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The eat is on: Cannibalism and sexual cannibalism (revisited)

Recently, I was approached by Ben Biggs, the editor of the Real Crime magazine, who was running an article on the practicalities and psychology of cannibalism, with expert commentary running through it (and with me as the “expert”). The article has just been published in the May 2016 issue and I was assured that the feature would “highlight how nasty cannibalism is, not glorify it”. I responded to the questions as part of an email interview and today’s blog contains the unedited responses to the questions that I was asked.

What are the main reasons a human might eat another human being?

There are a number of possible reasons including:

Out of necessity – For instance, in 1972, a rugby team from Uruguay was in a plane crash in the Andes. Fifteen people died and the only way they prevented themselves starving to death was to eat the flesh of the deceased (which given the fact it took 72 days for them to be rescued, was one of the few viable options to prevent starvation).

As a way of controlling population size – The Aztecs were said to have eaten no less than 15,000 victims a year as – some have argued – a form of population control).

As part of a religious belief – There are some religious beliefs involving the need to eat human flesh as a way of sustaining the universe or as part of magical and ritualistic ceremonies.

As part of the grieving process – Some acts of cannibalism are where dead people’s body parts are eaten as either part of the grieving process, as a way of guiding the souls of the dead into the bodies of the living, and/or as a way of imbibing the dead person’s ‘life force’ or more specific individual characteristics.

As part of tribal warfare – Cannibalistic acts were most often carried out as part of a celebration victory after battles with rival tribes.

For sexual gratification – Some individuals have claimed to get sexually aroused from eating (or thinking about eating) the flesh of others. When it comes to sexual cannibalism in humans, there are arguably different subtypes (although this is based on my own personal opinion and not on something I’ve read in a book or research paper). Most of these behaviours I have examined in previous blogs:

  • Vorarephilia is a sexual paraphilia in which individuals are sexually aroused by (i) the idea of being eaten, (ii) eating another person, and/or (iii) observing this process for sexual gratification. However, most vorarephiles’ behaviour is fantasy-based, although there have been real cases such as Armin Meiwes, the so-called ‘Rotenburg Cannibal’.
  • Erotophonophilia is a sexual paraphilia in which individuals have extreme violent fantasies and typically kill their victims during sex and/or mutilate their victims’ sexual organs (the latter of which is usually post-mortem). In some cases, the erotophonophiles will eat some of their victim’s body parts (usually post-mortem). Many lust murderers – including Jack the Ripper – are suspected of engaging in cannibalistic and/or gynophagic acts, taking away part of the female to eat later. Other examples of murderers who have eaten their victims (or parts of them) for sexual pleasure include Albert Fish, Issei Sagawa, Andrei Chikatilo, Ed Gein, and Jeffrey Dahmer.
  • Sexual necrophagy refers to the cannibalizing of a corpse for sexual pleasure. This may be associated with lust murder but Brenda Love in her Encyclopedia of Unusual Sex Practices says that such cases usually involve “one whose death the molester did not cause. Many cases of reported necrophilia include cannibalism or other forms of sadism and it is believed that many others fantasize about doing it”.
  • Vampirism as a sexual paraphilia in which an individual derives sexual arousal from the ingestion of blood from a living person.
  • Menophilia is a sexual paraphilia in which an individual (almost always male) derives sexual arousal from drinking the blood of menstruating females.
  • Gynophagia is a sexual fetish that involves fantasies of cooking and consumption of human females (gynophagia literally means “woman eating”). There is also a sub-type of gynophagia called pathenophagia. This is the practice of eating young girls or virgins. Several lust murderers were known to consume the flesh of young virgins, most notably Albert Fish).
  • ‘Sexual autophagy’ refers to the eating of one’s own flesh for sexual pleasure (and would be a sub-type of autosarcophagy).

A recent 2014 paper by Dr. Amy Lykins and Dr. James Cantor in the Archives of Sexual Behavior entitled ‘Vorarephilia: A case study in masochism and erotic consumption’ referred to the work of Dr Friedemann Pfafflin (a forensic psychotherapist at Ulm University, Germany): 

“Pfafflin (2008) commented on the many phrases that exist in the English language to relate sex/love and consumption, including referring to someone as ‘looking good enough to eat’, ’that ‘the way to a man’s heart is through his stomach’, and describing a sexually appealing person as ‘sweet’, ‘juicy’, ‘appetizing’, or ‘tasty’. Christian religions even sanction metaphorical cannibalism through their sacrament rituals, during which participants consume bread or wafers meant to represent the ‘body of Christ’ and wine intended to represent the ‘blood of Christ’ – a show of Jesus’s love of his people and, in turn, their love for him, by sharing in his ‘blood’ and ‘flesh’. This act was intended to ‘merge as one’ the divine and the mortal”.

It’s not unusual for a serial killer to cannibalise parts of their victims. Why is this, and what can cause that behaviour? 

I think it’s a rare behaviour, even among serial killers. As noted above, in these instances the eating (or the thought of eating) others is sexually arousing. It has also been claimed that the sexual cannibal may also release sexual frustration or pent up anger when eating human flesh. Some consider sexual cannibalism to be a form of sexual sadism and is often associated with the act of necrophilia (sex with corpses). Others have claimed that cannibals feel a sense of euphoria and/or intense sexual stimulation when consuming human flesh. All of these online accounts cite the same article by Clara Bruce (‘Chew On This: You’re What’s for Dinner’) that I have been unable to track down (so I can’t vouch for the veracity of the claims made). Bruce’s article claimed that cannibals had compared eating human flesh with having an orgasm, and that flesh eating caused an out-of-body-experience experience with effects comparable to taking the drug mescaline.

