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Small talk: Alice in Wonderland Syndrome (Revisited)

Last week, I was contacted by the journalist Abigail Moss about a previous blog that I wrote on Alice in Wonderland Syndrome (AIWS). AIWS is a non-contagious disorientation disorder and refers to when a person’s sense of body image, vision, hearing, touch, space, and/or time are distorted. AIWS sufferers typically experience micropsia (a neurological condition that affects human visual perception in which objects are perceived to be smaller than they actually are and make people feel bigger than they are) or macropsia (a neurological condition that affects human visual perception in which objects are perceived to be larger than they actually are and makes people feel smaller than they actually are).

Moss suffers from AIWS herself and has described her experiences both on camera (for instance in a short YouTube news piece and in numerous news articles such as one in the Daily Mail). Moss also sent me a first-person account of AIWS that she published in the online magazine Planet Ivy. Here are some of the things she recounted:

Extract 1: “When I was about five years old, I started to experience strange visual and sensory hallucinations. My hands and legs would seem too big for my body and the room around me would start to shrink inwards. All movements and sounds would seem extremely fast and hugely exaggerated, giving everything an odd feeling of urgency. This would last about 20 minutes and slowly fade away, and happened about four or five times a week…Luckily, my dad knew exactly what I was describing…He’d experienced the same thing as a kid. I haven’t outgrown it…but it doesn’t happen as often – maybe only five times a year”.

The first doctor suggested she might have a form of epilepsy but after that proved negative she was never diagnosed with anything official. Since then, other medics have suggested that she may have a sleep disorder or some kind of schizophrenia. It wasn’t until she was in her early twenties that she came across something that fitted her symptoms:

Extract 2: “Last year a tiny 50-word inset in a newspaper supplement caught my eye. ‘Alice in Wonderland Syndrome’ the heading read, and underneath it, a perfect description of my experience…I joined a Yahoo forum for ‘AIWS sufferers’…Countless fellow sufferers got in contact, all describing the same thing. Their descriptions were remarkably unvaried and it was immediately obvious this was the same thing I experience: ‘My body felt minuscule’, ‘Sounds were amplified’, ‘Everything was bigger and smaller at the same time’.”

The article outlined the many psychiatrists and psychologists Moss had visited about her condition with all the experts she saw claiming that they had never heard of AIWS. While the condition is rare, the condition has been well documented in the medical and clinical literature (see ‘Further reading’ below) so I was quite surprised that the experts she visited couldn’t have at least spent some time reading up on the syndrome. She then went on to say:

Extract 3: “My brain does something extremely weird, and nobody – not even the world’s leading bodies in the study of brains – can tell me what this is or why it happens. For me, this isn’t a problem, I don’t want to get rid of my episodes – they’re an interesting talking point and everyone knows writers are basically quite boring people. The experience, when it does pop up, doesn’t actually bother me or affect my day-to-day life”.

Moss wanted to ask me a few questions about my understanding of AIWS and how much is now known about it. She knew this wasn’t my primary area of expertise, but said that any opinions I might be able to offer would be invaluable to the article she was writing. I told her that my article on AIWS was written in a journalistic capacity rather from any position of expertise but she still wanted me to answer a few questions. Moss asked me three questions and I thought I would use this blog to share my full answers with my readers.

Question 1: “In my research I’ve found it almost impossible to find solid answers about what causes AIWS. Can you offer a view on what makes this condition so difficult to pin down?”

AIWS has been reported in the psychiatric and psychological literature since the early 1950s. However, since the first papers in the topic less than 20 papers have ever been published and all of them are case reports. Finding ‘solid answers’ based on so few cases is therefore inevitable. The literature is also biased because it is (a) based on those sufferers who seek out medical assistance, and (b) based on those doctors or clinicians that have written the cases for publication. If people don’t seek help and/or there cases remain unwritten, there is little chance if finding ‘solid answers’.

Secondly, the symptoms are not always identical which is why it is referred to as syndrome (that is, a group of symptoms that together are characteristic of a specific disorder or disease, or a predictable, characteristic condition or pattern of behavior that tends to occur under certain circumstances). Syndromes typically have many different causes which again means it is difficult to find ‘solid answers’.

Finally, given that the experiences (like your own) are often short-lived, it is very rare to be able to monitor people neurologically. The few published cases are based on chronic sufferers (who may not be representative of the vast majority of AIWS sufferers). Several neurologists have done M.R.I.s on patients with the condition, though once the bout has passed, there’s usually no sign of unusual brain activity. I read that Dr. Sheena Aurora was the first to scan the brain of someone — a 12-year-old girl — in the middle of an episode. According to Dr. Aurora, electrical activity caused abnormal blood flow in the parts of the brain that control vision and process texture, shape and size.

