, including New Guinea, also fall into this group as Roy Scragg has shown ...
  • ... AND so did KURU.

Sketch map of Papua New Guinea highlighting Okapa sub-district.  Source unknown.
Sketch map of Papua New Guinea highlighting Okapa sub-district. Source unknown.
Map of PNG highlighting Highland region. Source unknown.
Map of PNG highlighting Highland region. Source unknown.
Typical ridge-top village, Okapa, 1968. Photo AJR
Typical ridge-top village, Okapa, 1968.
Photo AJR.
Okapa Valley, Eastern Highlands District, TPNG. Photo AJR
Okapa Valley, Eastern Highlands District, TPNG. Photo AJR.
Women preparing food in bamboo pipes. See text. Okapa 1968. Photo AJR. Photo AJR
Women preparing food in bamboo pipes. See text. Okapa 1968.
Photo AJR.
Sample of books concerning kuru. Photo AJR
Sample of books concerning kuru.
Photo AJR.
Patrol Officer John Colman, Dr Vin Zigas, medical orderlies and villagers, Okapa ca 1955. Courtesy  Bill Brown.
Patrol Officer John Colman, Dr Vin Zigas, medical orderlies and villagers, Okapa ca 1955.
Courtesy Bill Brown.

South Australian 'Honour Board'

The Adelaide Group:

  • Professor Norrie Robson (physician)
  • Professor Henry Bennett (geneticist)
  • Mr Donald Simpson (neurosurgeon)
  • Dr Harry Lander (physician)
  • Dr Clive Auricht, Dr Bronte Gabb & Caroline Leach
  • Dr Michael Alpers
  • Dr Roy Scragg

Table 3: Earliest references & descriptions.
Arthur T. Carey1950-51
Ronald & Catherine Berndt1951 in Berndt R, 1958
John R. McArthur? Dec 1953 (in Z & G 1957)
William 'Bill' BrownFebruary 1954
John R. McArthurAugust 1954, 1955
John ColmanSeptember 1956
Frank EarlSeptember 1956
Vin ZigasDecember 1956
Charles JuliusJanuary 1957
Roy ScraggFebruary 1957
D. Carleton Gajdusek and Vin ZigasNovember 1957
Vin Zigas and D. C. GajdusekNovember 1957

It was government patrol officers who, three to five years earlier, were the first to report and describe the features of 'kuru' .

"one of the vast group of chronic, progressive, heredo-familial degenerations of the central nervous systems ... though genetic predisposition is strongly suggested ... but any ethnic environmental variables [operating] ... have not been determined."
(Zigas and Gajdusek, 1957).


Table 4: Postulated causes of Kuru
SorceryFore villagersDecades
PsychosomaticPatrol officers & R. Berndt1950s
Encephalitis with post-encephalic signsH. F. Earl1955
HysteriaVin Zigas1955
Somatic illness: type unknownC. Julius1956
Chronic progressive heredo-familial encephalopathyC. Gajdusek & V. Zigas1957
Toxic - 'Strongly suggested'R. Scragg; D. C. Gajdusek & V. Zigas1957
Genetic polymorphismJ. H. Bennett et al.1957
Slow virus with very long incubationW. Hadlow1959
PrionS. Pruisner1982

Graph of kuru deaths.
Change in childhod and adolescent mortality from kuru from 1957 to 1964.
Change in childhood and adolescent mortality from kuru by age group, from 1957 to 1964.
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