Alien hand syndrome

Alien hand syndrome
Classification and external resources
ICD-9	781.8
MeSH	D055964

Alien hand syndrome (also known as anarchic hand or Dr. Strangelove syndrome^[1]) is a neurological disorder in which the afflicted person's hand appears to take on a mind of its own. Alien hand syndrome is best documented in cases where a person has had the two hemispheres of their brain surgically separated, a procedure sometimes used to relieve the symptoms of extreme cases of epilepsy. It also occurs in some cases after other brain surgery, strokes, or infections.

Symptoms

A person with alien hand syndrome can feel normal sensation in the hand and leg, but believes that the hand, while still being a part of their body, behaves in a manner that is totally distinct from the sufferer's normal behavior. They lose the 'sense of agency' associated with the purposeful movement of the limb while retaining a sense of 'ownership' of the limb. They feel that they have no control over the movements of the 'alien' hand, but that, instead, the hand has the capability of acting autonomously — i.e., independent of their voluntary control. The hand effectively has 'a will of its own.' "Alien behavior" can be distinguished from reflexive behavior in that the former is flexibly purposive while the latter is obligatory. Sometimes the sufferer will not be aware of what the alien hand is doing until it is brought to his or her attention, or until the hand does something that draws their attention to its behavior. A related syndrome described by the French neurologist François Lhermitte involves the release through disinhibition of a tendency to compulsively utilize objects that present themselves in the surrounding environment around the patient (Lhermitte 1983; Lhermitte et al. 1986). The behavior of the patient is, in a sense, obligatorily linked to the "affordances" (using terminology introduced by the American ecological psychologist, James J. Gibson) presented by objects that are located within the immediate peri-personal environment. This condition, termed "utilization behavior", is most often associated with extensive bilateral frontal lobe damage and might actually be thought of as "bilateral" alien hand syndrome in which the patient is compulsively directed by external environmental contingencies (e.g., the presence of a hairbrush on the table in front of them elicits the act of brushing the hair) and has no capacity to "hold back" and inhibit pre-potent motor programs that are obligatorily linked to the presence of specific external objects in the peri-personal space of the patient. When the frontal lobe damage is bilateral and generally more extensive, the patient completely loses the ability to act in a self-directed manner and becomes totally dependent upon the surrounding environmental indicators to guide his behavior in a general social context, a condition also identified by Lhermitte (1986), and referred to as "Environmental Dependency Syndrome".

Sufferers of alien hand will often personify the rogue limb, for example believing it to be "possessed" by some intelligent or alien spirit or an entity that they may name or identify. There is a clear distinction between the behaviors of the two hands in which the affected hand is viewed as "wayward" and sometimes "disobedient" and generally out of the realm of their own voluntary control, while the unaffected hand is under normal volitional control. At times, particularly in patients who have sustained damage to the corpus callosum that connects the two cerebral hemispheres (see also split-brain), the hands appear to be acting in opposition to each other. For example, one patient was observed putting a cigarette into her mouth with her intact, 'controlled' hand (her right, dominant hand), following which her alien, non-dominant, left hand came up to grasp the cigarette, pull the cigarette out of her mouth, and toss it away before it could be lit by the controlled, dominant, right hand. The patient then surmised that "I guess 'he' doesn't want me to smoke that cigarette." This type of problem has been termed "intermanual conflict" or "diagonistic Ideomotor apraxia."

Subtypes

There are several distinct subtypes of alien hand syndrome that appear to be associated with specific distributions of associated brain injury.

Corpus Callosum

Damage to the corpus callosum can give rise to "purposeful" actions in the sufferer's non-dominant hand (an individual who is left-hemisphere-dominant will experience the left hand becoming alien, and the right hand will turn alien in the person with right-hemisphere dominance) as well as a problem termed "intermanual conflict" in which the two hands appear to be directed at opposing purposes.

Frontal lobe

Unilateral injury to the brain's frontal lobe can trigger reaching, grasping and other purposeful movements in the contralateral hand. With anteromedial frontal lobe injury, these movements are often exploratory reaching movements in which external objects are frequently grasped and utilized functionally, without the simultaneous perception on the part of the patient that they are "in control" of these movements. Once an object is maintained in the grasp of this "frontal variant" form of alien hand, the patient often has difficulty with voluntarily releasing the object from grasp and can sometimes be seen to be peeling the fingers of the hand back off the grasped object using the opposite controlled hand to enable the release of the grasped object.