The case studies that I have read have provided lots of possible reasons for AIWS but there is no consensus and they could all be true (as having the same symptoms doesn’t mean there has to be the same cause). Some research appears to indicate that AIWS can be due to abnormal amounts of electrical activity that causes blood to flow abnormally in the brain areas that process texture and visual perception. AIWS has been associated with migraines, severe depression, and (in extreme cases) brain tumours. Case study research has indicated that AIWS manifestations are due to disturbed function of either medial temporal, hippocampal, tempro-occipital or tempro-parieto-occipital regions of the brain. Unfortunately, chronic AIWS is untreatable and time is the only healer. However, sharing experiences with other sufferers is also thought to be therapeutically beneficial

Question 2: “Is academic disagreement just part and parcel of all psychological conditions or does AIWS seems particularly open to discussion?”

I don’t think there is ‘disagreement’ as no two clinicians or psychiatrists have ever published papers examining the same individuals. They have all published papers based on the AIWS sufferers that they themselves saw and that who came in seeking help. All of the explanations could be correct as syndromes have multiple causes. This is not disagreement. It’s simply a case of multiple possible causes.

Question 3: “I have spoken to a large number of people who also say they also experience AIWS – how useful or valid do you think it would be to think of AIWS as more of a mental phenomenon than a syndrome, comparable with something like déjà vu, for example?”

The word ‘large’ is what we psychologists call a ‘fuzzy quantifier’ as ‘large’ to one individual is small to another. If you have spoken to 100 other sufferers worldwide I would say this is very very small. The condition appears to be rare although in one of my other areas of research, we have demonstrated that a small proportion of video gamers experience disorienting visual effects (that we call game transfer phenomena) like AIWS so such phenomena may be multi-faceted and may arise from specific activities (such as excessive and immersive game playing).

AIWS should not be compared with déjà vu as most scientific evidence suggests that déjà vu is an anomaly of memory and totally different from AIWS on a neurological level (but I’m not an expert on déjà vu and am only basing my opinion on what I have read in the psychological literature). However, there may be some conditions (such as schizophrenia and temporal lobe epilepsy) where individuals may experience both AIWS and déjà vu but these are symptoms of a specific medical disorder. Most individuals that have experienced déjà vu (as many as two-thirds of the population in some studies) and AIWS (very rare) do not have any underlying serious medical conditions.

I don’t know if any of my responses to Moss were of help either in relation to her own experiences or in writing her article but I was pleased with the observations I had made.

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

Further reading

Bui, E., Chatagner, A. & Schmitt, L. (2010). Alice in Wonderland Syndrome in major depressive disorder. Journal of Neuropsychiatry and Clinical Neurosciences, 22, 352.e16-352.e16.

Cinbis, M. & Aysun, S. (1992). Alice in Wonderland syndrome as an initial manifestation of Epstein-Barr virus infection (case report). British Journal of Ophthalmology, 76, 316.

Eshel, G.M., Eyov, A., & Lahat, E., et al (1987). Alice in Wonderland syndrome, a manifestation of acute Epstein-Barr virus infection (brief report). Pediatric Infectious Diseases Journal, 6, 68.

Kew, J., Wright, A., & Halligan, P.W. (1998). Somesthetic aura: The experience of “Alice in Wonderland”. The Lancet, 351, 1934.

Kitchener, N. (2004). Alice in Wonderland Syndrome. International Journal of Child Neuropsychiatry, 1, 107-112.

Kuo, Y, Chiu, N.C., Shen, E.Y., Ho, C.S., Wu, M.C. (1998). Cerebral perfusion in children with “Alice in Wonderland” syndrome. Pediatric Neurology, 19, 105-108.

Lahat, E., Eshel, G., & Arlazoroff A (1990). “Alice in Wonderland” syndrome and infectious mononucleosis in children (letter). Journal of Neurology, Neurosurgery and Psychiatry, 53, 1104.

Lambert, M.V., Sierra, M., Phillips, M.L. & David, A.S. The spectrum of organic depersonalization: A review plus four new cases. Journal of Neuropsychiatry and Clinical Neuroscience, 14, 141-154.

Podoll, K., Ebel, H., Robinson, D., & Nicola, U. (2002). Obligatory and facultative symptoms of the Alice in wonderland syndrome. Minerva Medicine, 93, 287-293.

Podoll, K. & Robinson, D. (1999). Lewis Carroll’s migraine experiences. The Lancet, 353, 1366.

Rolak, L.A. (1991). Literary neurologic syndromes. Alice in Wonderland. Archives of Neurology, 48, 649–651.

Todd, J. (1955). The syndrome of Alice in Wonderland. Canadian Medical Association Journal, 73, 701–704.

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

“I see dead people”: Are hallucinations always bad?

I have to admit that I know relatively little about the neuropsychology of hallucinations. The only time I have written about them in scientific journals is in the context of excessive video gaming where there are case studies of people who appear to display auditory and/or visual game-related hallucinations, and may be part of a wider repertoire of sensory consequences of video game playing that we have coined ‘game transfer phenomena’ (and which I outlined in a previous blog).

However, in a completely different context, I recently came across a really interesting 2011 case study by Dr. Amin Gadit who published a short paper in BMJ Case Reports entitled ‘Insightful hallucination: psychopathology or paranormal phenomenon?’ Dr. Gadit noted that hallucinations are usually indicative of a serious psychiatric problem (i.e., typically some kind of psychosis) and typically require treatment. However, Dr. Gadit described the case of a 26-year old successful Pakistani businessman who was suffering hallucinations but experienced a dilemma as to whether to treat him or not because his hallucinations appeared to be providing some therapeutic benefit to his patient.

The man was married to his first cousin (also from Pakistan) and was described as being “extremely close” to his mother. Dr. Gadit reported that his patient’s wife sometimes got extremely upset (which I interpreted as being jealous) about her husband’s attachment to his mother. Following the mother’s diagnosis of a terminal illness with only a few months left to live, the man (understandably given the relationship with his mother) experienced deep emotional turmoil and upset. Dr. Gadit wrote that according to his patient that:

“[His] mother told him before dying that she would remain in contact with him after death. The patient went through a complicated bereavement period when she died. However, 6 months later, he regained his cheerful mood and started taking an interest in business again. His wife noticed that he was talking to himself for at least an hour each day. When asked, he said that his mother visits him every day and he talks to her. This was his firm belief. There was no deterioration in his personality and no other features worthy of note”.

Following these episodes of speaking to his dead mother almost every night at different times in the evening, the man’s wife persuaded him to seek psychiatric help. Dr. Gadit claimed that his patient resented being in treatment and argued that the regular “contact” with his dead mother was a positive experience and made the man happy and helped bring normality to his day-to-day life. Following initial psychiatric assessment, Dr. Gadit noted that:

“There was no significant medical history or family history indicative of any mental disorder. A thorough clinical history revealed nothing except this hallucination. The patient had retained insight as he believed that this would not happen normally but in his case was a special occurrence. He attributed this to his Muslim belief of God’s blessing in sending his mother back to him in this way. His physical examination was unremarkable and all laboratory results were normal. MRI did not reveal any pathology. His mental state examination revealed normothymic mood, delusion, visual hallucination, psychosis (with no supporting evidence), intact cognitive function and reasonable insight into his problem”.

The man’s mother appeared most evenings wearing different dresses (ones that she used to wear when she was alive) but he said his mother would not allow him to touch her when she appeared. The man was adamant that his mother appeared before him in the real world and refused any medical treatment. Organ pathology (often associated with auditory hallucinations) was ruled out as a cause, and there was insufficient evidence for a diagnosis of schizophrenia (often associated with auditory hallucinations). Ultimately, Dr. Gadit did not reach a psychiatric diagnosis and he sought a second opinion (which also failed to produce a diagnosis). The lack of formal diagnosis posed a dilemma in terms managing the presenting condition. The man had monthly appointments for over half a year with Dr. Gadit but the condition remained constant. In discussing the case, Dr. Gadit wrote that:

The patient recognises the hallucination (perception without the presence of an external stimulus) as happening in the real world. It is important to differentiate true hallucination from ‘pseudo-hallucination’ and ‘imagery’. A pseudo-hallucination is an involuntary sensory experience vivid enough to be regarded as a hallucination but recognised by the patient as not the result of external stimuli; it would not be considered by the person to be ‘real’. Imagery is a collection of images used to create a sensory experience and is the element in a literary work used to evoke mental images and stimulate an emotional response. In the current case report, the patient believes that he can see and talk to his mother in the real world and that he is not imagining it”.

In discussing the case in relation to previous literature, Gadit made reference to a 2009 paper by H. Haween in the Dartmouth Undergraduate Journal of Science (DJUS) that reported hallucinations following bereavement typically resolve over time. Such hallucinations are most commonly in reported during the grieving process in males aged 25 to 30 years. Other similar non-psychiatric illnesses include Charles Bonnet’s Syndrome (typical sufferers being the elderly) that comprises clear hallucinations experienced among visually impaired individuals. A study dating back to 1971 by Dr. W.D. Rees and published in the British Medical Journal reported ‘widowhood hallucinations’ in 14% of Welsh widows and widowers (n=293). A more recent study in a 2002 issue of the British Journal of Psychiatry, a team led by Dr. L.C. Johns reported a 4% prevalence of hallucinations in white and ethnic minority populations and suggested that hallucinations are not always associated with psychotic disorders.

Gadit claimed that his male case study was “unique” as the persistent hallucinations resulted in no noticeable psychopathology, and appeared beneficial to his patient. He also speculated that the visions might be a paranormal experience or “a case of hallucinosis with a secondary delusional explanation”. Gadit claimed that paranormal phenomena are fairly common in both the developed and the developing world (and typically associated with rituals and myths).

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

Further reading

Gadit, A.A.M. (2011). Insightful hallucination: psychopathology or paranormal phenomenon? BMJ Case Reports 2011; doi:10.1136/bcr.10.2010.3456

Heewan K. (2009). Hallucination: a normal phenomenon? Dartmouth Journal of Undergraduate Science, November 21. Located at: http://dujs.dartmouth.edu/fall-2009/hallucination-a-normal-phenomenon

Johns, L.C., Nazroo, J.Y., Bebbington, P., et al. (2002). Occurrence of hallucinatory experiences in a community sample and ethnic variations. British Journal of Psychiatry, 180, 174-178.

Menon, G.J., Rahman, I. & Menon, SJ, et al. (2003) Complex visual hallucinations in the visually impaired: the Charles Bonnet Syndrome. Survey of Ophthalmology, 48, 58-72.

Ortiz de Gotari, A., Aronnson, K. & Griffiths, M.D. (2011). Game Transfer Phenomena in video game playing: A qualitative interview study. International Journal of Cyber Behavior, Psychology and Learning, 1(3), 15-33.
Rees, W.D. (1971). The hallucinations of widowhood. British Medical Journal, 4, 37-41.

Spence, S. A. (1993). Nintendo hallucinations: A new phenomenological entity. Irish Journal of Psychological Medicine, 10, 98–99.

Small claims caught: A beginner’s guide to Alice in Wonderland Syndrome

While researching a blog on Cotard Syndrome I came across a case study of Alice in Wonderland Syndrome published by Dr. Eric Bui and his colleagues published in the Journal of Neuropsychiatry and Clinical Neurosciences. They wrote:

“A 74-year-old retired French executive was admitted to hospital for major depressive disorder with psychotic features triggered by prostate surgery 3 months previously…The patient was described by his wife as usually sociable, jolly, scrupulous and a perfectionist. On admission, the patient presented with a depressed mood, loss of pleasure and interest, disordered sleeping, severe fatigue, loss of appetite, psychomotor retardation, and persecutory (being broken into and burgled) as well as somatic (his stools being contaminated) delusions…Ten days after admission, the patient exhibited new delusional symptoms: he believed his hands and feet were shorter than usual and was convinced that his clothes had shrunk. Organic causes (intracranial tumor and infection, thyroid disease, and nutritional deficiency) were ruled out by physiological, laboratory, neurological, and ophthalmological investigations…The patient continued to be delusional and severely depressed…He was discharged on day 45 of hospitalization with complete remission”.

As can be probably be surmised from this brief case study, Alice in Wonderland Syndrome (AIWS) is a non-contagious disorientation disorder and refers to when a person’s sense of body image, vision, hearing, touch, space, and/or time are distorted. AIWS sufferers typically experience micropsia (a neurological condition that affects human visual perception in which objects are perceived to be smaller than they actually are and make people feel bigger than they are) or macropsia (a neurological condition that affects human visual perception in which objects are perceived to be larger than they actually are and makes people feel smaller than they actually are). They may also experience feelings of paranoia.

AIWS has nothing to do with a malfunctioning of the eyes, but is a change in how the world is perceived with those suffering seeing objects the wrong shape or size. Arguably the most disturbing symptom for sufferers is the perceived alteration of their body image. AIWS sufferers become confused about the size and shape of their body (or specific body parts). Time perception may also be affected with many sufferers reporting that time seems to pass incredibly slowly as if they were on an LSD trip. Sufferers (most commonly thought to be children and migraine sufferers) often become very frightened, scared and panic-stricken, although it can often be treated successfully through complete rest and relaxation (and in most cases is a relatively temporary condition). Some research appears to indicate that AIWS can be due to abnormal amounts of electrical activity that causes blood to flow abnormally in the brain areas that process texture and visual perception.

AIWS was named after the 1865 book Alice’s Adventures in Wonderland by Lewis Carroll, but is also known as Todd Syndrome named after the psychiatrist who first wrote about the condition in a 1955 issue of the Canadian Medical Association Journal. (Interestingly, a short article by Dr. Klaus Podoll and Dr. Derek Robinson in The Lancet from 1999 highlighted that Carroll was a migraine sufferer and that his book may have been inspired by his own personal experiences!). Todd’s original paper reported five patients (of which four were female) all of who suffered from severe migraines. All of these patients described their body, body parts and/or objects around them changing in size (with two of them also having time disorientation too). On the basis of these five cases, Todd coined the term ‘Alice in Wonderland Syndrome’ (although such hallucinations had been noted three years prior to this by Dr. C.W. Lippman in a 1952 paper on certain hallucinations peculiar to migraine”).

The condition has also been associated with other medical conditions besides migraines, including (mononucleosis) infections, and severe depression, and (in extreme cases) brain tumours. A paper by Dr. Nabil Kitchener in a 2004 issue of the International Journal of Child Neuropsychiatry also noted that AIWS can also be the presenting manifestations in some patients with epilepsy, hyperpyrexia (i.e., extremely elevated body temperatures), typhoid encephalopathy, and other psychiatric disorders. In a 2005 paper in the journal European Neurology, Dr. Valmantas Budrys also reported that AWS could occur in hypnagogic, delirious states, encephalitis, cerebral lesions, drug intoxication, and schizophrenia.

