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10.1177/1534582303260119
BEHAVIORAL AND COGNITIVE NEUROSCIENCE REVIEWS
Scepkowski, Cronin-Golomb / THE ALIEN HAND

The Alien Hand: Cases, Categorizations,


and Anatomical Correlates
Lisa A. Scepkowski
Alice Cronin-Golomb
Boston University
toms, the patients mental status was normal. Upon the
womans death, Goldstein (1908) examined her brain
and found lesions in the right hemisphere and in the
corpus callosum, the large fiber bundle connecting the
right and left hemispheres.
Years later, Akelaitis (1945) described another patient
whose left hand would involuntarily perform the opposite of what she wanted her right hand to do following
section of the corpus callosum. Akelaitis termed this
phenomenon diagonistic dyspraxia. The term alien hand
(la main trangre) was first applied to this type of behavior by Brion and Jedynak (1972) to describe patients with
midline brain tumors who exhibited a denial of ownership of one of their hands. The hallmark of all these cases
was the individuals perception of the affected hand as
being out of volitional control as it performed simple to
complex motor activities.
Renewed interest in the alien hand has spurred
reports of numerous cases in which affected individuals
described strange manual activities following damage to
certain parts of the brain. In many instances, damage to
the corpus callosum was cited, just as in the original
cases. Efforts in the past decade have focused on creating
a way to classify subtypes of alien-hand phenomena
based on specific behaviors and areas of brain damage
beyond the corpus callosum. The present review of these

The clinical characteristics and neuroanatomical damage


reported in more than 50 published cases of observed alienhand signs are reviewed. The terms alien-hand sign and
alien-hand syndrome describe phenomena experienced by patients in which an upper limb performs complex motor activities
outside of volitional control. The categories of frontal and
callosal subtypes and their relation to behavior and neuropathology are evaluated with reference to the dual premotor system theory, which emphasizes the role of the supplementary motor
areas in alien-hand phenomena. Detailed consideration is given
to the more recently described posterior subtype, which is purported to result from damage to the parietal lobe or other posterior
brain areas. The lack of uniformity in reported assessment methods (behavioral tests, neuroimaging) in published cases contributes to the difficulty in establishing clear subtypes of alien-hand
phenomena. Suggestions are made regarding current categorizations and available assessment methods.
Key Words: alien hand, diagonistic dyspraxia, corpus
callosum, cerebral infarction, motor disturbance

The very name alien hand conjures up scenes from B

movies and Dr. Strangelove, depictions of a terrifying,


uncontrollable limb that is trying with all its might to
strangle or otherwise injure its owner-victim. The idea
that such behavior could occur outside of movies understandably strains credibility in the average person. In the
clinical world, the phenomenon of the alien hand has
been known since 1908, when Kurt Goldstein described
the case of a 57-year-old woman who suffered a stroke
and thereafter perceived her left hand as having a will of
its own. On one occasion, the hand grabbed her by the
throat and choked her, requiring great effort to pull it
off. She described the hand as possessing an evil spirit
and stated that it did not belong to her: Those are two
very different people, the arm and I. With the exception
of the alien hand and other discrete stroke-related symp-

Authors Note: We would like to thank Lissa Davis for artwork (Figures
2 and 3), Bonnie Wong and Ari Juels for translation services, Helen
Tretiak-Carmichael and Tom Laudate for expert technical assistance,
and Sandra Witelson for permitting us to adapt her figure of the corpus
callosum (Figure 1). Correspondence concerning this article should
be addressed to Alice Cronin-Golomb, Department of Psychology,
Boston University, 648 Beacon Street, 2nd floor, Boston, MA 02215; email: alicecg@bu.edu.
Behavioral and Cognitive Neuroscience Reviews
Volume 2 Number 4, December 2003 261-277
DOI: 10.1177/1534582303260119
2003 Sage Publications