In the case of Japanese cannibal Issei Sagawa, he said that he might have been satisfied with consuming some, non-vital part of his victim Renee Hartevelt, such as her pubic hair, but he couldn’t bring himself to ask her for it. Does the murder and the consumption of flesh stem from the same mental disorder, or is murder just a necessary evil? 

I have not seen these claims. I have only read that his desire to eat women was to “absorb their energy”.

Do you think Issei Sagawa would have been satisfied with eating her hair?

Again, I have never read about this. He seems to have claimed that he had cannibalistic desires since his youth and that his murder of women was for this reason and no other.

Serial Killer Jeffrey Dahmer said he liked to eat mens’ biceps, because he was a ‘bicep guy’. Does the body part consumed necessarily bear a direct relation to the part of the victim’s anatomy the cannibal has a sexual preference for?

Not that I am aware of. Most people that are partialists (i.e., derive sexual arousal from particular body parts such has hands, feet, buttocks, etc.) would be unlikely to get aroused if the body part was not attached to something living.

There are rarer cases where, rather than having a fantasy of eating a sexual partner, the ‘victim’ consents to being eaten by the killer. Does this stem from the same psycho-sexual disorder that leads to a cannibal killing?

This is something entirely different and is part of vorarephilia (highlighted earlier). My understanding is that the flesh eating would only occur consensually (as in the case of Armin Meiwes and Bernd Jürgen Brand).

What reason would there be for someone to eat their own body parts? 

The practice is very rare and has only been documented a number of times in the psychological and psychiatric literature (and all are individual case studies). It has sometimes been labeled as a type of pica (on the basis that the person is eating something non-nutritive) although personally I think this is misguided as it could be argued that human flesh may be nutritious (even if most people find the whole concept morally repugnant). However, there are documented cases of autosarcophagy where people have eaten their own skin as an extreme form of body modification. Some authors argue that auto-vampirism (i.e., the practice of people drinking their own blood) should also be classed as a form of autosarcophagy (although again, I think this is stretching the point a little).

The practice has certainly come to the fore in some high profile examples in the fictional literature. Arguably the most infamous example, was in Thomas Harris’ novel Hannibal (and also in the film adaptation directed by Ridley Scott), where Hannibal ‘the Cannibal’ Lecter psychologically manipulates the paedophile Mason Verger into eating his own nose, and then gets Verger to slice off pieces of his own face off and feed them to his dog. In what many people see as an even more gruesome autosarcophagic scene, Lecter manages to feed FBI agent Paul Krendler slices of his own brain. In real life (rather than fiction), autosarcophagy is typically a lot less stomach churning but in extreme examples can still be something that makes people wince.

Depending on the definition of autosarcophagy used, the spectrum of self-cannibalism could potentially range from behaviours such as eating a bit of your own skin right through eating your own limbs. There are many reasons including for art, for the taste, for body modification, for protest (associated to mental illness), because they had taken mind-altering drugs, and for sexual pleasure. Here are four autosarcophagic examples that have been widely reported in the media but are very different in scope and the public’s reaction to them.

  • Example 1: Following a liposuction operation in 1996, the Chilean-born artist Marco Evaristti held a dinner party for close friends and served up a pasta dish with meatballs made from beef and the fatty liposuction remains. The meal was claimed by Evaristti to be an artistic statement but was highly criticized as being “disgusting, publicity-seeking and immoral”.
  • Example 2: On a February 1998 episode of the Channel 4 British cookery programme TV Dinners, a mother was shown engaging in placentophagy when she cooked her own placenta (with fried garlic and shallots), made into a pate and served on foccacia bread. The programme received a lot of complaints that were upheld by the British Broadcasting Standards Commission who concluded that the act of eating placenta pate on a highly watched TV programme had  “breached convention”.
  • Example 3: In 2009, Andre Thomas, a 25-year old murderer on Texas death row (and with a history of mental problems) pulled out his eye in prison and ate it.
  • Example 4: The German man Bernd Jürgen Brande who engaged in self-cannibalism (cutting off and then eating his own cooked penis) before being killed and eaten by Armin Meiwes, the ‘Rotenburg Cannibal’ (who also shared in the eating of Brande’s cooked penis).

Dr Friedemann Pfafflin (a forensic psychotherapist at Ulm University, Germany) and who has written about Armin Meiwes, the ‘Rotenburg Cannibal’ asserts that “apart from acts of cannibalism arising from situations of extreme necessity…the cannibalistic deeds of individuals are always an expression of severe psychopathology”.

Dr Mark Griffiths, Professor of Behavioural Addiction, International Gaming Research Unit, Nottingham Trent University, Nottingham, UK

Further reading

Aggrawal A. (2009). Forensic and Medico-legal Aspects of Sexual Crimes and Unusual Sexual Practices. Boca Raton: CRC Press.

Ahuja, N. & Lloyd, A.J. (2007). Self-cannibalism: an unusual case of self-mutilation. Australian and New Journal of Psychiatry, 41, 294-5.

Arens, William (1979). The Man-Eating Myth: Anthropology and Anthropophagy. Oxford: Oxford University Press.

Beier, K. (2008). Comment on Pfafflin’s (2008) “Good enough to eat”. Archives of Sexual Behavior, 38, 164-165.

Beneke M. (1999). First report of nonpsychotic self-cannibalism (autophagy), tongue splitting, and scar patterns (scarification) as an extreme form of cultural body modification in a western civilization. American Journal of Forensic Medicine and Pathology, 20, 281-285.

Benezech, M., Bourgeois, M., Boukhabza, D. & Yesavage, J. (1981). Cannibalism and vampirism in paranoid schizophrenia. Journal of Clinical Psychiatry, 42(7), 290.

Beier, K. (2008). Comment on Pfafflin’s (2008) “Good enough to eat”. Archives of Sexual Behavior, 38, 164-165.

Betts, W.C. (1964). Autocannibalism: an additional observation. American Journal of Psychiatry 121, 402-403.

Cannon, J. (2002). Fascination with cannibalism has sexual roots. Indiana Statesman, November 22. Located at: http://www.indianastatesman.com/vnews/display.v/ART/2002/11/22/3dde3b6201bc1

de Moore, G.M. & Clement, M. (2006). Self-cannibalism: an unusual case of self-mutilation. Australian and New Zealand Journal of Psychiatry, 40, 937.

Gates, K. (2000). Deviant desires: Incredibly strange sex. New York: Juno Books.

Huffington Post (2009). Andre Thomas, Texas Death Row inmate, pulls out eye, eats it. TheHuffington Post, September 9. Located at: http://www.huffingtonpost.com/2009/01/09/andre-thomas-texas-death-_n_156765.html

Krafft-Ebing, R. von (1886). Psychopathia sexualis (C.G. Chaddock, Trans.). Philadelphia: F.A. Davis.

Love, B. (2001). Encyclopedia of Unusual Sex Practices. London: Greenwich Editions.

Lykins, A.D., & Cantor, J.M. (2014). Vorarephilia: A case study in masochism and erotic consumption. Archives of Sexual Behavior, 43, 181-186.

Mikellides, A.P. (1950). Two cases of self-cannibalism (autosarcophagy). Cyprus Medical Journal, 3, 498-500.

Mintz, I.L. (1964). Autocannibalism: a case study. American Journal of Psychiatry, 120, 1017.

Monasterio, E. & Prince, C. (2011). Self-cannibalism in the absence of psychosis and substance use. Australasian Psychiatry, 19, 170-172.

Pfafflin, F. (2008). Good enough to eat. Archives of Sexual Behavior, 37, 286-293.

Pfafflin, F. (2009). Reply to Beier (2009). Archives of Sexual Behavior, 38, 166-167.

Prins, H. (1985). Vampirism: A clinical condition. British Journal of Psychiatry, 146, 666-668.

Reuters (1997). Meatballs made from fat, anyone? May 18. Located at: http://uk.reuters.com/article/2007/05/18/oukoe-uk-chile-artist-idUKN1724159420070518

Sunay, O. & Menderes, A. (2011). Self cannibalism of fingers in an alzheimer patient. Balkan Medical Journal, 28, 214-215.

Unlimited Blog (2007). Sexual cannibalism and Nithari murders. November. Located at: http://sms-unlimited.blogspot.co.uk/2007/11/sexual-cannibalism-and-nithari-murders.html

Wikipdia (2012). Cannibalism. Located at: http://en.wikipedia.org/wiki/Cannibalism

Wikipedia (2012). Sexual cannibalism. Located at: http://en.wikipedia.org/wiki/Sexual_cannibalism

Turn on the eating: A beginner’s guide to sexual cannibalism

“People who have consumed human blood and flesh reportedly claim to experience an intoxicating euphoric effect. This reaction is similar to that experienced by anyone who satisfies a strong sexual craving that is not considered normal (exhibitionism, necrophilia, rape, etc.). However, in this case, it must have reinforced the beliefs of worshippers that indeed their god was present in the victim” (Dr. Brenda Love, Encyclopedia of Unusual Sex Practices).

Today’s blog takes a brief look at sexual cannibalism in humans. I added “in humans” at the end of the sentence because sexual cannibalism is quite common in some animal species. As Dr. Brenda Love notes in her Encyclopedia of Unusual Sex Practices, sexual cannibalism is known to occur in some types of spider, praying mantis, scorpion, cricket, grasshopper, and fly. The Wikipedia entry also notes that sexual cannibalism has been observed in various types of crustacean (e.g., amphipods, copepods), slugs and snails (i.e., gastropods), and squids and octopuses (i.e., cephalopods). In the non-human species, it is typically the female that kills and eats the male before, during or after sexual union has taken place. Amongst humans, sexual cannibalism is extremely rare, and most humans who engage in cannibalistic acts for sexual purposes are generally considered sociopaths.

