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
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.
In a previous blog I examined clinical lycanthropy, a delusional psychiatric syndrome or neurological condition in which individuals believe they are transforming (or have already have transformed) into a non-human animal (often – but not necessarily – a wolf). As I noted in that blog, these identity disorders should be really be referred to as ‘therianthropy’ (as noted in Dr. Edward Podolsky’s 1953 Encyclopedia of Aberrations) or ‘zoanthropy’ but in the psychiatric literature it is ‘lycanthropy’ that tends to be used as the ‘catch-all’ name of the disorder.
A 2004 study by Dr. Petra Garlipp and her German colleagues in the journal Acta Psychiatrica Scandinavica noted the different types of animal transformation that have occurred in the psychological and psychiatric literature. Obviously human-to-wolf (or other canine) cases exist, but they appear to be in the minority. Other types include humans believing they have transformed into cats, dogs, tigers, hyenas, horses, birds, frogs and bees. Predictably, psychiatry authors claimed that the mythology surrounding human-animal transformation is controversial in Western popular culture.
Perhaps one of the most unusual cases of therianthropy was a case described by Drs. Shivanand Kattimani, Vikas Menon, Manohar Kant Srivastava and Aniruddha Mukharjee in a 2010 issue of the online journal Psychiatry Reports. They published a case report of about a 24-year old well educated woman who believed she had turned into a live snake. They published the case study because of its rarity and unique phenomenology. The case also provided difficult diagnostic problems and treatment was a challenging. The authors wrote that she:
“…presented to us with complaints that she had died 15 days before and that in her stead she had been turned into a live snake. At times she would try to bite others’ claiming that she was a snake. Her food intake and other behaviour remained as normal. We showed her photos of snakes and when she was made to face the large mirror she failed to identify herself as her real human self and described herself as snake. She described having snake skin covering her and that her entire body was that of snake except for her spirit inside. She repeatedly said that her saliva was that of snake’s and very poisonous. She would often complain of difficulty in swallowing telling that was due to snake skin being wrapped around her neck. She had protruding sharp upper incisor teeth which she could claim were snake fangs. She was distressed that others did not understand or share her conviction. She felt hopeless that nothing could make her turn into real self. She made suicidal gestures and attempted to hang herself twice on the ward and once when she was sent for short leave home. There were depressive symptoms accompanying this theme. There was no significant contributory medical history”.
The woman was subsequently treated with ten sessions of electro-convulsive therapy (ECT) along with a variety of drugs. The authors first tried fluoxetine (but there was no response), followed by risperidone, then olanzapine, trifluperazine, and carbamazepine. She remained on the psychiatric ward for nearly half a year “and during each drug trial there was some initial improvement in her symptoms but later she would return back to same conviction of her beliefs”. The ECT was administered to inhibit her severe depression and suicidal ideation. At the time the case study was written up for publication she was administered a drug combination of escitalopram and quetiapine but still had the same psychopathology as when she entered treatment (in fact the authors . In a discussion of the case, the authors noted:
“Our initial diagnosis – as she predominantly presented with depressive symptoms and suicidal attempt along with delusion of nihilism – was a severe depressive disorder with psychotic symptoms…As she additionally had a delusion of being transformed into a snake we consider the diagnosis of an acute transient psychosis. While she remained in the ward her behaviour with our other patients and personal hygiene was well preserved except for brief periods of exacerbation of her psychopathology lasting for few hours to two days wherein she would enact being a snake”
The authors also considered possible diagnoses of both dissociative disorder and schizophrenia. However, the authors decided that her “psychopathology pointed towards something more than that”. They also noted that the delusion that she had become a snake “was bizarre from the point of view of the patient’s cultural background”. Consequently (and after discussion with other psychiatric colleagues) the authors reached no consensus on the diagnosis for the disorder (except that it was clearly a psychotic illness). They did, however, propose that this “novel delusion” could be called ‘ophidanthropy’. The patient’s relatives weren’t interested in diagnosis, only that she be treated. However, this was something that the authors admitted they “were unable to provide”. In the paper’s conclusions, the authors said they planned next to try “supportive psychotherapy in combination with pharmacotherapy” but the results of these therapies have not yet (to my knowledge) been published.
Interestingly, the authors commented in passing that the woman believed she was dead but they made no other reference to this at all in their paper. To me, this looks like a possible case of Cotard’s Syndrome (where individuals hold the delusional belief that they are dead). I did note in my previous blog on clinical lycanthropy that there was an interesting case report in the psychiatric literature of a man who had both clinical lycanthropy and Cotard’s Syndrome. This case was reported by Dr. A.G. Nehad and Dr. K. Toofani in a 2005 issue of the journal Acta Psychiatrica Scandinavica. Their patient had a bipolar mood disorder, along with a psychotic delusion that he had transformed into a dog. He also suffered from the delusion that he was dead. There would certainly seem to be comparisons that can be made between these two cases.
