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org/wiki/Hemispherectomy
Hemispherectomy was first tried on a dog in 1888 by Friedrich Goltz. The first
such operation on humans was done by Walter Dandy in 1923 for glioblastoma
multiforme. In the 1960s and early 1970s, hemispherectomy involved removing
half of the brain, but this resulted in unacceptable complications and side effects
in many cases, predominantly filling of excessive body fluids in the skull with
subsequent pressure to the remaining brain (known as hydrocephalus). The
procedure was revitalized in children in the 1980s by Dr. Ben Carson at The
Johns Hopkins Hospital. In many centers, the functional hemispherectomy
has largely replaced this procedure, in which the temporal lobe is removed; a
procedure known as corpus callosotomy is performed; and the frontal and
occipital lobes are disconnected from the rest of the brain; however the traditional
"anatomic" hemispherectomy has remained a viable procedure, due to its
superiority in preventing future seizures compared with functional
hemispherectomy.
Results
All hemispherectomy patients suffer at least partial hemiplegia on the side of the
body opposite the removed or disabled portion, and may suffer problems with
their vision as well.
Symptoms
An alien hand sufferer 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 hands can perform complex acts such as
undoing buttons, removing clothing, and manipulating tools. 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.
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."
This condition has been thought to provide a fascinating window into the nature
of human consciousness as it relates to voluntary action, processes underlying
decision making and conscious volition, as well as the general nature of human
agency and intentionality. It provides insight into the neural mechanisms involved
in the emergence of a sense of agency association with bodily action. Besides its
relevancy to the understanding of the neurobiologic basis of human action and
volition/intentionality, these observations would appear to have significant
relevance for the general philosophy of action. In that the recognition and study
of this condition depends upon linking an observation of a particular behavior—
the appearance of a purposeful limb action—to either a direct report or inference
regarding the subjective experience of the actor associated with producing the
movement, and then correlating this transformed relation with brain
pathophysiology, alien hand syndrome and its study may be viewed as within the
purview of neurophenomenology. Furthermore, the dissociation of the sense of
agency from the sense of ownership that is observed in alien hand syndrome can
also be seen in other neuropsychiatric conditions, such as schizophrenia, and
may serve to help provide insight into the neural mechanisms underlying these
conditions.
Subtypes
There are several distinct subtypes of alien hand syndrome that appear to be
associated with specific distributions of associated brain injury. 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, whereas
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.
Alternatively, they may attribute the forces initiating and controlling these
purposeful movements to some external being that is somehow "alien" to their
self-perceived persona. For example, a particularly religious woman with alien
hand syndrome, when asked who or what was actually controlling the alien
movements of her hand if she was not, replied that "God must be doing all of
that." Some patients choose to "personify" the hand and dissociate themselves
from the behavior of the hand by giving it a proper name and attributing to it a
separate and distinct personality and personhood. They may choose to "hold
back" these unwanted, bothersome "alien" movements and behaviors by
grasping and constraining the "wayward" hand with the controlled contralateral
hand, an action that has been termed "self-restriction" or "self-grasping." Such
alien movements and behaviors can also be seen in progressive degenerative
diseases that produce a process of steady systematic disintegration of motor
control circuitry within the central nervous system, such as Creutzfeldt-Jakob
Disease, and corticobasal degeneration.
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 very
recent 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" (Kayser et al. 2009). 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 recent fMRI study
conducted by McGill neurologist Elena Dugan 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
(Assal et al. 2007).
One theory posed 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.
The critical difference between the two hemispheres, however, 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. Thus, 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 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. Through this process of
basically "telling oneself a story" about how an act occurs, a language-based
"action-capable" self is constructed through consistent correlations between
intended actions and subsequent perceived outcomes.
Under such circumstances, the two agents therefore 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 thus 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.
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. Also, specific learned tasks can restore voluntary
control of the hand to a significant degree. For example, 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. Thus, 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 popular culture
It is also shown as a vital symptom in 'Both Sides Now', an episode from the
5th season of House MD.
The film Idle Hands is very loosely based on the conditions associated with Alien
Hand Syndrome.
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Isaac’s writing: