Alien Hand Syndrome: Causes, Diagnosis and Symptoms.

Author.

Hayk S. Arakelyan. Full Professor in Medicine,

Doctor of Medical Sciences, Ph.D , Grand Ph.D?.

Senior Expert of?Interactive?Clinical?Pharmacology?, Drug Safety,?????????????????????????????????????????????????????????????????????Treatment Tactics, General Medicine and Clinical Research.????????????????????????????????????????????????????????????????????????????????President of Rare and Incurable Diseases Association.

Yerevan-Armenia, Tokyo-Japan.

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“Natural forces within us are

the true healers of disease.”

“ Hippocrates”

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Introduction.

Alien hand syndrome is?a phenomenon in which one hand is not under control of the mind. The person loses control of the hand, and it acts as if it has a mind of its own. The etiology includes neurosurgery, tumor, aneurysms, and rarely stroke.

Cause of Alien Hand Syndrome.

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".?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 frontal aspect of the hemisphere (including the?supplementary motor area)?prior?to activation of the primary motor cortex in the pre-central gyrus on the lateral aspect of the hemisphere, when the hand movement is being generated. Activation of the primary motor cortex, presumed to be directly involved in the execution of the action via projections into the corticospinal component of the?pyramidal tracts, is then followed by activation of the?posterior parietal cortex, possibly related to the receipt of recurrent or?re-afferent?somatosensory feedback generated from the periphery by the movement which would normally interact with the?efference copy?transmitted from primary motor cortex to permit the movement to be recognized as self-generated rather than imposed by an external force. That is, the efference copy allows the recurrent afferent somatosensory flow from the periphery associated with the self-generated movement to be recognized as?re-afference?as distinct from?ex-afference. Failure of this mechanism may lead to a failure to distinguish between self-generated and externally generated movement of the limb. This anomalous situation in which re-afference from a self-generated movement is mistakenly registered as ex-afference due to a failure to generate and successfully transmit an efference copy to sensory cortex, could readily lead to the interpretation that what is in actuality a self-generated movement has been produced by an external force as a result of the failure to develop a sense of agency in association with emergence of the self-generated movement (see below for a more detailed discussion). 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 involved anomalous?isolated?activation of the primary motor cortex in the damaged hemisphere contralateral to the alien hand, while non-alien movement involved the normal process of activation described in the preceding paragraph in which primary motor cortex in the intact hemisphere activates in concert with frontal premotor cortex and posterior parietal cortex presumably involved in a normal cortical network generating premotor influences on the primary motor cortex along with immediate post-motor re-afferent activation of the posterior parietal cortex. Combining these two fMRI studies, one could hypothesize that the alien behavior that is unaccompanied by a sense of agency emerges due to autonomous activity in the primary motor cortex acting independently of?premotor cortex?pre-activating influences that would normally be associated with the emergence of a sense of agency linked to the execution of the action. As noted above, these ideas can also be linked to the concept of?efference copy?and?re-afference, where efference copy is a signal postulated to be directed from premotor cortex (activated normally in the process associated with emergence of an internally generated movement) over to somatosensory cortex of the parietal region, in advance of the arrival of the "re-afferent" input generated from the moving limb, that is, the afferent return from the moving limb associated with the self-generated movement produced. It is generally thought that a movement is recognized as internally generated when the efference copy signal effectively "cancels out" the re-afference. The afferent return from the limb is effectively correlated with the efference copy signal so that the re-afference can be recognized as such and distinguished from "ex-afference", which would be afferent return from the limb produced by an externally imposed force. When the efference copy is no longer normally generated, then the afferent return from the limb associated with the self-generated movement is mis-perceived as externally produced "ex-afference" since it is no longer correlated with or canceled out by the efference copy. As a result, the development of the sense that a movement is not internally generated even though it actually is (i.e. the failure of the sense of agency to emerge in conjunction with the movement), could indicate a failure of the generation of the efference copy signal associated with the normal premotor process through which the movement is prepared for execution.Since there is no disturbance of the?sense of ownership?of the limb (a concept discussed in the Wikipedia entry on?sense of agency) in this situation, and there is no clearly apparent physically ostensible explanation for how the owned limb could be moving in a purposive manner without an associated sense of agency, effectively through its own power, a?cognitive dissonance?is created which may be resolved through the assumption that the goal-directed limb movement is being directed by an "alien" unidentifiable external force with the capacity for directing goal-directed actions of one's own limb.

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Diagnosis of Alien Hand Syndrome.

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A doctor may diagnose alien hand syndrome through observation and evaluation. Diagnosing alien hand syndrome is complicated because it’s a neurological disorder that lacks a psychiatric component. This makes it more difficult to diagnose because behavioral issues are more common than alien hand syndrome. Symptoms can sometimes be attributed to a psychiatric disorder, which may be frustrating to the person affected.

Sign and Symptoms of ?Alien Hand Syndrome.

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"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. 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.

A related syndrome described by the French neurologist Fran?ois Lhermitte involves the release through disinhibition of a tendency to compulsively utilize objects that present themselves in the surrounding environment around the patient.?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 referred to as "environmental dependency syndrome".

In order to deal with the alien hand, some patients engage in personification of the affected hand.?Usually these names are negative in nature, from mild such as "cheeky" to malicious "monster from the moon".For example, Doody and Jankovic described a patient who named her alien hand "baby Joseph". When the hand engaged in playful, troublesome activities such as pinching her nipples (akin to biting while nursing), she would experience amusement and would instruct baby Joseph to "stop being naughty".?Furthermore, Bogen suggested that certain personality characteristics, such as a flamboyant personality, contribute to frequent personification of the affected hand.

Neuroimaging and pathological research shows that the?frontal lobe?(in the frontal variant) and?corpus callosum?(in the callosal variant) are the most common anatomical lesions responsible for the alien hand syndrome.?These areas are closely linked in terms of?motor planning?and its final pathways.

The callosal variant includes advanced willed motor acts by the non-dominant hand, where patients frequently exhibit "intermanual conflict" in which one hand acts at cross-purposes with the other "good hand".[15]?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." Another patient was observed to be buttoning up her blouse with her controlled dominant hand while the alien non-dominant hand, at the same time, was unbuttoning her blouse. The frontal variant most often affects the dominant hand, but can affect either hand depending on the lateralization of the damage to medial frontal cortex, and includes grasp reflex, impulsive groping toward objects or/and tonic grasping (i.e. difficulty in releasing grip). In most cases, classic alien-hand signs derive from damage to the medial frontal cortex, accompanying damage to the?corpus callosum.?In these patients the main cause of damage is unilateral or bilateral infarction of cortex in the territory supplied by the?anterior cerebral artery?or associated arteries.?Oxygenated blood is supplied by the anterior cerebral artery to most medial portions of the frontal lobes and to the anterior two-thirds of the corpus callosum,[17]?and infarction may consequently result in damage to multiple adjacent locations in the brain in the supplied territory. As the medial frontal lobe damage is often linked to lesions of the corpus callosum, frontal variant cases may also present with callosal form signs. Cases of damage restricted to the callosum however, tend not to show frontal alien-hand signs.

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If you have any questions?concerning?“Alien Hand Syndrome: Causes, Diagnosis and Symptoms”,?interactive clinical pharmacology?,?or any other?questions, please?inform?me .?

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??????Prof. Hayk S. Arakelyan

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