Of course, cannibalism for non-sexual purposes – known I more scientific circles as anthropophagy – has long been known among certain tribes and cultures. Throughout history, cannibalism has been practiced in many forms across Asia, Australia, Europe, and the Americas. Though rare today, it is believed to be still practiced in a few remote parts of Asia.  Cannibalism can be classed as either endocannibalism (i.e., consumption of another human being from within the same group or community) or exocannibalism (i.e., consumption of another human being from outside the group or community). Some acts of endocannibalism are actually acts of necro-cannibalism (i.e., the eating of flesh from dead humans also known as necrophagy) where dead people’s body parts are eaten as either part of the grieving process, as a way of guiding the souls of the dead into the bodies of the living, and/or as a way of imbibing the dead person’s ‘life force’ or more specific individual characteristics. Such endocannibalistic practices were common among certain tribes in New Guinea (which led to the prion disease kuru that I examined in a previous blog). However, it is known that many males among various tribes would not consume females for fear of emasculation. Exocannibalistic acts were most often carried out as part of a celebration victory after battles with rival tribes. There are various theories from many perspectives on why cannibalism may occur. These have included:

  • Religious theories (e.g., religious beliefs involving the need to eat human flesh as a way of sustaining the universe or as part of magical and ritualistic ceremonies).
  • Political theories (e.g., eating human flesh as a political tool to intimidate and control potential hostiles or subordinates).
  • Socio-psychological theories (e.g., eating human flesh due to unconscious factors such as a response to trauma).
  • Ecological theories (e.g., eating human flesh as a way of controlling the size of the population. The Aztecs were said to have eaten no less than 15,000 victims a year as – some have argued – a form of population control).
  • Dietary theories (e.g., eating human flesh as a source of protein).

There are of course other reasons (including sexual ones) that may be the root of someone’s cannibalistic desire to eat human flesh. One reason could be out of necessity. For instance, in 1972, a rugby team from Uruguay was in a plane crash in the Andes. Fifteen people died and the only way they prevented themselves starving to death was to eat the flesh of the deceased (which given the fact it took 72 days for them to be rescued, was one of the few viable options to prevent starvation). At its simplest level, human sexual cannibalism is usually considered a psychosexual disorder and involves individuals’ sexualizing (in some way) the consumption of another human being’s flesh. One online article claims that:

“This does not necessarily suggest that the cannibal achieves sexual gratification only in the act of consuming human flesh, but also may release sexual frustration or pent up anger. Sexual cannibalism is considered to be a form of sexual sadism and is often associated with the act of necrophilia (sex with corpses)”.

When it comes to sexual cannibalism in humans, there are arguably different subtypes (although this is based on my own personal opinion and not on something I’ve read in a book or research paper). Most of these behaviours I have examined in previous blogs (so click on the links if you want to know more:

  • Vorarephilia is a sexual paraphilia in which individuals are sexually aroused by (i) the idea of being eaten, (ii) eating another person, and/or (iii) observing this process for sexual gratification. However, most vorarephiles’ behaviour is fantasy-based, although there have been real cases such as Armin Meiwes, the so-called ‘Rotenburg Cannibal’.
  • Erotophonophilia is a sexual paraphilia in which individuals have extreme violent fantasies and typically kill their victims during sex and/or mutilate their victims’ sexual organs (the latter of which is usually post-mortem). In some cases, the erotophonophiles will eat some of their victim’s body parts (usually post-mortem). Many lust murderers – including Jack the Ripper – are suspected of engaging in cannibalistic and/or gynophagic acts, taking away part of the female to eat later. Other examples of murderers who have eaten their victims (or parts of them) for sexual pleasure include Albert Fish, Issei Sagawa, Andrei Chikatilo, Ed Gein, and Jeffrey Dahmer.
  • Sexual necrophagy refers to the cannibalizing of a corpse for sexual pleasure. This may be associated with lust murder but Brenda Love in her Encyclopedia of Unusual Sex Practices says that such cases usually involve “one whose death the molester did not cause. Many cases of reported necrophilia include cannibalism or other forms of sadism and it is believed that many others fantasize about doing it”.
  • Vampirism as a sexual paraphilia in which an individual derives sexual arousal from the ingestion of blood from a living person
  • Menophilia is a sexual paraphilia in which an individual (almost always male) derives sexual arousal from drinking the blood of menstruating females.
  • Gynophagia is (according to Dr. Anil Aggrawal’s 2009 book Forensic and Medico-legal Aspects of Sexual Crimes and Unusual Sexual Practices) a sexual fetish that involves fantasies of cooking and consumption of human females (gynophagia literally means “woman eating”). There is also a sub-type of gynophagia called pathenophagia. This (according to Dr. Brenda Love) is the practice of eating young girls or virgins. Several lust murderers were known to consume the flesh of young virgins, most notably Albert Fish). 

Added to this list, is something I would call ‘sexual autophagy’ which refers to the eating of one’s own flesh for sexual pleasure (and would be a sub-type of autosarcophagy discussed in a previous blog). I am basing this sub-type on an entry I came across in Brenda Love’s Encyclopedia of Unusual Sex Practices and relating to a case study reported by Krafft-Ebing:

“Krafft-Ebing recorded the case of a man who at 13 [years of age] became infatuated with a young white-skinned girl. However, instead of desiring intercourse, he was overwhelmed by the urge to bite off a piece of her flesh and eat it. He began stalking women, and for years he carried a pair of scissors with him. He was never successful in accosting a woman, but when he came close he would cut off and eat a piece of his own skin instead. This act produced an immediate orgasm for him”.

This account seems to be confirmed by some online articles on sexual cannibalism claiming that cannibals feel a sense of euphoria and/or intense sexual stimulation when consuming human flesh. All of these online accounts cite the same article by Clara Bruce (‘Chew On This: You’re What’s for Dinner’) that I have been unable to track down (so I can’t vouch for the veracity of the claims made). Bruce’s article claimed that cannibals had compared eating human flesh with having an orgasm, and that flesh eating caused an out-of-body-experience experience with effects comparable to taking the drug mescaline. In another publication that I’ve failed to track down, the following snippet appears on at least 20 websites with articles on sexual cannibalism:

“Lesley Hensel, author of ‘Cannibalism as a Sexual Disorder’ [says] eating human flesh can cause an increase in levels of vitamin A and amino acids, which can cause a chemical effect on the blood and in the brain. This chemical reaction could possibly lead to the altered states that some cannibals have claimed to have experienced. However, this theory has not been substantiated by scientific evidence”.

As I’ve covered many of the cannibalistic sub-types in previous blogs, I tried to do some further research on gynophagia. There is almost nothing written from an academic or clinical perspective about gynophagia (in fact when I typed in ‘gynophagia’ only one reference turned up – a paper on ‘the psychophysical basis of feelings’ published by Dr. C.L. Herrick in an 1892 issue of the Journal of Comparative Neurology that only mentioned gynophagia in passing). However, there are quite a few dedicated gynophagia websites out there including dedicated pages on the Deviant Art website and an interesting set of cannibalistic links (that you can check out for yourself on the Indie Film website. There is also a reasonably lengthy article in the Urban Dictionary but it features little of any substance. The person writing the article makes the following observations:

“Gynophagia is the fetish of a person becoming food for someone else as a fantasy. As a fantasy it’s just as taboo as BDSM or other kinks…Gynophagia can really be a more gentle fetish than BDSM because torture is almost never applied. Honestly, when you boil it down to its essentials (no pun intended), gynophagia is an extension of the ‘Damsel in Distress’ scenario…Gynophagia is present in a lot of the older media we have, the most widely recognized being a helpless woman being boiled alive by a native tribe when the hero rescues her. Another example would be in Little Red Riding Hood where the wolf devours Red Riding Hood, but this could also be classified as a separate but similar fetish called Vorarephillia. One of the more widely known scenarios of gynophagia is known as the Dolcett method which usually centers around the main female character of a Dolcett comic being spit roasted alive and enjoying every moment of it. But again I must stress that gynophagia is one of those few fetishes that can only be a fantasy and should not be practiced in real life”.

If you really want to find out what gynophagia disciples are into, I suggest you check out the Carnal Consummations fetish website (but you’ve been warned!).

Dr Mark Griffiths, Professor of Gambling Studies, International Gaming Research Unit, Nottingham Trent University, Nottingham, UK

Further reading

Aggrawal A. (2009). Forensic and Medico-legal Aspects of Sexual Crimes and Unusual Sexual Practices. Boca Raton: CRC Press.

Arens, William (1979). The Man-Eating Myth: Anthropology and Anthropophagy. Oxford: Oxford University Press.

Beier, K. (2008). Comment on Pfafflin’s (2008) “Good enough to eat”. Archives of Sexual Behavior, 38, 164-165.

Benezech, M., Bourgeois, M., Boukhabza, D. & Yesavage, J. (1981). Cannibalism and vampirism in paranoid schizophrenia. Journal of Clinical Psychiatry, 42(7), 290.

Cannon, J. (2002). Fascination with cannibalism has sexual roots. Indiana Statesman, November 22. Located at: http://www.indianastatesman.com/vnews/display.v/ART/2002/11/22/3dde3b6201bc1

Krafft-Ebing, R. von (1886). Psychopathia sexualis (C.G. Chaddock, Trans.). Philadelphia: F.A. Davis.

Love, B. (2001). Encyclopedia of Unusual Sex Practices. London: Greenwich Editions.

Pfafflin, F. (2008). Good enough to eat. Archives of Sexual Behavior, 37, 286-293.

Pfafflin, F. (2009). Reply to Beier (2009). Archives of Sexual Behavior, 38, 166-167.

Prins, H. (1985). Vampirism: A clinical condition. British Journal of Psychiatry, 146, 666-668.

Unlimited Blog (2007). Sexual cannibalism and Nithari murders. November. Located at: http://sms-unlimited.blogspot.co.uk/2007/11/sexual-cannibalism-and-nithari-murders.html

Wikipdia (2012). Cannibalism. Located at: http://en.wikipedia.org/wiki/Cannibalism

Wikipedia (2012). Human sacrifice in Aztec culture. Located at: http://en.wikipedia.org/wiki/Human_sacrifice_in_Aztec_culture

Wikipedia (2012). Sexual cannibalism. Located at: http://en.wikipedia.org/wiki/Sexual_cannibalism

Brain food: A beginner’s guide to kuru

In a previous blog, I examined the scientific literature on fatal familial insomnia (FFI), an incredibly rare genetic sleep disorder cause caused by a genetic mutation that leads to prion disease. Today’s blog takes a brief look at another prion disease – ‘kuru’. Like FFI, kuru is also an incurable and degenerative neurological disorder (i.e., a transmissible spongiform encephalopathy) although the only people known to have experienced it are a few cannibalistic tribes in the Eastern Highlands Province of New Guinea (most notably the Fore tribe) where it is known as the ‘laughing sickness’ or the ‘laughing disease’ (that refer to one of the disease’s most noticeable symptoms – the hysterical and pathological outbursts of laughter that suffering individuals produce in the latter stages of the disease).