In a literature review on organic depersonalization in the Journal of Neuropsychiatry and Clinical Neuroscience, Dr. Michelle Lambert and her colleagues examined the literature on AIWS. The noted that since Todd’s case studies were published, the subsequent published case reports of body image distortion associated with AIWS, often included depersonalization and/or derealization. They suggested that such symptom were consistent with parietal lobe pathology. More specifically, they argued that the frequent accompanying symptoms of fear, anxiety, and panic implicate the role of the temporal lobe. Dr. Kitchener’s 2004 paper also concludes that AIWS manifestations are due to disturbed function of either medial temporal, hippocampal, tempro-occipital or tempro-parieto-occipital regions of the brain based on the research of Dr. Kuo and colleagues published in a 1998 study in the Pediatric Neurology journal.

However, the case study published by Dr. Bui and his colleagues that I began this blog suggested that AIWS may be similar to Cotard Syndrome (CS). As the authors noted, that:

“Cotard Syndrome comprises any one of a series of delusions ranging from the belief that one has lost organs to the conviction that one is dead. Since Cotard’s syndrome is also usually associated with severe depression and improves rapidly with ECT [electro-convulsive therapy], it is possible that the somatic delusions experienced by our [AIWS] patient were a variant of this syndrome. According to our observations, the psychotic features of major depressive disorder might present in the form of Alice in Wonderland syndrome although the relationship between this syndrome and Cotard’s syndrome remains to be determined”.

Depending upon co-morbid conditions, medical treatments include beta blockers, anti-depressants, and anti-convulsants, AIWS has also been reported in both the Pediatric Infectious Diseases Journal (1987) and British Journal of Ophthalmology (1992) as one of the early signs of the Epstein-Barr Virus, one of the most common human viruses and of the herpes family. Most commonly it causes glandular fever but is associated with various forms of cancer including Hodgkin’s lymphoma. Chronic AIWS is untreatable and time is the only healer. Sharing experiences with other sufferers is also thought to be therapeutically beneficial (although I know of no clinical support for the claim).

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

Further reading

Budrys, V. (2005). Neurological eponyms derived from literature and visual art. European Neurology, 53, 171-178.

Bui, E., Chatagner, A. & Schmitt, L. (2010). Alice in Wonderland Syndrome in major depressive disorder. Journal of Neuropsychiatry and Clinical Neurosciences, 22, 352.e16-352.e16.

Cinbis, M. & Aysun, S. (1992). Alice in Wonderland syndrome as an initial manifestation of Epstein-Barr virus infection (case report). British Journal of Ophthalmology, 76, 316.

Eshel, G.M., Eyov, A., & Lahat, E., et al (1987). Alice in Wonderland syndrome, a manifestation of acute Epstein-Barr virus infection (brief report). Pediatric Infectious Diseases Journal, 6, 68.

Kew, J., Wright, A., & Halligan, P.W. (1998). Somesthetic aura: The experience of “Alice in Wonderland”. The Lancet, 351, 1934.

Kitchener, N. (2004). Alice in Wonderland Syndrome. International Journal of Child Neuropsychiatry, 1, 107-112.

Kuo, Y, Chiu, N.C., Shen, E.Y., Ho, C.S., Wu, M.C. (1998). Cerebral perfusion in children with “Alice in Wonderland” syndrome. Pediatric Neurology, 19, 105-108.

Lahat, E., Eshel, G., & Arlazoroff A (1990). “Alice in Wonderland” syndrome and infectious mononucleosis in children (letter). Journal of Neurology, Neurosurgery and Psychiatry, 53, 1104.

Lambert, M.V., Sierra, M., Phillips, M.L. & David, A.S. The spectrum of organic depersonalization: A review plus four new cases. Journal of Neuropsychiatry and Clinical Neuroscience, 14, 141-154.

Lippman, C.W. (1952). Certain hallucinations peculiar to migraine. Journal of Nervous and Mental Diseases, 116, 346-351.

Podoll, K., Ebel, H., Robinson, D., & Nicola, U. (2002). Obligatory and facultative symptoms of the Alice in wonderland syndrome. Minerva Medicine, 93, 287-293.

Podoll, K. & Robinson, D. (1999). Lewis Carroll’s migraine experiences. The Lancet, 353, 1366.

Rolak, L.A. (1991). Literary neurologic syndromes. Alice in Wonderland. Archives of Neurology, 48, 649–651.

Todd, J. (1955). The syndrome of Alice in Wonderland. Canadian Medical Association Journal, 73, 701–704.

An eye for an I! A beginner’s guide to auto-enucleation

I have to say that I have no idea what it must be like to lose an eye (i.e., enucleation) but one thing I can’t possibly begin to imagine is what it must like is to remove my own eye (i.e., auto-enucleation). However, there are many clinical and medical reports of people that self-mutilate by stabbing or removing their eye(s). Arguably the most infamous auto-enucleator was Oedipus (in Sophocles’ play) who removed both his eyes after he realized he had unwittingly slept with his own mother and killed his own father.

