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

Spare fib: Should pathological lying be considered a mental disorder?

In a previous blog on weird addictions, compulsions and obsessions, I briefly looked at pathological lying. Writings relating to pathological lying first appeared in the psychiatric literature over 100 years ago and have been given names such as ‘pseudologia fantastica’ and ‘mythomania’ and often used interchangeably. There is some consensus that Dr. Anton Delbruck, a German physician was the first person to describe the concept of pathological lying in 1891 after publishing an account of five of his patients. Despite the long history of research, pathological lying is not included in either the American Psychiatric Association’s Diagnostic and Statistical Manual (DSM-IV) or the World Health Organization’s International Classification of Diseases (ICD-10). The only mention of pathological lying in the DSM-IV is in association with Factitious Disorder (discussed below), However, many psychologists and psychiatrists claim that it is a distinct psychiatric disorder as highlighted in the many papers that have been published on the topic over the last two decades.

At a very simplistic level, pathological lying refers to a person that incessantly tells lies. However, Dr Charles Dike and his colleagues at Yale University define it as “falsification entirely disproportionate to any discernible end in view, may be extensive and very complicated, and may manifest over a period of years or even a lifetime, in the absence of definite insanity, feeble-mindedness or epilepsy”. However, there are other psychiatric conditions (such as people with Manipulative Personality) that may also engage in pathological lying as part of a wider set of behaviours and symptoms. In fact, there is a lot of debate as to whether the behaviour is really a discrete and unique entity or whether it typically manifests itself as an adjunct to other recognized psychological and/or psychiatric conditions. Dr Dike and colleagues note that:

“Pathological liars can believe their lies to the extent that, at least to others, the belief may appear to be delusional; they generally have sound judgment in other matters; it is questionable whether pathological lying is always a conscious act and whether pathological liars always have control over their lies; an external reason for lying (such as financial gain) often appears absent and the internal or psychological purpose for lying is often unclear; the lies in pathological lying are often unplanned and rather impulsive; the pathological liar may become a prisoner of his or her lies; the desired personality of the pathological liar may overwhelm the actual one; pathological lying may sometimes be associated with criminal behavior; the pathological liar may acknowledge, at least in part, the falseness of the tales when energetically challenged; and, in pathological lying, telling lies may often seem to be an end in itself. However, it is evident that no single descriptive tableau of a pathological liar settles all the nosological and etiological questions raised by the phenomenon of pathological lying” (p.344)

Dike and colleagues then went on to list a wide range of psychiatric conditions that have been associated pathological lying in an attempt to contextualize how the lying behaviour is manifested within these known conditions. The list of psychological and psychiatric conditions included: (i) Malingering, (ii) Confabulation, (iii) Ganser’s Syndrome, (iv) Factitious Disorder, (v) Borderline Personality Disorder, (vi) Antisocial Personality Disorder, (vii) Histrionic Personality Disorders. Arguably it is these last three disorders with which pathological lying is most associated with. The following briefly describes the symptoms and context of each of these conditions as outlined by Dr Dike and his colleagues:

• Malingering: This is deliberate lying where the person grossly exaggerates or totally lies about physical and/or psychological symptoms. Unlike ‘archetypal’ pathological liars, malingerers are typically motivated to tell lies for a specific purpose such as to obtain financial compensation, to avoid working, to avoid military service, to avoid criminal prosecution, etc.
• Confabulation: This is where people tell lies incessantly as a way of covering up memory lapses caused by specific memory loss conditions (e.g., organically derived amnesia). In ‘archetypal’ pathological liars, the condition is psychological (rather than organic) in origin.
• Ganser’s Syndrome (GS): GS is a rare dissociative disorder (only 101 recorded cases ever) characterized by affected people giving nonsensical answers to questions (and goes under many other names including ‘nonsense syndrome’ and ‘balderdash syndrome’). Unlike the elaborate and sometimes fantastical stories told by ‘archetypal’ pathological liars, the lies told by those with GS are very simplistic and approximate.
• Factitious Disorder (FD): FD is the deliberate use of lies and/or exaggerations concerning psychological and/or physical symptoms solely for the purpose of assuming the role of a sick person (formerly known as Munchausen’s Syndrome). In contrast, the ‘archetypal’ pathological liar doesn’t want to appear sick to other people.
• Borderline Personality Disorder (BPD): BPD is the condition where people have long-term patterns of unstable and/or turbulent emotions. Pathological lying and being deceitful are core characteristics of BPD and lies are typically told for personal profit or pleasure. Although. BPD patients typically have contradictory views about themselves and lack a consistent self-identity. A lack of impulse control may facilitate the distortions and lies told.
• Antisocial Personality Disorder (APD): APD is the condition in which the sufferer has a long-term pattern of manipulating, exploiting, or violating the rights of others (and is often criminal). Those with APD often lie repeatedly and consistently for personal satisfaction alone. Although those with APD are often pathological liars, ‘archetypal’ pathological liars rarely have disordered antisocial personalities.
• Histrionic Personality Disorder (HPD): Those with HPD act in a highly emotional and dramatic way to draw attention to themselves. They often lie as a way to enhance and/or facilitate their dramatic and attention-seeking behaviour. In contrast, ‘archetypal’ pathological liars do not constantly seek attention.

Based on the list above, it is evident that the symptom of pathological lying can occur in some mental disorders (e.g., FD, BPD) and could be called secondary pathological lying. However, it is much less clear whether it can occur independently of a known psychiatric disorder and be seen as primary pathological lying. Unlike other the other forms of lying outlined above, Dr Dike says pathological lying appears to be unplanned and impulsive. Despite all the speculation, there is still relatively little known although it’s thought to affect men and women equally with an onset in late adolescence. There are no reliable prevalence figures although one study estimated that one in a 1000 repeat juvenile offenders suffered from it.

On a biological and neurological level, a paper published in the Journal of Neuropsychiatry and Clinical Neurosciences reported the case of a pathological liar who was given a brain scan. Results showed that his condition was associated with right hemithalamic dysfunction. This supported the hypothesized roles of the thalamus and associated brain regions in the modulation of behavior and cognition.

A study published in the British Journal of Psychiatry reported differences in brain structure between pathological liars and control groups. Pathological liars showed a relatively widespread increase in white matter (approximately one-quarter to one-third more than controls) and the authors suggested that this increase may predispose some individuals to pathological lying.

Those working in the mental health system need to pay attention to pathological lying so that they can inform legal practitioners about whether pathological liars should be held responsible for their behaviour. Whether pathological liars are aware of the lies they tell has major implications for forensic psychiatry practice. Dr Dike says it could help determine how a court deals with pathological liars who provide false testimony while under oath.

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

Further reading

Delbruck, A (1891). Die pathologische Luge und die psychisch abnormen Schwindler: Eine Untersuchung uber den allmahlichen Uebergang eines normalen psychologischen Vorgangs in ein pathologisches Symptom, fur Aerzte und Juristen. Stuttgart, 1891, p 131.

Dike, C.C., Baranoski, M. & Griffith, E.E.H. (2005). Pathological lying Revisited. Journal of the American Academy of Psychiatry and Law 33, 342-349.

Healy W, Healy MT: Pathological Lying, Accusation, and Swindling. Boston: Little, Brown, 1926

King, B.H. & Ford, C.V. (1988). Pseudologia fantastica. Acta Psychiatrica Scandinavica, 77, 1-6.

Miller, P., Bramble, D., & Buxton, N. (1997). Case study: Ganser syndrome in children and adolescents. Journal of the American Academy of Child and Adolescent Psychiatry, 36, 112-115.

Modell, J.G., Mountz, J.M. & Ford, C.V. (1992). Pathological lying associated with thalamic dysfunction demonstrated by [99mTc]HMPAO SPECT. Journal of Neuropsychiatry and Clinical Neurosciences, 4, 442-446.

Yang, Y., Raine, A. Narr, K.L., Lencz, T., LaCasse, L., Colletti, P. & Toga, A.W. (2007). Localisation of increased prefrontal white matter in pathological liars. British Journal of Psychiatry, 190, 174-175.