Dr Mark Griffiths, Professor of Gambling Studies, International Gaming Research Unit, Nottingham Trent University, Nottingham, UK
Garlipp, P., Gödecke-Koch, T., Dietrich, D.E. & Haltenhof, H (2004). Lycanthropy: Psychopathological and psychodynamic aspects. Acta Psychiatrica Scandinavica, 109, 19-22.
Kattimani, S, Menon, V., Srivastava, M.K. & Aniruddha Mukharjee, A. (2010). Ophidianthropy The case of a woman who ‘turned into a snake’. Psychiatry Reports. Located at: http://www.priory.com/psychiatry/ophidianthropy.htm
Keck, P.E., Pope, H.G., Hudson, J.I., McElroy, S.L. & Kulick, A.R. (1988). Lycanthropy: alive and well in the twentieth century. Psychological Medicine, 18, 113–20.
Larner, A.J (2010). Neurological signs: Lycanthropy. Advances in Clinical Neurocience and Rehabilitation, 10(4), 50.
Nejad, A.G. & Toofani, K. (2005). Invited comment (on ‘Co-existence of lycanthropy and Cotard’s syndrome in a single case’). Acta Psychiatrica Scandinavica, 111, 250-252.
Podolsky, E. (1953). Encyclopedia of Aberrations: A Psychiatric Handbook. New York: Philosophical Library.
One of the strangest and rarest mental disorders that has been studied academically is Cotard’s Syndrome (CS), and also known as the Cotard Delusion, the Nihilistic Delusion, and the Walking Corpse Syndrome, CS is where individuals hold the delusional belief that they are dead (figuratively or literally) and do not exist. Alternative versions of CS involve the individual believing they are putrefying and/or have lost their blood and/or internal organs. In even rarer instances the condition may include delusions of immortality. Unsurprisingly, CS is often found in psychotic individuals with schizophrenia but has also presented itself following adverse reactions to the anti-viral drug acyclovir (typically used in the treatment of herpes simplex virus). Some research has also indicated that individuals with impaired renal function are also at a higher risk of displaying CS symptoms.
CS is named after the 19th century French neurologist Jules Cotard who first described the condition in 1880. Cotard named the disorder the ‘negation delerium’ (le délire de négation). The severity of the disorder can differ between individuals (i.e., mild to severe). The characteristics of CS include despair and self-loathing at one end through to intense delusions and chronic depression at the other. The original case described by Cotard was a female given the pseudonym ‘Mademoiselle X’. She eventually died of starvation (because she believed she didn’t need to eat and denied that some parts of her body even existed) and believed she was eternally damned.
According to Yamada and colleagues in a 1999 issue of Acta Psychiatrica Scandinavica, CS has three distinct stages (i.e., germination, blooming and chronic). The first (germination) stage comprises psychotic depression and hypochondria. The second (blooming) stage comprises the full development of the syndrome and the delusions of negation. In the final (chronic) stage comprises severe delusions and chronic depression, and leads to a completely distorted view of the world in which they live. They also become socially withdrawn, completely neglect their hygiene, and cannot make sense of reality. As far back as 1921, Tissot described two components of the syndrome – an affective component associated with anxiety and a cognitive component associated with the presence of delusion.
Here are a couple of examples from the clinical literature.
- Example 1: In 1996, Dr. A.W. Young and Dr. K.M. Leafhead reported a case of CS in a Scottish man who suffered brain injury following a motorcycling accident. Following his discharge from hospital, he was taken to South Africa by his mother and became convinced that he had died of septicaemia or AIDS and had gone to hell.
- Example 2: A case study reported by Dr. D.N. Mendhekar and Dr. N. Gupta in a 2005 issue of the Indian Journal of Pediatrics described CS in a 14-year-old male epileptic. He had a history of depression, social withdrawal, talking about death, and disturbed biological function. His CS episodes occurred around twice a year (lasting 1-3 months each time). During each affected episode, the child believed that he and everyone around was dead.
Recently, Hans Debruyne and colleagues wrote an excellent review of CS in a 2011 issue of Mind and Brain, and wrote what they believed is a “classical description” of CS:
“In its early stages, Cotard’s syndrome is characterized by a vague feeling of anxiety, with a varying time span from weeks to years. This anxious state gradually augments and can result in nihilistic delusions where denial of life or denial of body parts are the prominent features. The patient loses sense of reality. Despite the delusion of being dead, these patients show an increased tendency to automutilation or suicidal behavior. Additional symptoms may include analgesia and mutism. The core symptoms always reflect a preoccupation with guilt, despair, and death”.
In the same 2011 review, the authors examined all the epidemiological studies and summarized the findings:
“The prevalence and incidence of this rare syndrome is not known. Only one study reported on prevalence in a selected psychogeriatric population in Hong Kong. In 2 out of 349 patients, Cotard’s syndrome was diagnosed suggesting a prevalence of 0.57% in this population. A prevalence of 3.2% was reached when severely depressed elderly were included. A recent study found 0.62% (n=3) of patients in a Mexican sample (screened over a 2-year period) of primary psychiatric patients having Cotard’s syndrome. Using the same methodology they found also 0.11% (n=1) having Cotard’s syndrome in a sample of neurological patients with mental disturbances”.
In 1995, Dr. G.E Berios and Dr. R. Luque (University of Cambridge, UK) carried out a statistical analysis of 100 cases of CS. They reported no differences between men and women with CS, and age appeared to increase the likelihood of someone developing CS. In relation to affective disorders and mood state, the authors reported that CS sufferers experienced depression (89%), anxiety (65%) and guilt (63%). In relation to delusions, CS sufferers experienced hypochondriacal delusions (58%) and delusions of immortality (55%). The most common nihilistic delusions concerned the body (86%) and existence (69%). The authors also carried out exploratory factor analysis and reported that the factors appeared to suggest three types of CS (i.e., Psychotic Depression Type; Cotard Type I; and Cotard Type II) and that each type may need to be treated differently and have different therapeutic implications.
- The Psychotic Depression type includes individuals with melancholia and a few nihilistic delusions.
- The Cotard Type I includes individuals with no depression or other disease and comprises a “pure” CS whose nosology is closer to delusional disorders (rather than the affective disorders).
- The Cotard Type II includes individuals with anxiety, depression and auditory hallucinations and comprise a mixed group.
Neurologically, CS is thought to be related to the Capgras Syndrome (i.e., a disorder in which an individual has the delusion that someone close to them – partner, friend, or family member – has been replaced by an identical-looking imposter). Both syndromes are thought to originate from a disconnection between the areas of the brain that recognize faces and the areas that associate emotional content with such facial recognition. This can lead to feelings of derealization (i.e., an alteration in experience or perception of the external world so that it seems unreal). Brain scans show that those displaying CS often have lesions in the parietal lobe and greater brain atrophy than those in control groups. There are several reports of successful treatment in the psychiatric literature with ant-depressants, anti-psychotics, and various mood stabilizers. Therapeutic interventions such as electro-convulsive therapy have also been used to positive effect. In their 2011 review, Debruyne and colleagues concluded:
“Despite that Cotard’s syndrome was first described more than a century ago, literature on this topic remains restricted to case reports. Although the syndrome is rare, more larger scale research is needed to further clarify the pathophysiologic underpinnings of the disease and its relation to other delusions of misidentification such as Capgras syndrome”.
Berrios G.E. & Luque R. (1995) Cotard’s delusion or syndrome? Comprehensive Psychiatry, 36, 218-223.
Berrios G.E. & Luque R. (1995) Cotard Syndrome: clinical analysis of 100 cases. Acta Psychiatrica Scandinavica, 91, 185-188.
Berrios G.E. & Luque R. (1999) Cotard’s ‘On hypochondriacal delusions in a severe form of anxious melancholia’. History of Psychiatry, 10, 269-278.
Cohen, D. & Consoli, A. (2006). Production of supernatural beliefs during Cotard’s syndrome, a rare psychotic depression. Behavioral and Brain Sciences, 29, 468-470.
Debruyne H, Portzky M, Peremans, K. & Audenaert K (2011). Cotard’s syndrome. Mind and Brain, 2, 67-72.
Debruyne H, Portzky M, Van den Eynde F. & Audenaert K (2009). Cotard’s syndrome: a review. Current Psychiatry Reports, 11, 197-202.
Mendhekar, D. N., & Gupta, N. (2005). Recurrent postictal depression with Cotard delusion. Indian Journal of Pediatrics, 72, 6, 529-31.
Pearn, J. & Gardner-Thorpe, C (2002). Jules Cotard (1840-1889) His life and the unique syndrome that bears his name. Neurology, 58,1400–3.
Tissot F. (1921). De lire des negations termine par guerison. Cosiderations sur l’hypochondrie et la melancholie. Ann Med Psychol, 79, 321-328.
Yamada, K., Katsuragi, S. & Fujii, I. (1999). A case study of Cotard’s syndrome: stages and diagnosis. Acta Psychiatrica Scandinavica, 100, 396-399.
Young, A.W., Robertson, I.H., Hellawell, D.J., de, P.K.W., & Pentland, B. (1992). Cotard delusion after brain injury. Psychological Medicine, 22, 799-804.
Young, A.W. & Leafhead, K.M. (1996) Betwixt life and death: Case studies of the Cotard Delusion. In P.W. Halligan & J.C. Marshall. (Eds.), Method in Madness: Case studies in Cognitive Neuropsychiatry). Hove: Psychology Press.