The psychiatrist Dr. Armando Favazza defines self-mutilation as “the deliberate, direct, non-suicidal destruction or alteration of one’s body tissue”. Dr. Niraj Ahuja and Dr. Adrian Lloyd writing in the Australian and New Zealand Journal of Psychiatry also add that self-mutilation relates to bodily self-damage without wishing to die. Dr. Favazza also believes there are three fundamentally different types of self-mutilation. Enucleation is included in the first type (major self-mutilation) and is the least common. Other forms of self-mutilation in this category include self-castration, penectomy (cutting off one’s own penis) and self-limb amputation.

The second type includes “monotonously repetitive and sometimes rhythmic acts such as head-banging, hitting, and self-biting” (which according to Dr Favazza occur mostly in “moderate to severely mentally retarded persons as well as in cases of autism and Tourette’s syndrome”). The final and most common forms of bodily self-mutilation are moderately superficial and include a compulsive sub-type (e.g., hair-pulling, skin scratching and nail-biting), as well as an episodic/repetitive sub-type (e.g., skin-cutting, skin carving, burning, needle sticking, bone breaking, and wound picking). Many of these self-harming behaviours are a symptom and/or an associated feature in a number of mental personality disorders (e.g., anti-social, borderline, and histrionic personality disorders).

Reports of auto-enucleation in the medical literature were first described in the 1840s. By the early 1900s, the act of removing one’s own eye was actually termed ‘Oedipism’ by Blonel. Auto-enucleation is (of course) exceedingly rare although a couple of studies in the American Journal of Ophthalmology (in 1984) and an analysis of 1,146 enucleations between 1980 and 1990 in the British Journal of Ophthalmology (in 1994) estimated there were 2.8 to 4.3 per 100,000 in the population. However, some papers (such as those by Dr. Favazza) on major self-mutilation have put the incidence as low as one in 4 million.

Enucleators are also known to be at increased risk of further self-harming, and (predictably) are more likely to be living in psychiatric institutions when the auto-enucleation event occurs. They are also at increased risk of removing the second eye at a later date if they didn’t pull out both eyes to start with. A review by Dr. H.R. Krauss and colleagues in a 1984 issue of the Survey of Opthalmology examined 50 cases of self-enucleation and reported that 19 of them had bilateral auto-enucleation (i.e., had removed both of their eyes). A 2007 paper by Dr. Alireza Ghaffari-Nejad and colleagues in the Archives of Iranian Medicine examined the many theories behind self-harming behaviour. They briefly overviewed theories ranging from Fruedian psychoanalytic theory to biologically-based theories. They wrote:

Psychoanalytically self-injurious behaviour has been linked to castration and explained as a process of failure to resolve oedipal complex, repressed impulses, self punishment, focal suicide and aggression turned inwards especially in cases of depression. [Other authors] have postulated interpersonal loss preceding self-injurious behaviour and linked it to rejection sensitivity…Biologically serotonergic depletion preceding self-mutilation has been linked to aggression and depression…Some authors have claimed strong moral, religious and delusional component”

A recent literature review by Dr. Alexander Fan in the journal Psychiatry reported that the vast majority of auto-enucleation cases suffer from psychotic illness (particularly schizophrenia) although other medical and/or psychiatric conditions associated with auto-enucleation include obsessive-compulsive neuroses, severe depression, post-traumatic stress disorders, drug-induced psychoses, bipolar mania. There are also case studies where auto-enucleation has been linked with structural brain lesions, Down Syndrome, epilepsy, neurosyphilis, and Lesch-Nyhan syndrome (juvenile gout). These are similar to other forms of extreme self-mutilation. For instance, self-mutilation in schizophrenia in response to auditory hallucinations has often been described as Van Gogh Syndrome (in reference to the painter’s self-excision of his own left ear)

Other reviews of the psychiatric literature have reported that those who remove their own eyes commonly have delusions (typically sexual and/or religious) and that when asked about motivations for self-harming include reasons such as guilt, atonement, sin, evil, etc. Although some authors have noted that enucleators with religious beliefs are often Christian, other case studies have made reference to other religious faiths (e.g., Muslims). Finally, another paper by Favazza in Hospital and Community Psychiatry concluded that:

“Males in a first episode of a schizophrenic illness that is characterized by delusions associated with a body part or religious delusions are at the greatest risk for MSM [major self-mutilation]. However, MSM of this severity is so rare that it cannot be predicted accurately unless there has been a previous attempt at self-injury or the patient has spoken about wanting to remove or injure an organ. Threatened ocular mutilation deserves special mention because it may occur in a hospital setting, and the case histories suggest that one-to-one nursing is not always be sufficient to prevent enucleation”.

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

Further reading

Berguaa, A., Sperling, W. & Kuchlea M. (2002). Self-enucleation in drug-related psychosis. Ophthalmologica, 216, 269-271.