The disease has a relatively long incubation period (5 to 20 years, with an average of 10 to 13 years according to a study led by Dr. Jerome Huillard d’Aignaux in a 2002 issue of the journal Epidemiology. However, a study published by Dr. R.L. Klitzman and colleagues in an earlier issue of Neuroepidemiology reported that:

“Epidemiological data were collected on…65 kuru patients who died or were diagnosed between 1977 and 1981. From these…2 or more participants were exposed to the infectious agent for the first time and died within weeks or months of each other 25–30 years later. Thus, it is shown that the natural incubation period of kuru could be as long as 25–30 years”

A more recent paper by Dr. John Collinge and colleagues in The Lancet identified 11 kuru sufferers from July 1996 to June 2004 all living in the South Fore. They reported that the minimum estimated incubation periods ranged from 34 to 41 years. However, they also noted that the likely incubation periods in men ranged from 39 to 56 years and could have been up to 7 years longer. Therefore, incubation periods could be very long.

According to Dr. Robert Will (in a 2003 issue of the British Medical Bulletin), over 2700 cases of kuru have been recorded since 1957 (in a total population within the kuru region of 36,000 people). Those infected with kuru typically die between 3 and 24 months following the first symptoms. Because of the long incubation period, it is thought that the last person to die of kuru in New Guinea was only seven years ago (i.e., 2005).

The word ‘kuru’ (not to be confused with ‘koro’, the culture bound genital retraction syndrome typically found in south-east Asia) is derived from the Fore tribe’s word ‘to shake’ (‘kuria’). Research carried out in the 1960s and 1970s demonstrated beyond doubt that kuru was transmitted from one individual to another via the cannibalistic practices of the Fore tribe (particularly the South Fore of the Okapa Subdistrict that showed the most large and notable kuru infection rates). The first recorded cases of kuru were made by a number of Australians back in the early 1950s. W.T. Brown reported that

“The first sign of impending death is a general debility which is followed by general weakness and inability to stand. The victim retires to her house. She is able to take a little nourishment but suffers from violent shivering. The next stage is that the victim lies down in the house and cannot take nourishment and death eventually ensues”.

It was in the early 1960s at the Eastern Highlands Awande Hospital that kuru sufferers underwent medical research in an effort to locate the cause of the disease. It was the pioneering work Daniel Gajdusek and Michael Alpers that led to the discovery of the causative agent of kuru. Brain tissue samples were taken from an 11-year old girl who had died of kuru and subsequently injected into a couple of chimpanzees. One of the chimps developed kuru within 24 months, and the research showed unequivocally that kuru was capable of infection cross-species. According to Dr. Robert Will, “this seminal discovery led to the successful laboratory transmission of [Creutzfeldt-Jakob disease] and initiated research into the epidemiology and pathogenesis of human prion disease”

It is now generally believed that the kuru outbreak began following the consumption of an infected human brain with sporadic Creutzfeldt-Jakob disease (CJD) by people in the Fore tribe in around 1900. Kuru then spread to other nearby tribes following inter-tribe marrying (gradually spreading to the 169 villages and hamlets according to a 2010 paper by Dr. Nils Pedersen and Dr. Else Smith in a 2010 issue of Acta Pathologica Micobiologica Et Immunologica Scandinavica). Once infected with kuru, the disease has three distinct phases (ambulant, sedentary, and terminal):

  • Ambulant phase: Symptoms typically include decreased muscle and motor control leading to an unsteady gait and stance, tremors, and deterioration and slurring of speech (dysarthria).
  • Sedentary phase: Symptoms typically include deterioration of muscle coordination (ataxia) and severe tremors. Sufferers are unable to walk unaided and they suffer bouts of hysterical and uncontrolled laughter.
  • Terminal phase: Symptoms typically include complete loss of muscle co-ordination, incontinence, being unable to talk or sit unaided, great difficulty in swallowing food (dysphagia) (difficulty swallowing), and the outbreak of necrotic ulcerations (sores with pus).

The early research by Michael Alpers and colleagues showed that kuru spread very rapidly as a result of the Fore tribe’s endocannibalistic practice (i.e., of eating the flesh of human beings from within the same community after the person had died and taking on that individual’s ‘life force’). Interestingly, Alper’s research also showed that kuru infection was far more prevalent in women and children (in fact, up to 9 times more prevalent). Dr. Pedersen and Dr. Smith noted that the youngest ever kuru sufferer was five years old, and that 67% of those with kuru were adult women, 23% were children and adolescents, and only 10% were adult men.

There are two reasons why kuru might be more prevalent among women and children compared to men. Firstly, men in the tribe had first choice of which parts of the infected dead tribe member to eat. Once the men had eaten the ‘choice cuts’, women and children could only eat what was left and this included the dead person’s brain (where the infected prion particles were at their most concentrated). Secondly, women and children were far more likely than men to clean the bodies of the infected dead people. If those cleaning the body had open cuts or sores on their hands, the infection may have spread through the bloodstream. The 2002 research led by Dr. Jerome Huillard d’Aignaux and colleagues also demonstrated that the incubation period in females was shorter than that in males because adult women may have been exposed to the largest doses of infectious material. However, Dr. Robert Will has noted that as time has passed, the incidence of kuru has declined and the proportion of affected adult males and females has become more similar. Research (for example by Dr. Simon Mead) has also indicated that some members of the Fore tribe were immune from catching kuru as they carried a prion-resistant factor. Dr. Will also reported that:

“No children born after 1959 have been affected and there is no evidence of vertical transmission of infectivity in kuru, despite the breast-feeding of infants by many hundreds of clinically affected mothers”.

