Original article: L’endocannibalismo ritualistico della tribù Fore e malattia di Kuru (microbiologiaitalia.it) by Ilaria Salvatori
The cult of the dead has always played an important role in a lot of cultures and civilizations. In many communities, the death of an individual isn’t felt only by his closest relatives but involves a larger group of individuals, who gather around the relatives of the deceased, celebrating his passage to a better life through the most disparate rites.
Among the funeral rituals, the Egyptian’s is one of the better known. For the Egyptians, death represented the passage to an afterlife, and the funeral rite was indispensable to accompany the deceased to the afterlife.
The preservation of the deceased’s body was ensured through mummification: a process that involves the removal of internal organs (except the heart) and embalming in linen cloths.
Another noteworthy rite was one of the Zoroastrians, the Zoroaster’s disciples. They believed that the human body was impure. Therefore, during the funeral rites, it was custom to put the dead’s body on an elevated palace, in order to have the vultures feed on it. This fate also befell the Persian emperors Darius, Cyrus, Xerxes and Artaxerxes.
So, to summarize…There are those who embalm their deceased ones, those who get rid of the body appealing to the vultures and those who EAT the brain of their loved ones. You got it right, the brain.
Fore Tribe
The Fore tribe is a native tribe of the Okapa district, located in southeastern Papua New Guinea. These people live mainly rurally: practicing agriculture, eating the fruits of the earth and raisings animals, mainly pigs.
Their society is organized into kinship groups: although these groups of individuals are not parents, they have all the same privileges and responsibilities.
Comparatively to many other tribes, Fore is dedicated to magic and ritualistic practices that mainly concern the destiny of the deceased’s soul after death. They believed that the human being has 5 souls: auma, ama, kwela, aona e yesegi. The auma, similar to the western concept of soul, is the first to leave the body after death to reach the land of the deceased. The ama, a concept linked to the western concept of love, is the auma’s simulacrums and remains on the earth until the funeral rite is not completed.
Ritualistic endocannibalism
Two or three days after the end of the funeral rite, the deceased’s body can be cooked and eaten. But what is the reason behind it? They believe that ama (and so love) is able to provide a blessing to those who eat the remains. Women and children were the luckiest ones: they got to eat the brain, as an act of blessing.
This particular practice can be defined as ritualistic endocannibalism and consists of eating the remains of the deceased as a part of a ritual. Interesting, albeit disgusting!!!!
Members of the Fore tribe continued to practice this type of ritual until, between 1930 and 1960, it started to cause them some problems. Many individuals, especially women and children, began to die strangely of an inexplicable death.
In the beginning, it was thought that these deaths were due to acts of witchcraft by the “opponents” of the family. The sorcerer, through the use of hair and nails of the individual to curse, could carry out the spell and cause the onset of the disease.
These smashing explications could be interesting, since the situation began to collapse. So, the question arose spontaneously. What were the deaths due to?
Kuru!
In 1951, Arthur Carey started to pay attention closely to the events regarding Fore tribe. He was the first man to describe the clinical symptoms of the disease, naming it Kuru. In Fore language, the word Kuru means “tremble for fever or cold”, which refers to the classical signs and symptoms of the disease, for instance, loss of muscle control and trembling.
Emotional changes were also evident in the symptomatology (state of euphoria and compulsive laughter): therefore, kuru was also called the laughing disease. Another Fore word to identify this disease was “nagi nagi”, which means a foolish person because it afflicted women who laughed out loud.
The information provided by Fore on the disease was so detailed that it was possible to reconstruct, albeit partially, its epidemiology. Some evidences suggest that the disease first appeared in Uwami village around 1900, and then in North-Fore around 1920.
Although Kuru is believed to have developed in a small isolated population, the disease reached epidemic proportions. Daniel Carleton Gajdusek and Vincent Zigas (respectively a virologist and a doctor) started to study this phenomenon in order to identify the cause of kuru.
Gajdusek, through his research, discovered that the practice of endocannibalism allowed the transmission of an unconventional and filterable infectious agent, which he called slow virus. Later, Stanley B. Prusiner called this infectious agent, the prion.
Now that we have a name for the pathogen another question begs to be asked…what is that?
On the basis of the contribution of many scientists, Kuru has been included within the group of human transmissible spongiform encephalopathies (TSE). The TSE group includes the Gerstmann-Sträussler-Scheinker (GSS) syndrome, fatal familial insomnia (FFI) and Creutzfeldt-Jakob desease.