Eric, J.C., Nevitt, M.P., Hodge, D. &  Ballard, D.J. (1984). Incidence of enucleation in a defined population. American Journal of Ophthalmology, 113, 138-44.

Fan, A.H. (2007). Autoenucleation: A case report and literature review. Psychiatry, October, 60-62.

Favazza, Armando (1998) ‘Introduction’, in Marilee Strong A Bright Red Scream: Self-mutilation and the Language of Pain. New York: Viking.

Favazza, A. & Rosenthal R. (1993). Diagnostic issues in self-mutilation. Hospital and Community Psychiatry, 44, 134-140.

Field, H. & Waldfogel, S. (1995). Severe ocular self-injury. General Hospital Psychiatry, 17, 224-227.

Gamulescu, M.A., Serguhn, S., Aigner, J.M., Lohmann, C.P., & Roider J. (2001). Enucleation as a form of self-aggression, two case reports and review of the literature. Klin Monatsbl Augenheilkd, 218, 451-454.

Ghaffari-Nejad, A., Kerdegari, M., & Reihani-Kermani, H. (2007) Self-mutilation of the nose in a schizophrenic patient with Cotard Syndrome. Archives of Iranian Medicine, 10, 540-542.

Gottrau, P., Holbach, L.M. & Nauman, G.O. (1994). Clinicopathological review of 1,146 enucleations (1980-90). British Journal of Ophthalmology, 78, 260-5.

Jeffreys, S. (2000). ‘Body art’ and social status: Cutting, tattooing and piercing from a feminist perspective Feminism and Psychology, 10, 409-429.

Krauss, H., Yee, R. & Foos, R. (1994). Autoenucleation. Survey of Ophthalmology, 29, 179-87.

MacLean, C. & Robertson, B.M. (1976). Self enucleation and psychosis. Archives of General Psychiatry, 33, 242-249.

Patil, B. & James, N. (2004). Bilateral self-enucleation of eyes. Eye, 18, 431-432.

Patton N. (2004). Self-inflicted eye injuries: A review. Eye, 18, 867-872.

Rao, K.N. & Begum, S. (1996) Self enucleation in depression; A case report. Indian Journal of Psychiatry, 38, 267-70

Witherspoon, D., Feist, F., Morris, R. & Feist, R. (1989). Ocular self-mutilation. Annals of Ophthalmology, 21, 255-259.

Private practices: A brief overview of male genital self-mutilation

One of the rarest behaviours in the world is the act of genital self-mutilation (GSM) in males. To date, approximately 125 cases have been recorded in the clinical literature dating back to the turn of the twentieth century. The first recorded case is thought to be a letter in the Journal of the American Medical Association by Dr D. Stroch in 1901.

GSM has been recorded in a variety of forms (e.g., simple lacerations, scrotal cutting, testicle removal, penile amputations, self-castration, and a combination of the above, so called ‘lock, stock and barrel mutilation’) across a variety of countries (USA, Middle East, India, Kenya, and Nigeria). There appears to be an increased incidence of GSM over the last decade although this may be due to increased reporting rather than increasing number of cases. GSM usually occurs in Caucasian men in the 20s and 30s (although there is a minority of cases from African and Indian descent and some case reports of individuals over the age of 70 years).

The range of instruments used to enable GSM include kitchen knifes, Stanley knives, scissors, blades, chain saw, and axe. In many cases, the genitals are disposed of immediately such as a recent case reported in the Saudi Medical Journal where a 37-year old male schizophrenic cut off both his penis and testicles and flushed them down the toilet.

A 1988 study by Tobias and colleagues in the South Medical Journal reported that self-mutilators (including all types of self-mutilation not just GSM) were most likely to suffer from schizophrenia (particularly command hallucinations), religious preoccupation, substance abuse, and/or social isolation. Genital self-mutilators are similar, and tend to fall into one of four types – schizophrenics, transsexuals (i.e., those with a gender identity crisis), those with complex cultural and religious beliefs, and a small number of severely depressed people who engage in GSM as part of a suicide attempt (around one-tenth of cases). A 1991 study in the journal Psychopathology also reported that GSM may also be triggered by a feeling of guilt for sexual offences. Similarly, Dr A.C. Waugh writing in the British Journal of Psychiatry concluded that GSM most commonly occurred in men with chronic paranoid schizophrenia and a history of delusions where only castration absolves them of guilt for sexual wrongdoing

A 2003 case report in the journal Urology, reported an attempt by an Indian man to become a ‘hijra’ (i.e., eunuch of the Indian subcontinent) due to his dissatisfaction with the wait for gender reassignment surgery. Reports indicate that transsexuals often resort to genital self-mutilation especially if they are unaware of the availability of professional (medical) help. Dr. D.B. Russell and colleagues in a 2005 issue of Sexual Health reported that genital mutilation that has a more ‘rational’ basis usually involves removal of the testicles (i.e., auto-castration) whereas those in a psychotic state are more likely to engage in penile amputation. An early study in 1993 by Aboseif and colleagues in the Journal of Urology reported that among a group of 14 genital self-mutilators, 61% of episodes involved the mutilation of one or both testicles. They also reported that among repeat mutilators, around one-third (31%) had a history of alcohol abuse and over a half (55%) had a history of drug abuse. The degree of injury didn’t differ between the psychotic and non-psychotic self-mutilators. Reporting on 52 cases in the Archives of General Psychiatry, Greilsheimer and Groves found 87% of genital self-mutilators to be psychotic and 13% to be non-psychotic. The psychotic individuals ranged from those with functional psychosis through to those with brain damage.