Parietal and occipital lobes

A distinct "posterior variant" form of alien hand syndrome is associated with damage to the posterolateral parietal lobe and/or occipital lobe of the brain. The movements in this situation tend to be more likely to withdraw the palmar surface of the hand away from environmental contact rather than reaching out to grasp onto objects to produce palmar tactile stimulation, as is most often seen in the frontal form of the condition. Alien movements in the posterior variant of the syndrome also tend to be less coordinated and show a coarse ataxic motion during active movement that is generally not observed in the frontal form of the condition. The alien limb in the posterior variant of the syndrome may be seen to 'levitate' upward into the air and away from contact surfaces. Alien hand movement in the posterior variant may show a typical posture, sometimes referred to as a 'parietal hand' or 'instinctive avoidance reaction' (a term introduced by neurologist Derek Denny-Brown), in which the digits move into a highly extended position and the palmar surface is pulled back away from approaching objects. The 'alien' movements, however, remain purposeful and goal-directed, a point which clearly differentiates these movements from other forms of involuntary limb movement (e.g., athetosis, chorea, or myoclonus).

Similarities between frontal and posterior variants

In both the frontal and the posterior variants of the alien hand syndrome, the patient's reactions to the limb's apparent capability to perform goal-directed actions independent of conscious volition is similar. In both of these variants of alien hand syndrome, the alien hand emerges in the hand contralateral to the damaged hemisphere.

Explanatory theories

The common emerging factor in alien hand syndrome is that the primary motor cortex controlling hand movement is isolated from Premotor cortex influences but remains generally intact in its ability to execute movements of the hand.

A 2009 fMRI study looking at the temporal sequence of activation of components of a cortical network associated with voluntary movement in normal individuals demonstrated "an anterior-to-posterior temporal gradient of activity from supplemental motor area through premotor and motor cortices to the posterior parietal cortex".^[2] Therefore, with normal voluntary movement, the emergent sense of agency appears to be associated with an orderly sequence of activation that develops initially in the anteromedial frontal cortex in the vicinity of the supplementary motor complex on the medial surface of the hemisphere prior to activation of the primary motor cortex.

A 2007 fMRI study examining the difference in functional brain activation patterns associated with alien as compared to non-alien 'volitional' movement in a patient with alien hand syndrome found that alien movement involves isolated activation of the contralateral secondary motor cortex, while non-alien movement involved the activation of primary motor cortex in concert with frontal and parietal association cortex presumably involved in a cortical network generating premotor influences on the primary motor cortex.^[3]

Disconnection

It is theorized^{[according to whom?]} that alien hand syndrome results when disconnection occurs between different parts of the brain that are engaged in different aspects of the control of bodily movement. As a result, different regions of the brain are able to command bodily movements, but cannot generate a conscious feeling of self-control over these movements. As a result, the "sense of agency" that is normally associated with voluntary movement is impaired or lost. There is a dissociation between the process associated with the actual execution of the physical movements of the limb and the process that produces an internal sense of voluntary control over the movements, with this latter process thus normally creating the internal conscious sensation that the movements are being internally initiated, controlled and produced by an active self.

Recent studies have examined the neural correlates of emergence of the sense of agency under normal circumstances.^[4] This appears to involve consistent congruence between what is being produced through efferent outflow to the musculature of the body, and what is being sensed as the presumed product in the periphery of this efferent command signal. In alien hand syndrome, the neural mechanisms involved in establishing that this congruence has occurred may be impaired. This may involve an abnormality in the brain mechanism that differentiates between "re-afference" (i.e., the return of kinesthetic sensation from the self-generated 'active' limb movement) and "ex-afference" (i.e., kinesthetic sensation generated from an externally-produced 'passive' limb movement in which an active self does not participate). This brain mechanism is proposed to involve the production of a parallel "efference copy" signal that is sent directly to the somatic sensory regions and is transformed into a "corollary discharge," an expected afferent signal from the periphery that would result from the performance driven by the issued efferent signal. The correlation of the corollary discharge signal with the actual afferent signal returned from the periphery can then be used to determine if, in fact, the intended action occurred as expected. When the sensed result of the action is congruent with the predicted result, then the action can be labelled as self-generated and associated with an emergent sense of agency.

If, however, the neural mechanisms involved in establishing this sensorimotor linkage associated with self-generated action are faulty, it would be expected that the sense of agency with action would not develop.

Loss of inhibitions

One theory posed^{[according to whom?]} to explain these phenomena proposes that the brain has separable neural "premotor" or "agency" systems for managing the process of transforming intentions into overt action. An anteromedial frontal premotor system is engaged in the process of directing exploratory actions based on "internal" drive by releasing or reducing inhibitory control over such actions. Damage to this system produces disinhibition and release of such actions which then occur autonomously. A posterolateral temporo-parieto-occipital premotor system has a similar inhibitory control over actions that withdraw from environmental stimuli as well as the ability to excite actions that are contingent upon and driven by external stimulation, as distinct from internal drive. These two hemispheric systems interact through mutual inhibition that maintains a balance between responding to and withdrawing from environmental stimuli in the behavior of the contralateral limbs (Denny-Brown, 1956, 1958, 1966). Together, these hemispheric agency systems form an integrated intrahemispheric agency system.

When the anteromedial frontal system is damaged, involuntary but purposive movements of an exploratory reach-and-grasp nature—what Denny-Brown (1956, 1966) referred to as a positive cortical tropism—are released in the contralateral limb.

When the posterolateral parieto-occipital system is damaged, involuntary purposive movements of a release-and-retract nature, such as levitation and instinctive avoidance, are released.

Each intrahemispheric agency system has the capability of acting autonomously in its control over the contralateral limb although unitary integrative control of the two hands is maintained through interhemispheric communication between these systems via the projections traversing the corpus callosum at the cortical level and other interhemispheric commissures linking the two hemispheres at the subcortical level.

Disconnection of hemispheres due to injury

One major difference between the two hemispheres is the direct connection between the agency system of the dominant hemisphere and the encoding system based primarily in the dominant hemisphere that links action production and its interpretation with language. The overarching unitary conscious agent that emerges in the intact brain is based primarily in the dominant hemisphere and is closely connected to the organization of language capacity. It is proposed^{[according to whom?]} that while action precedes linguistic capacity during development, a process ensues through the course of development through which linguistic constructs are linked to action elements in order to produce a language-based encoding of action-oriented knowledge.

When there is a major disconnection between the two hemispheres resulting from callosal injury, the language-linked dominant hemisphere agent which maintains its primary control over the dominant limb loses, to some degree, its direct and linked control over the separate "agent" based in the nondominant hemisphere, and the nondominant limb, which had been previously responsive and "obedient" to the dominant conscious agent. The possibility of purposeful action occurring outside of the realm of influence of the conscious dominant agent can occur and the basic assumption that both hands are controlled through and subject to the dominant agent is proven incorrect. The sense of agency that would normally arise from movement of the nondominant limb now no longer develops, or, at least, is no longer accessible to consciousness. A new explanatory "story" for understanding the nature of the inaccessible agent moving the nondominant limb is necessitated.

Under such circumstances, the two agents that can direct actions in the two limbs that are directed at opposing purposes although the dominant hand remains linked to the dominant consciously accessible agent and is viewed as continuing to be under "conscious control" and obedient to conscious will, while the nondominant hand is no longer "tied in" to the dominant agent and is thus identified by the conscious language-based dominant agent as having a separate and alien agency. This theory would explain the emergence of alien behavior in the nondominant limb and intermanual conflict between the two limbs in the presence of damage to the corpus callosum.

The distinct anteromedial, frontal, and posterolateral temporo-parieto-occipital forms of the alien hand syndrome would be explained by selective injury to either the frontal or the posterior agency systems within a particular hemisphere, with the alien behavior developing in the limb contralateral to the damaged hemisphere.

Treatment

Although there is no known formal (primary) treatment for alien hand syndrome at this time, the symptoms can be reduced and managed to some degree by keeping the alien hand occupied and involved in a task, for example by giving it an object to hold in its grasp. Specific learned tasks can restore voluntary control of the hand to a significant degree. One patient with the "frontal" form of alien hand who would reach out to grasp onto different objects (e.g., door handles) as he was walking was given a cane to hold in the alien hand while walking, even though he really did not need a cane for its usual purpose of assisting with balance and facilitating ambulation. With the cane firmly in the grasp of the alien hand, it would generally not release the grasp and drop the cane in order to reach out to grasp onto a different object. Different strategies can be employed to reduce the interference of the alien hand behavior on the ongoing coherent controlled bodily actions of the patient.