It is a group of neurodegenerative, progressive and fatal diseases associated with the incorrect folding and aggregation in the brain of a protein called Prion (PRoteinaceous Infective ONly particle).
Prion PrPsc is the altered form of a protein normally expressed in mammals (PrPc); the alteration of this protein is believed to be caused by a conformational modification induced by a genetic and spontaneous mutation or by an infecting prion.
These types of disease are characterized by a long incubation period; another distinctive aspect is the morphological changes of the brain, which resembles a sponge.
Human prion diseases can either arise sporadically, be hereditary or be acquired (which is the case for Kuru). What is the origin of Kuru? It seems to have originated from the consumption of the remains of a cadaver infected with sporadic Creutzfeldt-Jakob disease.
How is the brain involved?
The physiological form of this protein is located in tissues such as: skeletal muscle, follicular dendritic cells, endothelial cells and neurons. The normal form is located mainly in the brain and, thus, its abnormal folding can lead to cerebral damage.
As soon as the abnormal form PrPsc enters a healthy organism, it is able to modify the normal form of the protein, converting it into the pathological form. These forms can also modify other proteins, setting off a chain reaction that leads to the production of large quantities of infectious protein.
In Fore tribes, women and children were more exposed to the disease as they came into contact more frequently with the infectious form of the protein, since it was custom for them to consume the brain, mainly. Mystery solved!
Clinical features
Despite the fact that Kuru has been classified in the same cluster as Creutzfeldt-Jakob disease, it is not characterized by classic dementia, which is commonly associated with other types of spongiform encephalopathies.
Kuru is characterized by the presence of 3 clinical phases (itinerant, sedentary and terminal), preceded by a period of headache, limbs and joints pain.
Brain abnormalities are due to the accumulation of PrPsc form, which leads to synaptic damage, spongiform degeneration, brain inflammation and neuronal death. The onset of this disease is characterized by a decline in motor ability, which results uncoordinated movements. The first phase is then followed by tremors, dysarthria and severe neurological symptoms, leading to a slow and excruciating death.
To make a long story short
Mortuary practices associated with ritualistic endocannibalism have been abandoned in the early 1960s. This has led to a decline in the number of recorded cases over the past 40 years.
Sporadic cases are recorded annually, mainly in elderly individuals, due to the long incubation times of the disease. In conclusion, every culture (and every individual) honors their loved ones as they prefer, but perhaps it is better to do it in a…healthy way.
Ilaria Salvatori
Bibliography
- Imran M, Mahmood S. An overview of human prion diseases. Virol J. 2011;8:559. Published 2011 Dec 24.
- Liberski PP, Gajos A, Sikorska B, Lindenbaum S. Kuru, the First Human Prion Disease. Viruses. 2019;11(3):232. Published 2019 Mar 7.
- Liberski PP. Kuru: a journey back in time from papua new Guinea to the neanderthals’ extinction. Pathogens. 2013;2(3):472‐505. Published 2013 Jul 18.
- Liberski PP, Sikorska B, Lindenbaum S, et al. Kuru: genes, cannibals and neuropathology. J Neuropathol Exp Neurol. 2012;71(2):92‐103.
- Sikorska B, Liberski PP. Human prion diseases: from Kuru to variant Creutzfeldt-Jakob disease. Subcell Biochem. 2012;65:457‐496.
- Soto C, Satani N. The intricate mechanisms of neurodegeneration in prion diseases. Trends Mol Med. 2011;17(1):14‐24.
- Whitfield JT, Pako WH, Collinge J, Alpers MP. Mortuary rites of the South Fore and kuru. Philos Trans R Soc Lond B Biol Sci. 2008;363(1510):3721‐3724.
- https://en.wikipedia.org/wiki/Fore_people
- https://www.sciencedirect.com/topics/medicine-and-dentistry/kuru-disease
- https://en.wikipedia.org/wiki/Kuru_(disease)
- https://www.pnas.org/content/105/10/3885.abstract
- http://www.salute.gov.it/imgs/C_17_normativa_1503_allegato.pdf
- https://www.msdmanuals.com/it/casa/disturbi-di-cervello,-midollo-spinale-e-nervi/malattie-da-prioni/kuru
- Disease Ecology: The rise and fall of kuru (eemb40.blogspot.com)