Those who engage in GSM as part of a religious belief are typically diagnosed as having Klingsor Syndrome. This was derived from the character Klingsor in Parsifol (a Wagner opera) who engaged in an act of self-castration to gain entry into the Brotherhood of the Knights of the Holy Grail. According to Samir Shirodkar and colleagues in the Saudi Medical Journal, group genital mutilation is a custom of a sect of Australian Aborigines where the blood is drunk by the infirm (who believe it restores their health).

In a fairly recent issue of the journal Mental Health and Substance Use, Dr Thomas Dunn and colleagues reported an unusual case of GSM. A 55-year-old non-psychotic homeless male turned up at hospital with penis and scrotal maggot infestation that was secondary to GSM. The man had gender identity issues and had performed GSM while he was drunk. However, he only sought medical help when he was barred from travelling on public transport because of the smell emanating from his maggot infection.

In a 2007 issue of the Jefferson Journal of Psychiatry, Dr. Craig Franke and Dr James Rush provided some risk factors that help in the identification of people at risk for GSM. These included: (i) psychotic patients with delusions of sexual guilt, (ii) psychotic patients with sexual conflict issues, (iii) prior self-destructive behaviour, (iv) depression, (v) severe childhood deprivation, and (vi) pre-morbid personality disorders. However, the condition is complex and as Dr Nagaraja Rao and colleagues highlighted in the Indian Journal of Psychiatry, “genital self mutilation like any other serious self injury is not a single clinical entity and it occurs in any psychiatric condition with corresponding psychopathology”.

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

Further reading

Aboseif, S., Gomez, R. & McAninch, J.W. (1993). Genital self-mutilation. Journal of Urology, 150, 1143-1146.

Ajape, A.A., Issa, B.A., Buhari, O.I.N., Adeoye, P.O., Babata, A.L. & Abiola, O.O. (2010). Genital self-mutilation. Annals of African Medicine, 9, 31-34.

Dunn, T.M., Collins, V., House, R.M. & Dunn, P.W. (2009). Male genital self-mutilation with maggot infestation in an intoxicated individual. Mental Health and Substance Use, 2, 235-238.

Eke N. (2000). Genital self-mutilation: there is no method in this madness. BJU International, 85, 295-298.

Franke, C.B. & Rush, J.A. (2007). Autocastration and autoamputation of the penis in a patient with delusions of sexual guilt. Jefferson Journal of Psychiatry, 21, Located at: jdc.jefferson.edu/jeffjpsychiatry

Greilsheimer, H. & Groves, J.E. (1979). Male genital self-mutilation. Archives of General Psychiatry, 36, 441.

Martin, T. & Gattaz, W.F. (1991). Psychiatric aspects of male genital mutilations. Psychopathology, 24, 170.

Master, V. & Santucci, R. (2003). An American hijra: A report of a case of genital self-mutilation to become India’s ‘‘third sex’’. Urology, 62, 1121.

Murota-Kawano, A, Tosaka, A. & Ando, M. (2001). Autohemicastration in a man without schizophrenia. International Journal of Urology, 8, 257-259.

Rao, K.N., Bharathi, G., & Chate S. (2002). Genital self-mutilation in depression: A case report. Indian Journal of Psychiatry. 44, 297-300.

Russell, D.B., McGovern, G. & Harte, F.B. (2005). Genital self-mutilation by radio frequency in a male-to-female transsexual. Sexual Health, 2, 203-204.

Shirodkar, S.S., Hammad, F.T. & Qureshi, N.A. (2007). Male genital self-amputation in the Middle East: A simple repair by anterior urethrostomy. Saudi Medical Journal, 28, 791-793.

Stroch, D. (1901). Self castration (Letter to the Editor). Journal of the American Medical Association, 36, 270.

Schweitzer, I. (1990). Genital self-amputation and the Klingsor syndrome. Australian and New Zealand Journal of Psychiatry, 24, 566-569.

Stunnell, H., Power, R.E., Floyd, M., & Quinlan, D.M. (2006). Genital self-mutilation. International Journal of Urology, 13, 1358-1360.

Tobias, C.R., Turns, D.M., Lippmann., S., Pary, R. & Oropilla, T.B. (1988) Evaluation and management of self-mutilation. South Medical Journal, 81(10), 1261-1263.

Waugh, A.C. (1986). Autocastration and biblical delusions in schizophrenia. British Journal of Psychiatry, 149, 656-658.