261

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BEHAVIORAL AND COGNITIVE NEUROSCIENCE REVIEWS

efforts describes current thinking in the field about the


nature of the dysfunction and its underlying brain
substrates.
The great majority of reports of alien-hand phenomena appear in the neurological literature, and its conventions are followed here. Hence, a sign is a behavior
observed by the neurologist, neuropsychologist, or
other investigator, whereas a symptom is a behavior
reported by the affected individuals, who are referred to
as patients because the etiology of the phenomena is usually stroke or surgical intervention in brain dysfunction.
A number of alien-hand signs have been observed in
individual patients, although as often happens in neurology, psychology, and psychiatry, there is debate about
which signs are necessary and sufficient to constitute a
syndrome.
As used by most investigators, the alien hand refers to
the general behavior of an upper limb in performing
autonomous complex movements against the patients
will (e.g., Fisher, 2000; Trojano, Crisci, Lanzillo,
Elefante, & Caruso, 1993). Specific alien-hand signs, not
seen in all patients, include (a) intermanual conflict, in
which one hand acts at cross-purposes with the other
(Bogen, 1979); (b) mirror movements and writing, in
which one hand automatically mimics the movements of
the other hand; (c) enabling synkinesis, in which one
hand can perform an action only in unison with the
other; (d) grasp reflex; (e) impulsive groping toward
objects; or, in Denny-Browns (1958) term, magnetic
apraxia, in which the affected hand reaches toward and
grasps objects as if drawn to them by a magnet, and
release of the objects is difficult; and (f) utilization
behavior, or compulsive manipulation of tools.
What is common to all cases is a perception of the
limb as foreign and its movements as beyond the
patients control. Many patients go so far as to deny ownership, and some engage in personification of the limb.
The names given by the patients to their alien hands usually reveal negative affect, from mild (cheeky) to more
sinister (monster, from the moon, the devils).
Aspects of the individuals premorbid personality may
affect the manifestation of the alien-hand signs, with
Bogen (1979) and Levine and Rinn (1986) suggesting
that a flamboyant personality may contribute to frequent
personification of the alien hand. Doody and Jankovic
(1992) provided a remarkably elaborate example of personification in which the patient referred to her arm as
a baby called Joseph whose actions, such as pinching
her nipples, were seen as mischievous behaviors, akin to
biting while nursing. These authors cautioned that
patients using such personifications might be mistaken
for psychotic. Della Sala, Marchetti, and Spinnler (1991)
suggested that in cases in which there is no denial of ownership but instead a perception that the arm is com-

Figure 1: Diagram of the Midsagittal View of the Corpus Callosum of


the Human Adult.
SOURCE: Adapted with permission from Witelson (1989).
NOTE: ACC-PCC was used as the linear axis to subdivide the callosum
into anterior and posterior halves; anterior, middle, and posterior
thirds; and the posterior one-fifth region (region 7), which is roughly
congruent with the splenium. A rough topography of callosal fibers in
relation to cortical regions of origin and termination is given, based
mainly on experimental work with monkeys and on some clinical work
with humans.

pletely autonomous and disruptive, the term anarchic


hand may be more appropriate than alien hand.
Although recognizing the point, we keep to the latter,
ubiquitously used term in this review.
In an influential paper, Feinberg, Schindler,
Flanagan, and Haber (1992) reviewed the alien-hand literature and proposed that there are two main subtypes,
callosal and frontal. The anatomical substrate for the former is the corpus callosum. Damage results either from
surgical section of part or all of the anterior callosum
(the genu and body, sparing the back part, or splenium;
see Figure 1), usually as treatment for epileptic seizures
that do not respond to medication, or from localized
damage caused by ischemia (blockage) or rupture of the
anterior cerebral artery or associated arteries (see Figure 2). The behaviors resulting from callosal damage
include disruption of complex willed motor acts by the
left hand (the hand termed nondominant for writing and
other daily functions in most patients reviewed here) as
well as classic hemispheric disconnection signs of the
type described by psychologist Roger Sperry and his colleagues in his Nobel Prizewinning work on hemispheric lateralization (Sperry, Gazzaniga, & Bogen,
1969). It should be noted that in all reviewed reports in
the literature, the patients were right-handed or ambidextrous, with no left-handers represented. Theories

Scepkowski, Cronin-Golomb / THE ALIEN HAND

263

between alien-hand signs and their neuropathological


bases have been complicated by recent descriptions of
cases of alien hand following damage to posterior brain
areas, in the absence of damage to the corpus callosum
or frontal areas. These cases will be considered later in
this review.
CASES OF DAMAGE TO
THE CORPUS CALLOSUM

Figure 2: Anterior Cerebral Artery, Including the Pericallosal Artery.


