Young blood: A brief look at ‘Orphan’ and the ‘evil child’ trope in horror films

(Please be warned, this article contains spoilers if you have not watched the films The Bad Seed and Orphan).

Regular readers of my blog know that I enjoy watching horror movies and I’ve written articles on why people enjoy watching horror movies, a look at scary clowns in film and television, as well as more direct and indirect in-depth looks at my personal favourites including the Hannibal Lecter and Alien franchises.

One of the most popular tropes in the horror genre is the ‘evil child’ (often referred to or seen as equivalent to the ‘demonic child’, ‘creepy child’, ‘bad seed’ and ‘demon seed’ trope). This has spawned dozens on online articles looking at celluloid examples of the evil child sub-genre such as ‘Top 25 Evil Child Movies’, ‘Evil Kid Horror Movies’, ‘16 Creepy Child Horror Movies That Will Make You Not Want Kids Ever’, ‘The Top 10 Most Evil Children In Movies’, ‘We’re Baaack: The 20 Most Evil Children From TV And Movies’, and ‘The 50 Spookiest Kids In Horror Movies, Ranked’ (to name just a few).

The film that arguably started the trope was Mervin LeRoy’s 1956 horror-thriller The Bad Seed. The film was based upon Maxwell Anderson’s play of the same name (itself based on the 1954 novel The Bad Seed by William March and – for you trivia fans – the inspiration for the name of Nick Cave’s band The Bad Seeds). The ‘demon child’ of both the book and the film is sociopath Rhoda Penmark, whose mother (Christine) – spoiler alert – learns that she is an adopted child and is the biological daughter of Bessie Denker, an infamous serial killer (and believes that she genetically caused Rhoda’s sociopathy).

As a teenager, the demonic child that had most impact on me was Damien Thorn (in Damien: Omen II) mainly because I shared my middle name with the titular character. However, there are hundreds to choose from that share many of Damien’s chilling characteristics (some horror and some not) including Joshua Cairn (Joshua), Dalton Lambert (Insidious), Lilith Sullivan (Case 39), Brahms (The Boy), Regan MacNeil (The Exorcist), Nicholas and Ann Stewart (The Others), Tomás (The Orphanage), Henry Evan (The Good Son), Delia (The Omen IV), Kevin (We Need To Talk About Kevin), Toshio (Ju-On/The Grudge), Samara (The Ring), Santi (The Devil’s Backbone), The Grady twins (The Shining), and Gage Creed (Pet Sematary). In addition to this there are those films where there are a group of demonic children (e.g., Children Of The Damned, Children Of The Corn, and the ‘psychoplasmic offspring’ of The Brood), as well as ‘demon seed’ children that are yet to be born (e.g., Rosemary’s Baby, The Omen, etc.).

When it comes to ‘evil child’ films, one of my more recent favourites (at least in terms of the film’s twist at the end) is the 2009 US psychological horror film Orphan (directed by Jaume Collet-Serra). When it comes to horror films I much prefer ‘psychological horror’ (which tends to be rooted in reality and is why I like the Hannibal Lecter franchise) as opposed to supernatural thrillers and the archetypal ‘slasher films’ (although I do like watching gory films). Orphan centres on married couple John and Kate Coleman (played by Peter Sarsgaard and Vera Farmiga) who after the death of their unborn baby adopt Esther, a nine-year old Russian girl from an orphanage (played by Isabelle Fuhrman).

In the scene where a provocatively-dressed nine-year old Esther attempts to seduce her new father (who had been drinking heavily) I began to guess the twist in the film that – spoiler alert – Esther was (because of a hormone disorder called hypopituitarism) a woman in a child’s body. Near the end of the film, it is Esther’s new mother (Kate) that receives a call from the Saarne Institute (a mental institution) and is informed that Esther is in fact a violent murderer from Estonia called Leena Klammer, a 33-year-old woman whose physical growth was stunted and had spent most of her adult life posing as a young girl and had killed at least seven people (including the father of an Estonian family who adopted her and who she killed for rejecting her sexual advances). According to the IMDb ‘Trivia’ page about Orphan:

“Earlier drafts of the script include more information about Esther’s past and explain why she attempts to seduce her adoptive fathers. She was molested by her father for years, starting when she was an infant; this sexualized her at a very young age and destroyed any future chance of her having her own children. Her father later took another lover, telling Esther that, because of her condition, she could never be a real woman. She murdered them both and was ultimately sent to Saarne, a mental institution. After escaping from Saarne, she worked as a prostitute in Estonia for years, mostly catering to wealthy pedophiles. When she was arrested for this, she kept up the pretense of being a child to stay out of jail and was sent to an orphanage. Esther sees herself as trapped inside the body of a child, and it disgusts her. She wants to ‘grow up’ and be a wife, a mother, and a lover (what her father considered a ‘real woman’), and tries to find ‘love’ with her father but she didn’t”.

After the film had been released, there was a lot of debate about whether the medical condition that Esther had really exists. According to Wikipedia entry on the condition:

“Hypopituitarism is the decreased (hypo) secretion of one or more of the eight hormones normally produced by the pituitary at the base of the brain…The signs and symptoms of hypopituitarism vary, depending on which hormones are undersecreted and on the underlying cause of the abnormality…Hypopituitarism is a rare disease but may be significantly underdiagnosed in people with previous traumatic brain injury…The first description of the condition was made in 1914 by the German physician Dr. Morris Simmonds”.

Not only does Esther’s medical condition exist, but her character was actually inspired by the true life case of Barbora Skrlova who was one of the individuals in a 2008 story that the Daily Mail entitled ‘Boy ‘skinned and eaten’ by his cannibal cult family after being held captive in a cellar’. The story in question was a disturbing case involving single parent Klara Mauerova (described as an aggressive schizophrenic) and the physical abuse of her two sons (Yakub and Ondrej). The story was recounted in a 2017 article on the Mundo.com website entitled ‘Barbora Skrlová: The woman who inspired the movie Orphan’. From what I’ve read, Mauerova became depressed after the father of her children left her and she asked her sister (Katerina, who also appears to have had some kind of mental illness) to move in with her to help her look after the children. According to Mundo.com:

“The sisters met Barbora Skrlová at the university, a 33-year-old woman who looked [like a] 13 [year-old] girl because of a difficult disease called hypopituitarism. [Skrlová] was really skilled manipulating, and that’s what she did with the two sisters, they became really good friends because of her tragic childhood stories, she made the sisters take her to live with them. Years before meeting Klara and Katerina, [Skrlová] had been hospitalized for several years in a psychiatric center because she had made herself known as an orphaned child to a family that wanted to adopt her, but they realized about it and sent her to an asylum”.

The story alleged that Skrlová and the Mauerova sisters imprisoned Klara’s two sons (naked) in an iron cage in the basement of their house. It was also alleged that Skrlová wanted “to fatten [the two boys] just as Hansel and Gretel and wanted to commit cannibal acts while filming with a camera”. According to the Daily Mail story:

“An eight year-old boy was skinned and his flesh fed to cannibal relatives after his mother kept him locked in a cellar… Evil Klara Mauerova – a member of a sinister religious cult – wept in court as she admitted torturing her son Ondrej and his ten year-old brother Jakub. The court also heard allegations that relatives had partially skinned eight-year-old Ondrej and then eaten the raw human flesh. The two boys told how their mother and relatives had stubbed cigarettes out on their bare skin, whipped them with belts and tried to drown them. The court heard how the family had sexually abused them and even made them cut themselves with knives. They said they were kept in cages or handcuffed to tables and made to stand in their own urine for days”.

A neighbour alerted the police that there was something suspicious going on in the Mauerova household (having picked up what was happening on his baby monitor). When they police eventually arrived they discovered “the worst scenes they had ever seen” in the Mauerova’s basement. They found the two naked boys in the cage alongside a “little girl crying” (i.e., Barbora Skrlová). Skrlová told the police that her name was ‘Anika’ and that she had been adopted by the Mauerova sisters. The ‘little girl’ was taken to a children’s home by the police but absconded the same night. She was later found many months later living with another couple who had adopted her (but this time as a boy called Adam and described by the couple as a ‘child genius’ who suffered severe anxiety and depression attacks). Skrlová was sentenced to 12 years in prison but released in 2012. Her whereabouts are currently unknown.

Given that the orphan in the titular film was eventually exposed as an adult, it could be argued that the film is not technically about an ‘evil child’ and therefore not part of the ‘evil child’ trope (but I think that’s pedantry and misses the point). For almost all of the film, the audience believes Esther to be a child and on that basis alone it belongs to the ‘evil child’ horror genre. As plot twists go, I think it was one of the better ones, up there with The Usual Suspects, The Crying Game, and The Sixth Sense (which I won’t spoil just in case there are a few of you reading this that haven’t seen these three films).

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

Further reading

Ananvisca, V. (2017). Barbora Skrlová: The woman who inspired the movie Orphan. Mundo.com, June 17. Located at: https://en.mundo.com/most_viewed/barbora-skrlova-the-woman-who-inspired-the-movie-orphan/

Daily Mail (2008). Boy ‘skinned and eaten’ by his cannibal cult family after being held captive in a cellar. June 21. Located at: https://www.dailymail.co.uk/news/article-1027962/Boy-skinned-eaten-cannibal-cult-family.html

International Movie Database (2018). Orphan trivia. Located at: https://www.imdb.com/title/tt1148204/trivia

Orphan Wiki (2018). Leena Klammer. Located at: http://orphan-movie.wikia.com/wiki/Leena_Klammer

Villians Wiki (2018). Esther Coleman. Located at: http://villains.wikia.com/wiki/Esther_Coleman

Wikipedia (2018). Hypopituitarism. Located at: http://en.wikipedia.org/wiki/Hypopituitarism

Glum drone pleasures: The psychology of Ian Curtis and Joy Division

“Now there’s a really good book…[by French economist] Jacques Attali wrote in the late [1970s] called ‘Noise: The Political Economy of Music’…and the main tenet of that book is that…music is the best form of prophecy that we have…so that working with music or sound is our best way of divining a future, and being able to show to ourselves what’s round the corner in that psychological, or even psychic sense” (writer and graphic designer Jon Wozencroft being interviewed for the 2007 film Joy Division)

As a poverty stricken teenager in the early 1980s, all of my minimal disposable income was spent on buying records, cassettes, and music magazines (and to be honest, 35 years later nothing much has changed except I now buy far too many CDs instead of cassettes). Unlike most of my friends at the time I refused to be pigeon holed as a new romantic, a punk, a mod, or a goth because I liked music from all those genres. In the early 1980s was as equally as likely to buy a record by Adam and the Ants and Bauhaus as I was to buy records by Secret Affair and The Clash. I was also into city music scenes with my favourites being the ‘Liverpool scene’ (Echo and the Bunnymen, Teardrop Explodes, Wah! etc.), the ‘Sheffield scene’ (Human League, Heaven 17, Cabaret Voltaire, etc.), and the ‘Manchester scene’ (Magazine, Buzzcocks, Joy Division, The Smiths, The Passage, etc.).

The Manchester music scene was incredibly buoyant although often portrayed by the music press at the time as psychologically and emotionally ‘miserablist’. My parents could never understand what I saw in the “depressing and alienating music” (as they saw it) of bands like Joy Division and The Smiths. But it was through these bands that I developed an interest in psychology and what could be described as ‘psychgeography of post-punk’. In the case of Joy Division, their geographical location in Manchester and its surrounding area (Salford, Macclesfield) was integral to their music. In fact, a number of commentators (such as Liz Naylor, the co-editor of City Fun fanzine) have asserted that Joy Division “relayed the aura of Manchester” in the late 1970s and early 1980s.

All of my information about Joy Division came from reading the NME, listening to the John Peel Show on Radio 1, and listening to their two studio LPs (Unknown Pleasures and Closer) and assorted singles (that I mainly taped off the radio as most of them were not widely available). I was too young to go to gigs and they rarely appeared on television. Of the four members of Joy Division – Ian Curtis (vocals), Peter Hook (bass guitar), Bernard ‘Barney’ Sumner (guitar), and Stephen Morris (drums) – it was Curtis that captivated my adolescent attention. It was through Curtis’ documented medical conditions that helped develop my interest in psychology. Curtis suffered from epilepsy (like one of musical heroes Jim Morrison of The Doors) and clinical depression. It has also been alleged that he suffered from bipolar disorder (i.e., what used to be called ‘manic depression’) although this was never formally diagnosed (and many of those close to Curtis claim that such a claim is speculative at best).

Descriptions of Curtis’ behaviour on first sight look like bipolar disorder given the reports by his wife and others of his severe mood swings (where on one day he could have feelings of happiness and elation but on the next day could have feelings of intense depression and despair). However, other members of the band claimed that the mood swings were caused by the epilepsy medication Curtis was taking. However, bipolar disorder is not uncommon among musicians given many other high profile rock and pop stars have suffered from it including Brian Wilson (Beach Boys), Syd Barrett (Pink Floyd), Kurt Cobain (Nirvana), Ray Davies (The Kinks), Sinéad O’Connor, Poly Styrene (X-Ray Spex), and Adam Ant (to name just a few). Curtis was never afraid to write about psychological and medical conditions and the song ‘She’s Lost Control’ is arguably the most insightful song ever written about epilepsy (based not on his own experiences, but his observations of a female epileptic client who died while he was an Assistant Disablement Resettlement Officer based at the Job Centre in Macclesfield).

As any Joy Division fan knows, as a result of his severe depression, Curtis committed suicide by hanging himself on May 18, 1980 (a date I always remember because it was my favourite gran’s birthday), just two days before Joy Division were due to go on their first US tour. Even as a 14-year old teenager, I remember going to my local library in Loughborough not long after his death to learn more about depression, epilepsy, suicide, and attempted suicide (as he had two previous attempts to commit suicide earlier that year). I’m not saying that this alone was responsible for my career choice but it certainly facilitated my growing interest in psychology and mental health issues.

It was also through Joy Division that I started to read history books (and still do) on various psychological and non-psychological aspects of Nazism (and is evidenced by my previous blogs on the personality of Adolf Hitler and Nazi fetishism). Back in the late 1970s and early 1980s, Joy Division were often accused of having Nazi tendencies. It didn’t help that their name came from the 1955 novella House of Dolls by Jewish writer and Holocaust survivor Yehiel De-Nu (writing under his pen name Ka-tzetnik 135633). The ‘Joy Division’ was the name given to a group of Jewish women in World War II concentration camps whose only purpose was to provide sexual pleasure to Nazi soldiers. I have to admit I’ve never read any of De-Nu’s books. According to an online article by David Mikies (‘Holocaust Pulp Fiction’), De-Nu’s writings were “often lurid novel-memoirs, works that shock the reader with grotesque scenes of torture, perverse sexuality, and cannibalism“. In the 2006 book Joy Division and the Making of Unknown Pleasures, Jake Kennedy asserted that “Curtis’ fascination with extremes would hint to anyone willing to look beyond the headlines that the choice of name was probably an old fashioned punk exercise,  matter of old habits dying hard”.

One of the bands earliest songs ‘Warsaw’ (which was also their band name prior to becoming Joy Division) is arguably a lyrical biography of Hitler’s deputy Führer Rudolf Hess. The song even begins with the lyric “3 5 0 1 2 5 Go!” (Hess’ prisoner of war serial number after he was captured after flying to the UK in 1941). Another of their early songs ‘No Love Lost’ features a spoken word section with a complete paragraph from The House of Dolls. A 2008 article by music writer Jon Savage in The Guardian newspaper noted that Curtis’ songs “such as ‘Novelty’, ‘Leaders of Men’ and ‘Warsaw’ were barely digested regurgitations of their sources: lumpy screeds of frustration, failure, and anger with militaristic and totalitarian overtones”.

Deborah Curtis (Ian’s wife) also remembered that her husband had a book by John Heartfield that included photomontages of the Nazi Period and that graphically documented the spread of Hitler’s ideals. The cover artwork of the band’s first record, the ‘An Ideal For Living’ EP, also featured a boy member the Hitler Youth drawn by guitarist Barney Sumner banging on a drum. Much of the flirtation with Nazi symbolism was arguably juvenile fascination and playful naivety. It’s also been noted that Joy Division’s early music concentrated on the nihilistic provocations of industrial music’s pioneers Throbbing Gristle (whose music I also examined at length in a previous blog). An interesting 2010 article by Mateo on the A View From The Annex website defended Joy Division’s use of Nazi imagery and lyrics:

“The Labour government´s betrayal of the working class during the 1970s and the rise of Thatcherism at the end of the 1970s heralded a future of mass unemployment, government repression and decaying industry. The perspective taken by Ian Curtis, the band´s sole lyricist, towards this growing authoritarianism and despair is crucial to understand if one is to place the references to fascism found in the band´s album art in the context intended by the artist, that is, a despairing anti-Nazism…Punk at that time was a unique music scene in which battles between anti-racists and neo-nazis were being thrashed out at concerts as the skinheads tried to appropriate the punk aesthetic and hijack the following of alienated, disillusioned working class youth who gravitated towards such a sub-culture in places like Manchester at the beginning of the 1980s…The lyrics of Ian Curtis made it clear that this was a presence suffered and feared as opposed to tolerated or toyed with by the band…Joy Division feared fascism, they did not flirt with it and the artwork and lyrics in ‘An Ideal for Living’ serves as a warning of growing fascistic tendencies in British society…For this, Curtis and his bandmates should be lauded for tackling such a controversial issue and expressing such a well-grounded fear and hostility towards such a veritable enemy of the working class during a swift turn to the right in Britain”.