In the presence of unilateral damage to a single cerebral hemisphere, there is generally a gradual reduction in the frequency of alien behaviors observed over time and a gradual restoration of voluntary control over the affected hand. One theory is that neuroplasticity in the bihemispheric and subcortical brain systems involved in voluntary movement production can serve to re-establish the connection between the executive production process and the internal self-generation and registration process. Exactly how this may occur is not well understood, but a process of gradual recovery from alien hand syndrome when the damage involves a single hemisphere has been reported^[who?].

In another approach, the patient is trained to perform a specific task, such as moving the alien hand to contact a specific object or a highly salient environmental target, which is a movement that the patient can learn to generate voluntarily through focused training in order to effectively override the alien behavior. It is possible that some of this training produces a re-organization of premotor systems within the damaged hemisphere, or, alternatively, that ipsilateral control of the limb from the intact hemisphere may be expanded. Another method involves simultaneously "muffling" the action of the alien hand and limiting the sensory feedback coming back to the hand from environmental contact by placing it in a restrictive "cloak" such as a specialized soft foam hand orthosis or, alternatively, an everyday oven mitt. Of course, this can limit the degree to which the hand can participate in addressing functional goals for the patient. Theoretically, this approach could slow down the process through which voluntary control of the hand is restored if the neuroplasticity that underlies recovery involves the exercise of voluntary will to control the actions of the hand in a functional context.

In popular culture

• In Stanley Kubrick's 1964 film, Dr. Strangelove, the eponymous character played by Peter Sellers is shown to be suffering from alien hand syndrome.
• The film Idle Hands is very loosely based on the conditions associated with alien hand syndrome.
• In Tales of Monkey Island, Guybrush Threepwood's poxed hand also has a mind of its own, providing a comical effect.
• In the House episode "Both Sides Now", the patient suffers from alien hand syndrome.
• In The Evil Dead the lead character Ash's hand becomes possessed.

Notes

1. ^ "The Mind's Strange Syndroms". BBC News. September 8, 2000. http://news.bbc.co.uk/2/hi/uk_news/916005.stm.  "Even today, "anarchic hand" has earned the popular sobriquet Dr Strangelove syndrome - named after the eponymous scientist with the unruly limb in Stanley Kubrick's 1964 film."
2. ^ Kayser, A. S.; Sun, F. T.; D'esposito, M. (2009). "A comparison of Granger causality and coherency in fMRI-based analysis of the motor system". Human Brain Mapping 30 (11): 3475. doi:10.1002/hbm.20771. PMC 2767459. PMID 19387980. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2767459.  edit
3. ^ Assal, F. �D. �R.; Schwartz, S.; Vuilleumier, P. (2007). "Moving with or without will: functional neural correlates of alien hand syndrome". Annals of Neurology 62 (3): 301. doi:10.1002/ana.21173. PMID 17638304.  edit
4. ^ Spengler, S.; Von Cramon, D. Y.; Brass, M. (2009). "Control of shared representations relies on key processes involved in mental state attribution". Human Brain Mapping 30 (11): 3704. doi:10.1002/hbm.20800. PMID 19517530.  edit

Selected references from medical literature

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Works cited

• Bellows, Allen (November 19, 2005). "Alien Hand Syndrome". Damn Interesting. http://www.damninteresting.com/?p=203. Retrieved October 6, 2011. 
• Bryant, Charles W. (September 12, 2007). "How Alien Hand Syndrome Works". HowStuffWorks. http://science.howstuffworks.com/science-vs-myth/unexplained-phenomena/alien-hand.htm. Retrieved October 6, 2011. 
• "Defininition of Alien Hand Syndrome". MedicalNet.com. December 15, 2000. http://www.medterms.com/script/main/art.asp?articlekey=12655. Retrieved October 6, 2011. 

External links

• Recent review article on Alien Hand Syndrome by LA Scepkowski & A Cronin-Golomb
• Editorial paper regarding the different forms of Alien Hand Syndrome by G Goldberg
• Recent review article from the Archives of Neurology by I. Biran and A. Chatterjee
• Information about the rare disorder, as well as how many times it has influenced the media.
• BBC Video: Woman with Alien Hand Syndrome