Most recent papers on kuru (such as one by Dr. Laura Manuelidis and colleagues in a 2009 issue of Proceedings of the National Academy of Sciences) have noted that the disease has now died out due to the cessation of the endocannibalistic rituals and therefore is not created spontaneously by the human host.

Dr Mark Griffiths, Professor of Gambling Studies, International Gaming Research Unit, Nottingham Trent University, Nottingham, UK

Further reading

Collinge, J., Whitfield, J.W., McKintosh, E., Beck, J., Mead, S., Thomas, D.J., & Alpers, M.P. (2006). Kuru in the 21st century—an acquired human prion disease with very long incubation periods. The Lancet, 367, 2068-2074.

Gajdusek, D.C., Gibbs, C.J. & Alpers, M. (1966). Experimental transmission of a Kuru-like syndrome to chimpanzees. Nature, 209, 794-796.

Gajdusek, D.C & Zigas, V. (1957). Degenerative disease of the central nervous system in New Guinea. The epidemic occurrence of ‘‘Kuru’’ in the native population. New England Journal of Medicine, 257, 974-978.

Huillard d’Aignaux, J.N., Cousens, S.N., Maccario, J., Costagliola, D., Alpers, M.P., Smith, P.G., Alpérovitch, A. (2002). The incubation period of kuru. Epidemiology, 13, 402-408.

Klitzman, R.L., Alpers, M.P. & Gajdusek, D.C. (1984). The natural incubation period of kuru and the episodes of transmission in three clusters of patients. Neuroepidemiology, 3, 3-20.

Lindenbaum, S. (1979). Kuru sorcery: Disease and danger in the New Guinea highlands. Palo Alto, CA: Mayfield.

Manuelidis, L., Chakrabarty, T., Miyazawa, K., Nduom, N. & Emmerling, K. (2009). The kuru infectious agent is a unique geographic isolate distinct from Creutzfeldt–Jakob disease and scrapie agents Proceedings of the National Academy of Sciences, 106, 13529-13534.

Pedersen, N.S. & Smith, E. (2010). Prion diseases: Epidemiology in man. Acta Pathologica Micobiologica Et Immunologica Scandinavica, 110, 14-22.

Will, R.G. (2003). Acquired prion disease: iatrogenic CJD, variant CJD, kuru. British Medical Bulletin, 66, 255-265.

Dead tired: A beginner’s guide to Fatal Familial Insomnia

For most of my life I have “suffered” from insomnia. I deliberately put the word ‘suffered’ in quotation marks as for the vast majority of the time I have always seen my lack of sleep as something positive (i.e., I had more time to do other things. In fact, when people ask me how I find the time to write so much, I usually say “Insomnia” but I don’t usually say it as a joke, it’s a matter of fact). Given my personal interest in insomnia, I’ve always enjoyed reading papers on insomnia (and no, they don’t send me to sleep!) and sexsomnia (which I looked at in a previous blog). In 1990, a Finnish man named Toimi Soini stayed awake for over 11 days (276 hours) and broke the world record for not going to sleep. However, this record no longer appears in the Guinness Book of World Records as it was withdrawn on health grounds because lack of sleep – as I’ll show in today’s blog – can lead to death.

One of the strangest (and deadliest) types of insomnia is ‘fatal familial insomnia’ (FFI). This is actually an incredibly rare genetic sleep disorder that affects around 40 families worldwide. The cause of FFI is a genetic mutation that leads to prion disease and is therefore related to bovine spongiform encephalopathy (BSE; i.e., ‘mad cow disease’), Creutzfeldt–Jakob disease (the human form of BSE), and ‘Kuru’ (the incurable and degenerative neurological disorder found in the cannibalistic tribes in New Guinea and known as the ‘laughing disease’). The (online) Medical Dictionary is a little more technical and notes:

“Fatal familial insomnia (FFI) is a very rare, autosomal dominant inherited, disease of the brain. It is caused by a mutation in a protein called prion protein (PrP): asparagine- 178 is replaced by aspartic acid. The mutation changes the shape of PrP so that it becomes a prion and makes other, normal PrP molecules change to the abnormal shape. This causes amyloid plaques in the thalamus, the region of the brain responsible for regulation of sleep patterns. The dysfunction of the thalamus results in insomnia first of all, which progresses to more serious problems over several years”

All prion diseases (known more scientifically as ‘transmissible spongiform encephalopathies’) are rare progressive neurodegenerative disorders that can affect both animals and humans. All of the prion diseases (including FFI) typically have (i) long incubation periods, (ii) a failure to induce inflammatory response, and (iii) characteristic spongiform changes that are associated with neuronal loss. The first recorded case of FFI is thought to be an Italian man who died in Venice in 1765. There are many descriptions of the disease online including case study accounts. The Wikipedia entry on FFI described the case of the American music teacher, Michael Corke from Chicago:

“He suddenly began to have trouble sleeping not long after his 40th birthday in 1991, and his health and state of mind quickly deteriorated as his sleeplessness grew worse. Eventually, he couldn’t sleep at all, and he was soon admitted to the hospital. Doctors there weren’t sure what was wrong with him, initially diagnosing multiple sclerosis; in a bid to send him to sleep in the later stages of the disease, physicians induced a coma with the use of sedatives, but they found that his brain still failed to shut down. Corke died in 1992 a month before his 41st birthday, by which time he had gone without sleep for six months”

Another 2011 online article on “bizarre brain disorders” by Anna McGann also described a family case study (which is very similar to paper published in a 2000 issue of the Journal of Neurology, Neurosurgery and Psychiatry by Dr. C. Tabernero and colleagues):

“Dr. Ignazio Rottier gained unwanted firsthand experience when he and his wife, Elisabetta, watched her family fall victim to [FFI]. First known to fall ill was Elisabetta’s grandfather. Decades later, Elisabetta’s uncle, Silviano, was 53 when he lost his ability to sleep. A few short months following initial onset, Silviano fell into a coma and died…In the 70s, an aunt of Elisabetta’s passed on, one year after her own initial onset of sleeplessness. Yet another year later, a second aunt too lost her life battling the very same affliction”.

Research has also shown that the condition (in a few cases) can result from a non-inherited genetic mutation that has been called ‘sporadic fatal insomnia’ (sFI). Less than 10 cases of sFI have ever been documented in the medical literature. As the conditions worsen, sufferers experience a wide range of symptoms including delirium, hallucinations (auditory, visual and tactile), elevated heart rate and blood pressure, hyperhidrosis (i.e., excess sweating), hyperthermia, hypertension, impotence (in men), amenorrhea (cessation of periods) and early menopause (in women), constipation, and dementia. Treating the symptoms (via vitamin therapy, meditation, use of narcoleptics) may extend the quality of life (but as noted above, there is no known cure and most interventions are purely palliative). The disease typically has four stages, and takes between half a year and a year and a half to run its course:

  • Stage 1 (typically four months): Symptoms include insomnia, paranoia, phobias and panic attacks.
  • Stage 2 (typically five months): Symptoms include severe hallucinations and increasing panic attacks.
  • Stage 3 (typically three months): Symptoms include permanent insomnia, limited mental functioning, and rapid weight loss.
  • Stage 4 (typically six months): Symptoms include dementia and general non-responsiveness leading to death.

Writing in a 2006 issue of the Medscape General Medicine journal, Dr. Joyce Schenkein outlined the etiology and characteristics of FFI. She noted that it often begins in middle age (average age of onset being 50 years) and has no cure (even ‘gene therapy has been unsuccessful to date). Unfortunately, the prognosis following initial diagnosis is poor with FFI sufferers’ only living for an average of about a year and a half (with Dr. Schenkein noting that survival ranged from 7 to 36 months from diagnosis of FFI). It originates in the form of unexplained sleeplessness before rapidly developing into a fatal insomnia. Writing in an issue of the Journal of Clinical Neuroscience, Dr. S. Collins and colleagues in a paper on prion diseases (including FFI) concluded:

“FFI [is] likely [to] remain, [a] very rare disease, [and] will be increasingly recognised as heightened clinical awareness prompts appropriate confirmatory genetic and other testing. Similarly, continued molecular biological and allied research of these less common prion diseases will undoubtedly provide fundamental insights into the pathogenesis of this group of disorders in general, disproportionate to their numerical frequency”.

Dr Mark Griffiths, Professor of Gambling Studies, International Gaming Research Unit, Nottingham Trent University, Nottingham, UK

Further reading

Collins, S., McLean, C.A. & Masters, C.L. (2001). Gerstmann–Sträussler–Scheinker syndrome, fatal familial insomnia, and kuru: a review of these less common human transmissible spongiform encephalopathies. Journal of Clinical Neuroscience, 8, 387–397.

McGann, A. (2011). 5 bizarre brain disorders. Suite 101, November 25. Located at: http://suite101.com/article/5-bizarre-brain-disorders-a397906

Moody, K.M., Schonberger, L.B., Maddox, R.A., Zou, W.Q., Cracco, L., & Cali, I. (2011). Sporadic fatal insomnia in a young woman: a diagnostic challenge: case report. BMC Neurology, 11, 136.

Schenkein, J. (2006). Self-management of fatal familial insomnia. Part 1: What Is FFI? Medscape General Medicine, 8(3), 65.

Schenkein, J. & Montagna, P (2006). Self-management of fatal familial insomnia. Part 2: Case report. Medscape General Medicine, 8(3), 66.

Tabernero, C., Polo, J.M., Sevillano, M.D., Muñoz, R., Berciano, J., Cabello, A., Báez, B., Ricoy, J.R., Carpizo, R., Figols, J., Cuadrado, N., Claveria, L.E. (2000). Fatal familial insomnia: clinical, neuropathological, and genetic description of a Spanish family. Journal of Neurology, Neurosurgery and Psychiatry, 68, 774–777.

Turner, R. (2012). Fatal Familial Insomnia: 
The FFI Sleep Disorder. World of Lucid Dreaming. Located at: http://www.world-of-lucid-dreaming.com/fatal-familial-insomnia.html

Wikipedia (2012). ‪Fatal familial insomnia‬. Located at: http://en.wikipedia.org/wiki/Fatal_familial_insomnia