By all accounts, Curtis was a voracious reader and read books by William Burroughs, Fyodor Dostoyevsky, Franz Kafka, Friedrich Nietzsche, Nikolai Gogol, Jean-Paul Sartre, Hermann Hesse and J.G. Ballard, many of which made their way into various Joy Division songs (an obvious example being their song ‘Interzone’ taken directly from a collection of short stories by William Burroughs). As Jon Savage noted:

“Curtis’s great lyrical achievement was to capture the underlying reality of a society in turmoil, and to make it both universal and personal. Distilled emotion is the essence of pop music and, just as Joy Division are perfectly poised between white light and dark despair, so Curtis’s lyrics oscillate between hopelessness and the possibility, if not need, for human connection. At bottom is the fear of losing the ability to feel”.

J.G. Ballard was a particular inspiration to Curtis (particularly the books High Rise and Crash, the latter of which was about the suffering of car accident victims and sexual arousal, and which I wrote about in a previous blog on symphorophilia). One of Joy Division’s best known songs (the opening ‘Atrocity Exhibition’ from their second LP Closer) took its’ name from Ballard’s collection of ‘condensed novels’ (and given its focus on mental asylums is of great psychological interest). So distinct is Ballard’s work that it gave rise to a new adjective (‘Ballardian’) and defined by the Collins English Dictionary as “resembling or suggestive of the conditions described in J.G. Ballard’s novels and stories, especially dystopian modernity, man-made landscapes and the psychological effects of technological, social or environmental developments”. Given this definition, many of Joy Division’s songs are clearly Ballardian as they examine the emotional and psychological effects of everything around them (including personal relationships on songs such as their most well known and most covered song, and only British hit ‘Love Will tear Us Apart’).

The overriding psychology and underlying philosophy of both Ian Curtis and Joy Division are both contradictory and complex but ultimately the band members were a product of the environment they were brought up in and the sum of their musical and literary influences. At the age of 24 years, Curtis’ suicide was undoubtedly tragic and like many other literary and musical ‘artists’, his death has been somewhat romanticized by the mass media. Although he didn’t quite make it into the infamous ‘27 Club’ of ‘rock martyr’ musicians that died when they were 27 years (e.g., Dave Alexander [The Stooges], Chris Bell [Big Star], Kurt Cobain [Nirvana], Richey Edwards [Manic Street Preachers], Pete Ham [Badfinger], Jimi Hendrix, Robert Johnson, Brian Jones [Rolling Sones], Janis Joplin, Jim Morrison [The Doors], Amy Winehouse) he is surely a candidate for being a prime honorary member (along with Jeff Buckley). Retrospectively looking at his lyrics (“In the shadowplay, acting out your own death, knowing no more” from ‘Shadowplay’, you can’t help but wonder (given that many of them were autobiographical) whether Curtis’ death could have been prevented by those closest to him.

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

Further reading

Curtis, D. (1995). Touching From A Distance. London: Faber and Faber.

Curtis, I., Savage, J. & Curtis, D. (2015). So This Is Permanence: Joy Division Lyrics and Notebooks. London: Faber and Faber.

Gleason. P. (2015). This Is the Way: “So This Is Permanence” by Ian Curtis. Located at: http://stereoembersmagazine.com/way-permanence-ian-curtis/

Hook, P. (2013). Unknown Pleasures: Inside Joy Division. London: Simon and Schuster.

Kennedy, J. (2006). Joy Division and the Making of Unknown Pleasures. London: Omnibus.

Mikies, D. (2012). Holocaust pulp fiction. The Tablet, April 19. Located at: http://www.tabletmag.com/jewish-arts-and-culture/books/97160/ka-tzetnik?all=1

Morley, P. (2007). Joy Division: Piece by Piece: Writing About Joy Division 1977-2007. London: Plexus Publishing.

Reynolds, S. (2006). Rip It Up and Start Again: Postpunk, 1978–1984. New York: Penguin.

Savage, J. (2008). Controlled chaos. The Guardian, May 10. Located at: http://www.theguardian.com/books/2008/may/10/popandrock.joydivision

Double trouble: Syndrome of subjective doubles‬

In a previous blog on Delusional Misidentification Syndromes, I briefly mentioned the rare syndrome of ‘subjective doubles’ (SSD). Also known as Christodoulou syndrome (after the Greek psychiatrist Dr. George Nikos Christodoulou who first wrote about the syndrome), SSD refers to individuals who have the belief that there are (one or more) doubles of themselves (i.e., doppelgangers) that carry out actions and behaviours independently and lead a life of their own but that have part or sometimes all of the SDD sufferer’s personality. If the sufferer believes that (some or all of) their personality has been transferred to their doppelganger, they may also experience depersonalization (i.e., a problem of self-awareness in which individuals feel they have little control over social situations and feel they are watching themselves act in a vague and dreamlike state. As with other DMSs, subjective doubles syndrome typically arises as a consequence of a mental disorder, brain injury (typically the right central hemisphere) or a neurological disorder. The Wikipedia entry on SSD cited the case of a man who became depersonalized after an operation and was convinced his brain had been placed into someone else’s head and then claimed he recognized the other person.

In the original paper on SSD in a 1978 issue of the American Journal of Psychiatry, Dr. Christodoulou described the case of a young 18-year-old woman who claimed that a female neighbour had (via an “elaborate transformation” involving “metapmophosis”) acquired all of her physical characteristics (“same face, same build, same clothes, same everything’) and become an identical double. To become her double, the female case study believed her doppelganger had used a mask, wig, and special makeup. Her female neighbour wasn’t the only doppelganger as the woman also claimed at least one other woman had become her doppelganger. In rare instances, there may be comorbidity with the Capgras delusion (another misidentification syndrome) and is then referred to ‘subjective Capgras syndrome’. In fact, there are a number of different sub-types of SDD. As the online Dictionary of Hallucinations notes:

“A subdivision of the syndrome of subjective doubles yields a ‘Capgras type’ (characterized by the delusional conviction that unseen doubles are active in the affected individual’s environment), an ‘autoscopic type’ (in which doubles of the self are perceived, ‘projected’ onto other people or objects, as in pareidolia), and a ‘reverse type’ (in which the affected individual believes to be an impostor or to be about to be replaced by someone else). The syndrome of subjective doubles is associated with various psychiatric disorders (notably the group of so-called schizophrenia spectrum disorders) and neurological disorders (notably disorders of the right parieto-temporal lobe). Conceptually and phenomenologically, the syndrome of subjective doubles constitutes the counterpart of a syndrome called ‘mirrored self-misidentification’, in which the affected individual is unable to identify his or her mirror image as oneself”.

Although most sources cite Dr. Christodoulou’s paper in the American Journal of Psychiatry as the first recorded case of SSD, he actually published a paper a year earlier in a 1977 issue of Acta Psychiatrica Belgica on the treatment of the syndrome of doubles. In this paper, Christodoulou used biological methods to treat 20 psychiatric patients with SDD or the related syndromes (Frégoli, intermetamorphosis, Capgras) aged 17 to 67 years of age. His patients were treated with ECT, antidepressants, neuroleptics, and antiepileptics (in some cases given singly whereas others were in combination). It was reported that:

“Results show that (a) the syndrome of doubles responded to various biological treatment methods; (b) in depression, it responded to tricyclic antidepressants; (c) in schizophrenia or organic psychosis, it usually responded to neurolytics; (d) in schizophrenia, it had more chances of responding to trifluoperazine given alone or in association with other psychopharmacological drugs; and (e) in certain cases, combination of antipsychotic treatment with treatment of coexisting organic dysfunctions appeared to be important”.

In another 1978 paper (in the Journal of Nervous and Mental Disease), Dr. Christodoulou described the course and prognosis of 20 patients with the syndrome of doubles (including Capgras syndrome, Fregoli syndrome, intermetamorphosis syndrome, and SDD – and presumably the same cases reported in the 1977 paper). He reported that the onset of the syndromes occurred either synchronously or at a later stage than the onset of the associated psychosis. In seven of the 20 cases, the syndrome failed to remit. In the remaining 13 cases, remission occurred either synchronously with or later than the remission of the basic psychosis. In all cases where there was comorbid depression, the syndromes cleared shortly after the successful treatment of the depressive illness. It was also noted that relapse of the basic psychotic condition in the setting of which the syndrome had originally developed was usually accompanied by the syndrome reappearing. In one of his most recent papers (from a 2009 issue of Current Psychiatry Reports), Christodoulou and three of his colleagues noted that:

“The delusional misidentification syndromes [including SDD] are rare psychopathologic phenomena that occur primarily in the setting of schizophrenic illness, affective disorder, and organic illness. They are grouped together because they often co-occur and interchange, and their basic theme is the concept of the double. They are distinguished as hypoidentifications (Capgras’ syndrome) and hyperidentifications (the other three syndromes [including SDD]).,,[We] propose that the appearance of these syndromes must alert physicians to investigate the existence of possible organic contributions”.

Compared to other misidentification syndromes, SDD appears to be relatively rare and is often comorbid with other similar conditions. For instance, in a 1986 issue of the Journal of Clinical Psychiatry, Dr. A.B. Joseph described the case of a 30-year old white male who had SDD along with paranoid schizophrenia, Cotard’s syndrome, Capgras delusion, and palinopsia (visual perseveration). Joseph concluded that cerebral dysfunctions in the confluence of the parietal, temporal, and occipital regions of the brain appeared to account for the disorders. Similarly, a 1996 paper in the journal Australasian Psychiatry, Dr. S. Atwal and Dr. M. Khan reported an unusual case of Capgras syndrome coexisting with three related syndromes (Fregoli syndrome, intermetamorphosis syndrome, and SDD).

In a more recent 1991 paper in the journal Psychological Medicine, Dr. H. Forstl and his colleagues examined the psychiatric, neurological and medical aspects of 260 cases suffering misidentification syndromes. Among the sample SDD was relatively rare as 174 cases had a Capgras syndrome misidentifying other persons, 18 a Fregoli syndrome, 11 intermetamorphosis, 17 reduplicative paramnesia and the rest had other forms or combinations of mistaken identification (including SDD). The most common comorbid disorders among those who misidentified themselves or other were schizophrenia (n=127; mostly paranoid schizophrenia), affective disorder (n=29), and organic mental syndromes including dementia (n=46). The authors reported that:

“The misidentification of persons can be a manifestation of any organic or functional psychosis, but the misidentification of place is frequently associated with neurological diseases, predominantly of the right hemisphere. Misidentification syndromes show a great degree of overlap and do not represent distinctive syndromes nor can they be regarded as an expression of a particular disorder. These patients deserve special diagnostic and therapeutic attention because of the possible underlying disorders and their potentially dangerous behaviour”.

Finally, I thought I would leave you with a paper from a 2005 issue of the journal Psychopathology that reported some extreme cases involving delusional misidentification syndromes (DMS) and the danger associated with them. Dr. M. Aziz and his colleagues reported on three cases with histories of paranoid schizophrenia tall of who developed DMSs:

“Two of them acted out on delusional thinking toward their sons. Case 1 managed to kill her son and Case 2 was caught twice trying to choke him. Our case reports suggest that the degree of threat perceived by the patient from the delusionally misidentified object is the most important factor in determining the patient’s response to the delusions. Alcohol and substance intoxication facilitated the patients’ acting out on their delusions, but did not explain the genesis of the delusions. There is a need to continue to study patients with DMS in order to provide opportunity for greater understanding of the psychopathology of DMS”.

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

Further reading

Atwal, S., & Khan, M. H. (1986). Coexistence of Capgras and its related syndromes in a single patient. Australasian Psychiatry, 20, 496-498.

Aziz, M.A., Razik, G.N., & Donn, J.E. (2005). Dangerousness and management of delusional misidentification syndrome. Psychopathology, 38(2), 97-102.

Christodoulou, G.N. (1977). Treatment of the syndrome of doubles. Acta Psychiatrica Belgica, 77(2), 254-259.

Christodoulou, G.N. (1978). Syndrome of subjective doubles. American Journal of Psychiatry, 135, 249-251.

Christodoulou, G.N. (1978). Course and prognosis of the syndrome of doubles. Journal of Nervous and Mental Disease 166, 73-78.

Christodoulou, G.N., Margariti, M., Kontaxakis, V. P., & Christodoulou, N. G. (2009). The delusional misidentification syndromes: strange, fascinating, and instructive. Current Psychiatry Reports, 11(3), 185-189

Dictionary of Hallucinations (2013). Syndrome of subjective doubles. Located at: http://hallucinations.enacademic.com/1828/syndrome_of_subjective_doubles

Enoch, D., Ball, H. (2001). Uncommon Psychiatric Syndromes (Fourth Edition). London: John Wright & Sons.

Forstl, H.A.N.S., Almeida, O. P., Owen, A. M., Burns, A., & Howard, R. (1991). Psychiatric, neurological and medical aspects of misidentification syndromes: A review of 260 cases. Psychological Medicine, 21, 905-910.

Joseph, A.B. (1986). Cotard’s syndrome in a patient with coexistent Capgras’ syndrome, syndrome of subjective doubles, and palinopsia. Journal of Clinical Psychiatry, 47, 605-606.

Bad hair daze: A beginner’s guide to uncombable hair syndrome

I’m sure some of you reading the title of this blog will be thinking that I have made ‘uncombable hair syndrome’ (UHS) up as a way to describe London Mayor Boris Johnson. But it’s not April Fool’s Day and I’m not joking. UHS occurs as the result of a structural deformity in the hair itself and is very rare. UHS goes by a number of different names including ‘pili trianguli et canaliculi’, ‘spun-glass hair’, and ‘cheveux incoiffables’. The condition was first written about scientifically in the 1970s (although earlier reports of the condition had been reported but was not labelled as UHS).

For instance, a case matching contemporary descriptions of UHS by Dr. A.F. Le Double and Dr. F. Houssay was published in 1912. However, it is generally agreed that the syndrome was first formally identified forty years ago (in 1973), by Dr. Dupré, P. Rochiccioli and Dr. J.L. Bonafé in a French paper that described the condition as ‘cheveux incoiffables’. In the following year, two different English speaking doctors (Dr. J.D. Stroud and Dr. A.H. Mehregan) coined the called it ‘spun-glass hair’ in the published proceedings of the ‘First Human Hair Symposium’. By the 1980s, the condition was re-named UHS.

The syndrome is usually noticeable in childhood. Although most parents notice it in their children during their pre-school years, some parents have noticed it a few months after birth but also as late as 12 years of age. A description of UHS can be found in the Wikipedia entry on the condition:

“The hair is normal in quantity and is usually silvery-blond or straw-colored. It is disorderly, it stands out from the scalp, and cannot be combed flat. The underlying structural anomaly is longitudinal grooving of the hair shaft, which appears triangular in cross section. There usually is no family history, though the characteristic hair shaft anomaly can be demonstrated in asymptomatic family members by scanning electron microscopy. To be noticeable, 50% of hairs must be affected by the structural abnormality. Improvement often occurs in later childhood. An autosomal dominant mode of inheritance has been suggested though an autosomal recessive pattern with varying degrees of penetrance has also been noted. The stiffness of the uncombable hair has been reasoned to be due to the triangular form of the hair shaft in cross section. It has been suggested that the condition may result from premature keratinization of the inner root sheath”.

There are also a number of medically recognized conditions where sufferers have the characteristics of UHS. This includes those with ectodermal dysplasia syndrome (these are heritable conditions in which there are abnormalities of two or more ectodermal structures such as the hair, teeth, nails, sweat glands, cranial-facial structure, digits and other parts of the body), retinal dysplasia/pigmentary dystrophy, juvenile cataracts, digit abnormalities, tooth enamel anomalies, oligodontia, and phalangoepiphyseal dysplasia. Both inherited (autosomal dominant and recessive with variable levels of penetrance) and sporadic forms of UHS (i.e., genetic are unclear) have been identified in the medical literature. Most academic papers note that UHS alone is not associated with neurologic, physical, or mental abnormalities. Most of the academic papers in the medical literature are single case studies. Here are a few typical cases that I have found in various medical journals:

• Case 1: “A 4-year-old boy was noted to have unruly, spangled hair, which could not be combed flat. His mother reported that his hair had always had that texture and that it seemed to grow slowly. A hair pull test demonstrated that hairs could not be easily extracted, and light microscopic examination of the hair revealed pathognomonic characteristics of uncombable hair syndrome, including a triangular cross-sectional shape and canal-like longitudinal depressions” (Pediatric Dermatology, 2007).
• Case 2: “The case was a 5-year-old girl. Her whole hair became sparse, and it became a condition, which was immediately formed, when it was held, and the decompression was done. One piece was a condition of curly hair. The scanning electron microscope view showed the pit, which was gentle in the length in paralleling the hair shaft with the hair of about the half. The cross section of a part of hair showed triangle or kidney-shape of which the angle was round. Thus she was diagnosed with uncombable hair syndrome” (Rinsho Derma, 2004).
• Case 3: 
”The patient is a 7 year old Chinese girl who was born vaginally at full term…There was no significant perinatal events and her developmental milestones were normal. She presented with coarse hair since birth, associated with multiple white hairs. There was difficulty with combing. There was no brittleness of hair and no skeletal, nail or skin abnormalities. There was no family history of note…On examination, her hair was coarse, especially over the frontal area of the scalp. There were multiple white hairs interspersed among black hairs. The scalp was normal. The rest of the skin, teeth and skeletal system were normal. Samples of her hair were examined. Under light microscopy the hair shaft appears normal. Under scanning electron microscopy, a longitudinal gutter was seen running the length of the hair shaft. Cross sections of the hair shafts appear triangular or elliptical in shape” (Bulletin For Medical Practitioners, 1995).
• Cases 4-7: “Four children had short, unmanageable, pale blond hair. They had no associated abnormalities and no family histories of abnormal hair. Light microscopy of the hair was normal in three patients, with piii torti present in the fourth. Electron microscopy of hairs from all four children revealed longitudinal grooves in the hair shaft, diagnostic of uncombable-hair syndrome” (Pediatric Dermatology, 1987).
• Cases 8-10: “Three children are reported with uncombable hair syndrome, consisting of slow-growing, straw-colored scalp hair that could not be combed flat. The hairs appeared normal on light microscopy but on scanning electron microscopy were triangular in cross section, with canal-like longitudinal depressions. Oral biotin, 0.3 mg three times a day, produced significant improvement after 4 months in one patient, with increased growth rate and with strength and combability of the hair, although the triangular shape remained. The other two patients were unique in having associated ectodermal dysplasia. Their hair slowly improved in appearance and combability over 5 years without biotin therapy” (Journal of the American Academy of Dermatology, 1985).

According to a review by Dr. P. Calderon and colleagues in a 2009 issue of the Journal of the American Academy of Dermatology, less than 100 cases ever have been reported. They also note that there is no definitive treatment, but that most cases improve with the onset of puberty.

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

Further reading

Calderon, P., Otberg, N., & Shapiro, J. (2009). Uncombable Hair Syndrome. Journal of the American Academy of Dermatology, 61, 512-515.

Hicks, J., Metry, D.W., Barrish, J., & Levy, M. (2001). Uncombable hair (cheveux incoiffables, pili trianguli et canaliculi) syndrome: Brief review and role of scanning electron microscopy in diagnosis. Ultrastructural Pathology, 25, 99-103.

Ishii, N., Mori, O., & Hashimoto, T. (2004). Uncombable Hair Syndrome. Rinsho Derma (Tokyo), 46, 540-541.

Jarrell, A.D., Hall, M.A., & Sperling, L.C. (2007). Uncombable hair syndrome. Pediatric Dermatology, 24, 436-438.

Matis, W.L., Baden, H., Green, R., Boiko, S., Lucky, A. W., Homstein, L., Ashraf, M. & Hood, A.F. (1987). Uncombable‐hair Syndrome. Pediatric Dermatology, 4, 215-219.

Por, A. (1995). Uncombable Hair Syndrome. Bulletin For Medical Practitioners. Located at: http://www.nsc.gov.sg/showpage.asp?id=373

Rochiccioli, D.A. Bonafé J.L. (1973). Cheveux incoiffables: anomalie con genitale des cheveux. Bull Soe Fr Dermatol Syph, 80, 111-112.

Rest, E.B., & Fretzin, D.F. (1990). Quantitative assessment of scanning electron microscope defects in uncombable‐hair syndrome. Pediatric Dermatology, 7(2), 93-96.

Shelley, W.B., & Shelley, E.D. (1985). Uncombable hair syndrome: observations on response to biotin and occurrence in siblings with ectodermal dysplasia. Journal of the American Academy of Dermatology, 13(1), 97-102.

Stroud, J.D. & Mehregan, A.H. (1974). Spun glass hair: A clinicopathologic study of an unusual hair defect. In: Brown, A.C. (Ed.) The first human hair Symposium. New York: Medcom Press.

Stats entertainment (Part 2): A 2013 review of my personal blog

My last blog of 2013 was not written by me but was prepared by the WordPress.com stats helper. I thought a few of you might be interested in the kind of person that reads my blogs. I also wanted to wish all my readers a happy new year and thank you for taking the time to read my posts.

Here’s an excerpt:

The Louvre Museum has 8.5 million visitors per year. This blog was viewed about 860,000 times in 2013. If it were an exhibit at the Louvre Museum, it would take about 37 days for that many people to see it.

Click here to see the complete report.

Muddy daughters: A beginner’s guide to geophagia

In previous blogs I have looked at pica and some of the pica sub-variants including pagophagia (the eating of ice) and coprophagia (the eating of faeces). Pica is defined as the persistent eating of non-nutritive substances for a period of at least one month, without an association with an aversion to food. Today’s blog takes a look at geophagia (the eating of earth, soil and/or clay). In a literature review published in the Journal of the Royal Society of Medicine by Dr Alexander Woywodt and Dr. Akos Kiss that geophagia has been regarded as a psychiatric disease, a culturally sanctioned practice and/or a sequel to poverty and famine. Geophagia is also a culturally sanctioned practice in some parts of the world. Woywodt and Kiss also stated that:

“[Geophagia] is not uncommon in southern parts of the United States5 as well as urban Africa. Fine red clay is often preferred. In particular, geophagia is observed during pregnancy or as a feature of iron-deficiency anaemia. Where poverty and famine are implicated, earth may serve as an appetite suppressant and filler; similarly, geophagia has been observed in anorexia nervosa. However, geophagia is often observed in the absence of hunger, and environmental and cultural contexts of the habit have been emphasized. Finally, geophagia is encountered in people with learning disability, particularly in the context of long-term institutionalization”.

The relationship between anaemia and pica (including geophagia) has been well documented. However, Woywodt and Kiss assert that it is still unclear whether anaemia prompts geophagia to compensate for iron deficiency or whether geophagia is the cause of anaemia. Prevalence rates of pica have range anywhere between 0.02% and 74% depending on the study and population studied although there are few reliable prevalence estimates of geophagia. One study of pregnant Tanzanian women found a prevalence rate of 26.5% (but this is – of course – a totally unrepresentative sample).

A recent review on pica led by Dr Sera Young (University of California, USA) noted that geophagia is the most common type of pica described in the psychological and medical literature although it did also report that geophagics frequently eat other non-food stuffs (particularly if the desired soil is unavailable or socially unacceptable). For many people, pica is not dangerous but for geophagia there may be complications including parasitic infections (from eating soil). Although eating soil and clay may be regarded as unappetizing (and perhaps bizarre) by most people, some authors have argued that eating soil can be nutritionally beneficial (which if that was the case, it wouldn’t technically be a form of pica).

While not being considered a social norm in Western society, eating soil or clay is said to be quite common among primitive or economically depressed peoples a way of augmenting a scanty and/or mineral-deficient diet. Having said that, the geophagia is most often confined to people suffering from chronic mental illness. Clay (as opposed to soil) consumption has been reported in India, Haiti, various parts of Africa (Cameroon, Gabon, Guinea), and even rural areas of the USA. Like soil consumption, clay consumption has also been associated with pregnant women and some women claim they eat it to eliminate nausea. The Wikipedia entry on geophagia noted:

“In Haiti, the poorest economy in the Western Hemisphere, geophagy is widespread. The clay mud is worked into what looks like pancakes or cookies, called ‘bon bons de terres’…The cookies have little or no nutritional value and are associated with various health problems”.

A study led by Dr. L.T. Glickman and colleagues, and published in a 1999 issue of the International Journal of Epidemiology, provided some data on geophagia by carrying out a study examining intestinal parasitism among children from three rural villages in Guinea (Africa). More specifically they examined the faecal stools of 266 randomly selected children (aged 1-18 years). The researchers found that 53% of children were infected by at least one type of soil-transmitted parasite. They also surveyed parents and reported that geophagia was reported by parents to occur in 57% of children aged 1-5 years, 53% of children aged 6-10 years, and 43%, of children aged 11-18 years. It was concluded that geophagia is an important risk factor for orally acquired parasitic infections in African children.

A small study carried out by Turkish researchers and published in a 1978 issue of Acta Haematologica carried out oral iron and zinc tolerance tests on 12 patients from Turkey and Iran aged between 8 and 21 years with iron deficiency anemia and geophagia. The research team reported decreased iron and zinc absorption in patients compared to control patients. They concluded that iron and zinc malabsorption may be an additional feature of the syndrome characterized by geophagia among those from Turkey and Iran. Finally, in their literature review on geophagia, Dr Woywodt and Dr Kiss concluded that:

“The causation is certainly multifactorial; and clearly the practice of earth-eating has existed since the first medical texts were written. The descriptions do not allow simple categorization as a psychiatric disease. Finally, geophagia is not confined to a particular cultural environment and is observed in the absence of hunger”

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

Further reading

Arcasoy, A., Cavdar, A.O. & Babacan, E. (1978). Decreased iron and zinc absorption in Turkish children with iron deficiency and geophagia. Acta Haematologica, 60, 76-84.

Ashworth, M., Hirdes, J.P. & Martin, L. (2008). The social and recreational characteristics of adults with intellectual disability and pica living in institutions. Research in Developmental Disabilities, 30, 512-520.

Danford, D.E. & Huber, A.M. (1982). Pica among mentally retarded adults. American Journal of Mental Deficiency, 87, 141-146.

Glickman, L.T., Camara, A.O., Glickman, N.W. & McCabe, G.P. (1999). Nematode intestinal parasites of children in rural Guinea, Africa: Prevalence and relationship to geophagia. International Journal of Epidemiology, 28, 169-174.

Kettaneh, A., Eclache, V., Fain, O., Sontag, C., Uzan, M. Carbillon, Stirnemann, J. & Thomas, M. (2005). Pica and food craving in patients with iron-deficiency anemia: A case-control study in France. American Journal of Medicine, 118, 185-188

Lacey, E. (1990). Broadening the perspective of pica: Literature review. Public Health Reports, 105, 29-35.

López, L.B., Ortega Soler, C.R. & de Portela, M.L. (2004). Pica during pregnancy: A frequently underestimated problem. Archivos latinoamericanos de nutricion, 54, 17-24.

Nyaruhucha, C.N. (2009). Food cravings, aversions and pica among pregnant women in Dar es Salaam, Tanzania. Tanzania Journal of Health Research, 11(1), 29–34.

Rose, E.A., Porcerelli, J.H, & Anne Neale, A.V. (2000). Pica: Common but commonly missed. Journal of the American Board of Family Practice, 13, 353-358.

Stein, D.J., Bouwer, C. & van Heerden, B. (1996). Pica and the obsessive- compulsive spectrum disorders. South African Medical Journal, 86, 1586-1592.

Woywodt, A. & Kiss, A. (2002). Geophagia: the history of earth-eating. Journal of the Royal Society of Medicine, 95:143-146.

Young, S.L., Wilson, M.J., Miller, D., & Hillier, S. (2008). Toward a comprehensive approach to the collection and analysis of pica substances, with emphasis on geophagic materials. PLoS One, 3(9), e3147.

Wikipedia (2012). Geophagy. Located at: http://en.wikipedia.org/wiki/Geophagy

Swearing blind: A brief look at Anton-Babinski Syndrome‬

The last time I examined some aspect of blindness in my blog was when I examined amaurophilia, a sexual paraphilia where the individual derives sexual pleasure and arousal “by a partner who is blind or unable to see due to artificial means such as being blindfolded or having sex in total darkness”. In today’s blog I briefly examine Anton-Babinski Syndrome (ABS), a rare symptom of occipital lobe brain damage in which sufferers who are “cortically blind”, adamantly claim they are capable of seeing and/or experiencing strange hallucinatory episodes. Consequently, confabulation is common among ABS sufferers. (Confabulation is viewed as a memory disturbance characterized by verbal statements or actions that do not accurately reflect the facts and evidence. Some have described it as “honest lying” because the person usually has no intention of to deceiving the people they are talking to and are usually unaware that the things they are saying are false). For instance, Raj Patel in a 2009 book The Value of Nothing reported one female ABS patient saw a new village outside her window that she couldn’t recall being built. On another occasion she saw a girl in her house that she claimed needed food.

ABS was named after Gabriel Anton (an Austrian psychiatrist and neurologist) and Joseph Babinski (a French neurologist of Polish heritage), and is a form of anosognosia (i.e., an unawareness of severe bodily impairment or disability) that typically arises following a stroke or head injury. According to a 2009 case report by Dr. M. Maddula and colleagues in the Journal of Medical Case Reports, ischemic cerebrovascular disease is the most common etiology of cortical blindness in ABS. A literature review in a 2012 issue of the Journal of Behavioral and Brain Science notes that other diseases described as causes of cortical blindness leading to ABS are “MELAS [Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes], preeclampsia, obstetric hemorrhage, trauma, adrenloeucodistrophy, hypertensive encephalopathy and angiographic procedures”.

In the late 1890s, Anton described three patients (one denying blindness, one denying deafness, and one denying left-sided paralysis) although it was Babinski that coined the term ‘anosognosia’ in 1914 (derived from the Greek for ‘lacking knowledge about disease’). Similar cases were then published sporadically over the next 50 years including Pötzl (1924), Nielsen (1946), Hécaen and de Ajuriaguerra (1954), Levin, Povorinsky, and Tonkonogy (1961), and Hécaen (1972). Some view this condition as the conceptual opposite of blindsight. The Wikipedia entry on ABS notes that:

“The sudden development of bilateral occipital dysfunction is likely to produce transient physical and psychical effects in which mental confusion may be prominent. It may be some days before the relatives, or the nursing staff, stumble onto the fact that the patient has actually become sightless. This is not only because the patient ordinarily does not volunteer the information that he has become blind, but he furthermore misleads his entourage by behaving and talking as though he were sighted. Attention is aroused however when the patient is found to collide with pieces of furniture, to fall over objects, and to experience difficulty in finding his way around. He may try to walk through a wall or through a closed door on his way from one room to another. Suspicion is still further alerted when he begins to describe people and objects around him which, as a matter of fact, are not there at all. Thus we have the twin symptoms of anosognosia (or lack of awareness of defect) and confabulation, the latter affecting both speech and behaviour”.

According to the US National Institute of Health, ABS affects less than 200,000 people in the whole of the US population. ABS sufferers typically explain their bruises and injuries as a result of clumsiness or absentmindedness (rather than poor vision or blindness). Consequently, confabulation is often a way in which ABS can be diagnosed.

There are a number of theories as to how ABS occurs as no-one knows for sure why patients deny they are blind. One school of thought is that visual cortex damage may result in an inability to communicate with the brain’s speech-language areas (i.e., visual information is received but not interpreted correctly and a verbal response is confabulated). In fact, Dr. G. Goldenberg and colleagues in a 1995 issue of the journal Neuropsychologia claim that damage to the visual association cortex is thought to be one of the main causes explaining the loss of awareness of the visual deficit. Others postulate that ABS patients are simply having hallucinatory sensations (unrelated to their actual surrounding reality).

In a 1978 French neurology journal, Dr. J.M. Verret and Dr. J. Lapresle described a female ABS patient presenting with an accompanying delusional conviction in which she “recognised her left upper limb with the aid of her right hand, but immediately denied its existence when she viewed it directly. In contrast, when placed in front of a mirror, she recognised this upper limb perfectly, recognition disappearing again when direct vision was associated with vision in the mirror”. The authors suggested there was a possibility of a resurgence in adult life of the duality of the visual body image, direct or reflected, such as is normally experienced in childhood and, more prolonged, in identical twins.

The most recently published paper on ABS was a 2012 paper by Dr. Juan Carvajal and his colleagues in the Journal of Behavioral and Brain Science. They reported the cases of two ABS patients. The first was a 96-year-old male with visual anosognosia secondary to cerebral artery thrombosis, and the second was a 56-year-old female with ABS secondary to central nervous system angiitis in relation with multiple sclerosis and Hashimoto’s thyroiditis. They reported that although ischemic vascular cerebral disease is a frequent etiology with ABS (as noted above), they believed that this was the first report of ABS in relation to angiitis with a clear autoimmune pathogenesis. ABS can be treated with cognitive therapy although in some instances it may simply fade away over time (but most in the medical profession recommend treatment rather than just letting it fade).

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

Further reading

Anton, G. (1898). Über Herderkrankungen des Gehirns, welche vom Patienten selbst nicht wahrgenommen werden. Wiener klinische Wochenschrift, 11, 227–229.

Anton, G. (1899). Über die selbst wharnehmoung der herederkrankungen des gehirns durch den kranken der rindenblindheit und rindentaubheit. Archiv für Psychiatrie und Nervenkrankheiten, 32, 86–127.

Babinski, J. (1914). Contribution à l’étude des troubles mentaux dans l’hemiplegie cerebrale (anosognosie). Revue Neurologique, 27, 845–847.

Carvajal, J.J.R., Cárdenas, A.A.A, Pazmiño, G.Z., & Herrera, P.A. (2012). Visual anosognosia (Anton-Babinski Syndrome): Report of two cases associated with ischemic cerebrovascular disease. Journal of Behavioral and Brain Science, 2, 394-398.

Critchley, M. (1953). The parietal lobes. London: E. Arnold and Co.

Goldenberg, G., Mullbacher, W. & Nowak, A. (1995). Imagery without perception: A case study of anosognosia for cortical blindness. Neuropsychologia, 33, 1373-1382.

Hécaen, H. (1972). Introduction a la neuropsychologie. Paris: Larousse.

Levin, G. Z., Povorinsky, Y. A., & Tonkonogy, J. M. (1961). Analysis of the case with agnosia of faces developed after air embolism of cerebral vessels [in Russian]. In G. B. Abramovich & G. Z. Levin (Eds.), Problems of Localization and Focal Diagnostic in Neurology and Psychiatry (pp. 111-123), Leningrad: Bechterev Institute Press.

Maddula, M., Lutton, S. & Keegan, B. (2009). Anton’s Syn-drome due to cerebrovascular disease: A case report. Journal of Medical Case Reports, 3, 9028.

McGlynn, S.M. & Schacter, D.L. (1989). Unawareness of deficits in neuropsychological syndromes. Journal of Clinical and Experimental Neuropsychology, 11, 143-205.

Misra, M., Rath, S. & Mohanty, A.B. (1989). Anton Syndrome and cortical blindness due to bilateral occipital infarction. Indian Journal of Ophthalmology, 37(4), 196.

Nielsen, J. M. (1946). Agnosia, apraxia, aphasia: Their value in cerebral localization. New York: Hoeber.

Patel, R. (2009). The Value of Nothing: How to Reshape Market. London: Portobello Books.

Poetzl, O. (1924). Uber die Storungen der Selbst wahrnehmung bey linksetiger hemiplegie. Zeitschrift für Neurologie und Psychiatrie, 93, 117–168.

Verret, J.M. & Lapresle, J. (1978). [Anton-Babinski syndrome with recognition of the left upper limb on visualization in a mirror] (Article in French). Rev Neurol (Paris), 134, 709-713.

Wikipedia (2012). Anton-Babinski Syndrome. Located at: http://en.wikipedia.org/wiki/Anton–Babinski_syndrome

Becoming less of a drag: The Stoptober campaign (revisited)

(The following blog is based on an article I published last year in the Nottingham Post on why I was actively supporting the Stoptober smoking campaign to get people to stop smoking for 28 days during October. I also published a blog last year outlining my 10 top tips for giving up smoking. Since that blog, my ten tips have been slightly changed and adapted in co-operation with the Department of Health running the Stoptober campaign. I make no apologies for repetition between today’s blog and that published last year, as my only aim is to help people give up smoking).

Although most of my academic research is on behavioural addiction, I have published quite a few papers on more traditional addictions such as alcohol and nicotine addiction (see ‘Further reading’ below). Last year I had to watch my mother fight a losing battle with smoking-related lung cancer and chronic obstructive pulmonary disease. She died in September 2012 aged 66 years, and had chain-smoked most of her adult life. This followed the death of my father who also died of smoking-related heart disease, aged just 54.

This October, the Department of Health (DoH) are re-launching the ‘Stoptober’ campaign for the second time, urging as many nicotine smokers as possible to give up smoking for 28 days from October 1. The DoH website claims that “people who stop smoking for 28 days are five times more likely to stay smoke free” compared to those that don’t give up for such a long period. Like last year, those that decide to try and stop for the month will be given a lot of encouragement during the campaign including access to the Smokefree Facebook page and the downloadable Stoptober app. People will also be sent daily emails providing additional encouragement.

In the UK smoking accounts for approximately one in four cancer deaths, and as I said, it’s something I’ve witnessed first-hand. I’m sure most people reading this are aware of the addictive nature of nicotine. As soon as nicotine is ingested via cigarettes, it can pass from lungs to brain within ten seconds and stimulates the release of the neurotransmitter dopamine. The release of dopamine into the body provides reinforcing mood modifying effects. Despite nicotine being a stimulant, many people use cigarettes for both tranquillising and euphoric effects. Most authorities accept that nicotine is one of the most addictive drugs on the planet and that smokers can become hooked quickly. One of the reasons my own parents were never able to give up was because of the prolonged withdrawal effects they experienced whenever they went more than a few hours without smoking. This would lead to intense cravings for a cigarette. Watching both my parents’ die of smoking-related diseases is enough incentive for me to never smoke a cigarette. Hopefully, others can find the incentives they need to help them give up permanently. Here are my top ten tips to help you (or someone you know and love) stop smoking:

• (1) Develop the motivation to stop smoking: Many smokers say they would like to stop but don’t really want to. When you take stock, make sure you are clear as to why you want to give up. It may be to save money, to improve your health, to prevent yourself getting a smoking-related disease, or to protect your family from passive smoking. (It could of course be all of the above). Really wanting to give up is the best predictor of successful smoking cessation.
• (2) Get all the emotional support you can: Another good predictor of whether someone will overcome their addiction to nicotine is having a good support network. You need people around you that will support your efforts to quit. Tell as many people that you know that you are trying to quit. It could be the difference between stopping and starting again.
• (3) Avoid ‘cold turkey’: Although some people can stop through willpower alone, most people will need to reduce their nicotine intake slowly. The best way of doing this is to replace cigarettes with a safe form of nicotine such as those available from the pharmacy, or on prescription from the doctor.
• (4) Get support from a professional: Even if you are using a safe form of nicotine from your pharmacist or doctor, cutting out cigarettes completely can be hard. Getting support from a trained NHS stop smoking adviser can double your chances of stopping smoking. To find your nearest free NHS stop smoking service (in the UK call 0800-1690169) or visit the smokefree website and click on the ‘ways to quit’ tab.
• (5) Use non-nicotine cigarette shaped substitutes: Smoking is also a habitual behaviour where the feel of it in your hands may be as important as the nicotine it contains. The use of plastic cigarettes or e-cigarettes will help with the habitual behaviour associated with smoking but contain none of the addictive nicotine.
• (6) Use relaxation techniques: When cravings strike, use relaxation exercises to help overcome the negative feelings. At the very least take deep breaths. There are dozens of relaxation exercises online. Practice makes perfect.
• (7) Treat yourself: One of the immediate benefits of stopping smoking will be the amount of money you save. At the start of the cessation process, treat yourself to rewards with the money you save.
• (8) Focus on the positive: Giving up smoking is one of the hardest things that anyone can do. Write down lists of all the positive things that will be gained by stopping smoking. Constantly remind yourself of what the long-term advantages will be that will outweigh the short-term benefits of smoking a cigarette. In short, focus on the gains of stopping rather than what you will miss about cigarettes.
• (9) Know the triggers for your smoking: Knowing the situations in which you tend to smoke can help in overcoming the urges. Lighting up a cigarette can sometimes be the result of a classically-conditioned response (e.g. having a cigarette after every meal). These often occur unconsciously so you need to break the automatic response and de-condition the smoking. You need to replace the unhealthy activity with a more positive one and re-condition your behaviour.
• (10) Fill the void: One of the most difficult things when cigarette craving and withdrawal symptoms strike is not having an activity to fill the void. Some things (like engaging in physical activity) may help you in forgetting about the urge to smoke. Plan out alternative activities and distraction tasks to help fill the hole when the urge to smoke strikes (e.g. chew gum, eat something healthy like a carrot stick, call a friend, occupy your hands, do a word puzzle, etc.). However, avoid filling the void with other potentially addictive substances (e.g. alcohol) or activities (e.g. gambling).

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

Further reading

Griffiths, M.D. (1994). An exploratory study of gambling cross addictions. Journal of Gambling Studies, 10, 371-384.

Griffiths, M.D.  (2005). A ‘components’ model of addiction within a biopsychosocial framework. Journal of Substance Use, 10, 191-197.

Griffiths, M.D. (2012). First person: Highly-addictive drug killed both of my parents. Nottingham Post, October 1, p.13.

Griffiths, M.D., Parke, J. & Wood, R.T.A. (2002). Excessive gambling and substance abuse: Is there a relationship? Journal of Substance Use, 7, 187-190.

Griffiths, M.D., Wardle, J., Orford, J., Sproston, K. & Erens, B. (2010). Gambling, alcohol consumption, cigarette smoking and health: findings from the 2007 British Gambling Prevalence Survey. Addiction Research and Theory, 18, 208-223.

Griffiths, M.D., Wardle, J., Orford, J., Sproston, K. & Erens, B. (2011). Internet gambling, health. Smoking and alcohol use: Findings from the 2007 British Gambling Prevalence Survey. International Journal of Mental Health and Addiction, 9, 1-11.

Resnick, S. & Griffiths, M.D. (2010). Service quality in alcohol treatment: A qualitative study. International Journal of Mental Health and Addiction, 8, 453-470.

Resnick, S. & Griffiths, M.D. (2011). Service quality in alcohol treatment: A research note. International Journal of Health Care Quality Assurance, 24, 149-163.

Resnick, S. & Griffiths, M.D. (2012). Alcohol treatment: A qualitative comparison of public and private treatment centres. International Journal of Mental Health and Addiction, 10, 185-196.

Sussman, S., Lisha, N. & Griffiths, M.D. (2011). Prevalence of the addictions: A problem of the majority or the minority? Evaluation and the Health Professions, 34, 3-56.

Umeh, K. & Griffiths, M.D. (2001). Adolescent smoking: Behavioural risk factors and health beliefs. Education and Health, 19, 69-71.

Next of skin: A brief look at Ehlers-Danlos Syndrome

Although I have seen various photos of people with super stretchy skin and incredibly flexible bodies, I never realized that most people who appear to have these seemingly ‘super-human’ abilities actually have a genetically inherited connective tissue disorder called Ehlers-Danlos Syndrome (EDS; sometimes referred to as ‘cutis hyperelastica’). The name of the syndrome comes from the people who were among the first to identify it in the early 1900s (the Danish dermatologist Edvard Ehlers in 1901, and the French physician Henri-Alexandre Danlos in 1908). However, the first description of the syndrome was actually by a Russian dermatologist called Tschernogobow in 1892.

The condition in some EDS sufferers is caused by the individual’s inability to synthesize collagen (a body protein found in human connective tissue) and for some people can be life threatening. Collagen plays a critical role in the strength of human muscles, skin, blood vessels, ligaments, etc. Abnormal collagen production leads to elasticity of human body structure. Unfortunately there is no cure for EDS and the only treatment available is of a supportive nature (e.g., occupational therapy, corrective surgery for identified weaknesses, etc.). According to the Wikipedia entry on EDS:

“Some of the specific mutations involved have been identified and they can be precisely identified by genetic testing; this is valuable due to a great deal of variation in individual cases. However, negative genetic test results do not rule out the diagnosis, since not all of the mutations have been discovered; therefore the clinical presentation is very important. Although the [six] classifications are well defined, it is rare for a case to fit neatly in a single category, and crossover symptoms lead to under-diagnosis or misdiagnosis. Therefore, patients should not rely on the ‘fact’ of having a certain type of EDS if crossover symptoms are evident because of possibly life-threatening symptoms. For example, it is possible for an individual with Classical EDS to exhibit symptoms of hypermobility or vascular EDS”.

Up until 1997, there were 10 types of EDS but a multi-authored paper published in a 1998 issue of the American Journal of Medical Genetics proposed a new descriptive formulation and reduced the number of sub-types to six: (i) classical, (ii) hypermobility, (iii) vascular, (iv) kyphoscoliotic, (v) arthrochalasia, and (vi) dermatosparaxis.

• Classical: This sub-type affects 1 in 10,000-15,000 people. The defining characteristic is joint hypermobility coupled with less severe skin manifestations. The most common symptoms are joint instability and chronic musculoskeletal pain.
• Hypermobility: This sub-type affects approximately 1 in 20,000-50,000 people. The most common symptom is mild to severe skin involvement.
• Vascular: This sub-type affects approximately 1 in 100,000-250,000 people. This sub-type often (i) slim build, (ii) has pale thin translucent skin, (iii) bruises incredibly easily, and (iii) has a characteristic facial appearance (large eyes, small chin, sunken cheeks, thin nose and lips, lobeless ears. Because blood vessels and organs are fragile and liable to rupture, this sub-type is considered one of the more serious.
• Kyphoscoliotic: This sub-type is very rare (no more than 60 cases reported worldwide). The most common symptom is progressive curvature of the spine, fragile eyes, and severe muscle weakness.
• Arthrochalasia: This sub-type is rare (no more than 30 cases reported worldwide). The most common symptoms are very loose joints and hip dislocations.
• Dermatosparaxis: This sub-type is the rarest (about 10 cases reported worldwide). The most common symptom is extremely fragile and sagging skin.

According to a paper by Dr. S. Ainsworth and Dr. P. Aulicino in a 1993 issue of the journal Clinical Orthopaedics, EDS affects both males and females of all socioeconomic status and ethnic backgrounds. X-rays of EDS sufferers tend to be normal. According to Dr. Peter Beighton who has written many papers on EDS over a thirty year period says that parents of EDS children should inform others (e.g., teachers, neighbours), so frequent and/or sizeable bruising is not mistaken for child abuse. In a 2007 issue of the British Medical Journal, Dr. Frances Gawthorp and colleagues featured three different case studies of people with EDS actually written by the person with the disorder. I found the one written by Amanda Sperrit who has the most common form of EDS of particular interest. She said:

“I am 36 years old and have hypermobile Ehlers-Danlos syndrome. As a child I was ‘double jointed’ – able to do party tricks that made people say ‘yuk’. When I was 15 [year old] my hips began to slip spontaneously. My general practitioner said that it was my imagination. Later, at college, I could only shuffle, hips slipping out with every step. I was referred to hospital. The consultants were mystified. They insisted I would grow out of it. Later, I was admitted to hospital and told that I had Ehlers-Danlos syndrome…I was devastated. Since then the symptoms have progressed. I have persistent pains in my shoulders, hips, neck, back, and hands. I have difficulty in standing and walking. I use a stick and occasionally a wheelchair. My grip is poor. I can’t write or even type easily. I have stretchy, fragile skin that doesn’t heal well. I have trouble speaking and swallowing.

I get palpitations and night sweats…Often I am just shown to medical students as an interesting case. Physiotherapists are much better. They allow me to play an active part in my treatment and have taught me to use my muscles to protect my joints. Cannabis was also helpful. I learnt to grow my own and it reduced my dependence on pills. I slept better and I could do more physiotherapy and even a part-time job. I told my doctors; none objected but my secret got out. The police came and took my plants away. A judge regarded my action as wrong doing and left me with a criminal record. How can that be right?

I have tried alternative medicine but always make sure that the practitioners know about the syndrome. The Alexander technique is good for relaxation and posture awareness. A short course of acupuncture also had a positive effect. My present philosophy is, ‘get all the information and advice you can and use what works for you’…I haven’t slept all night for 17 years. I often wake up after a couple of hours, drenched in sweat. I arrange my life so that I can sleep when I need to and have devised ways of getting back into activities such as art, music, and cookery. I help my sister with charity work for severely disabled children. Once more I feel that I am making a contribution to society. The most helpful professionals treat me as a partner in the management of my condition. They listen to the information I discover and support me in my struggle to get the equipment, services, and financial help that I need to make life that bit easier”

This first hand account made me realise that EDS sufferers are far from ‘super-human’ as they are often described. The condition can obviously be devastating and debilitating. As Dr. Gawthorp and colleagues concluded:

“The ramifications of Ehlers-Danlos syndrome are wide and various, and the effects may be disabling and even life threatening. Emerging data suggest that while the vascular form is fortunately rare, the hypermobility form is common but continues to go undetected, or worse still, ignored”.

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

Further reading

Ainsworth, S.R. & Aulicino, P.L. (1993). A survey of patients with Ehlers-Danlos syndrome. Clinical Orthopaedics, 286, 250-256.

Beighton P. (1970). The Ehlers-Danlos Syndrome. London: William Heine-mann Medical Books.

Beighton, P. & Bird, H. A. (1993). The Ehlers-Danlos Syndrome. Ehlers-Danlos Support Group.

Beighton, P., De Paepe, A., Steinmann, B., Tsipouras, P., Wenstrup, R.J. (1998). Ehlers–Danlos syndromes: revised nosology, Villefranche, 1997. Ehlers–Danlos National Foundation (USA) and Ehlers–Danlos Support Group (UK). American Journal of Medical Genetics, 77, 31–37.

Dereń-Wagemann, I., Kuliszkiewicz-Janus, M., & Schiller, J. (2010). Ehlers-Danlos syndrome. Advances in Clinical Experimental Medicine, 1, 537-542.

Gawthrop, F., Mould, R., Sperritt, A., & Neale, F. (2007). Ehlers-Danlos syndrome. British Medical Journal, 335, 448-450.