NOTE: Right hemisphere, midsagittal view. Alien-hand phenomena
may arise from lesions to several depicted brain areas including the
supplementary motor area, the anterior cingulate cortex, and the corpus callosum.

about the behavioral and neuropathological concomitants of dominant versus nondominant alien hands are
incomplete without acknowledgment of this fact. It is an
intriguing possibility that being left-handed, with the
accompanying differences in brain organization relative
to most right-handers, precludes or mitigates the development of alien-hand signs.
The anatomical substrate for the second subtype of
alien hand proposed by Feinberg et al. (1992), the frontal type, is mostly medial frontal cortex, including premotor and supplementary motor areas and the anterior
cingulate gyrus. The behaviors resulting from frontallobe damage include grasp reflex, impulsive groping,
and compulsive tool manipulation by the right hand,
termed the dominant hand (for writing and most other
daily activities). The authors suggested that because
medial frontal-lobe damage often is accompanied by
damage to the corpus callosum, frontal-type cases may
also show callosal-type alien-hand signs. Cases of damage
to only the callosum would not show frontal alien-hand
signs.
We discuss below the viability of the dual-subtype categorization of Feinberg et al. (1992) by reference to representative cases, mainly drawn from the literature of the
past decade since their review. More exhaustive reviews
of alien-hand cases with callosal or frontal injury have
been provided by other investigators (e.g., Doody &
Jankovic, 1992, Feinberg et al., 1992; Gasquoine, 1993).
At the same time that we evaluate the dual-subtype proposal, we note that efforts to understand the relation

Akelaitiss (1945) cases of diagonistic dyspraxia


appeared after his patients underwent section of the corpus callosum to treat intractable epileptic seizures. In
one case, the left alien hand would frequently interfere
with the activity of the womans right hand. For example,
she would pull clothing on with her right hand, only to
find her left hand pulling her clothing off. In any
intended bimanual activities, her left hand would consistently perform an action opposite to the right hand and
opposite to the action that she desired to perform. This
intermanual conflict reportedly began more than a
month after her callosotomy and lasted about 3 weeks.
It has been suggested by Tanaka, Yoshida, Kawahata,
Hashimoto, and Obayashi (1996) that a number of case
reports erroneously describe nonpurposive movements
or interference by the affected hand as intermanual conflict or diagonistic dyspraxia. These authors proposed
that the term diagonistic dyspraxia should be reserved for
motor behaviors that cannot be better accounted for by
impulsive groping or grasping movements. Using this
definition in cases reviewed here, intermanual conflict
will be mentioned only if there was sufficient information provided by the authors to confirm alien-hand signs
in which one hand is acting at cross-purposes with the
other, not simply interfering with the other hands
actions.
Fisher (2000) described a patient with a ruptured
aneurysm near the anterior cerebral artery. Whatever
activity she performed with her right hand would be
mimicked by her left hand, a behavior the patient characterized as mirror movements. She complained that
her left hand butted in and wanted to take over anything she tried to do with her right hand. For example,
when she would reach for a glass of water with her right
hand, her left hand would automatically come in and
take hold of the glass. Fisher colorfully referred to such
behavior as the buttinski phenomenon. The term can
be well appreciated by investigators conducting
lateralization research with split-brain patients with section of the corpus callosum and other forebrain
commissures, for whom it is standard practice to test the
nondominant left hand early in the test session before
the dominant right hand is exposed to the same task and

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BEHAVIORAL AND COGNITIVE NEUROSCIENCE REVIEWS

hence given the opportunity to butt in as the left hand


attempts to work (Cronin-Golomb, 1986).
Brion and Jedynak (1972), who coined the term alien
hand, were the first to describe their newly recognized
syndrome as part of callosal disconnection. They
reported findings with four patients, of whom one had
infarction probably of vascular origin and three had
callosal tumors (one affecting the entire structure and
two affecting posterior parts; no further detail given). As
a group, they showed numerous classic signs of
interhemispheric disconnection such as ideomotor
dyspraxia (impaired ability to enact a motor command
with the affected hand), tactile anomia (inability to
name an object palpated by the affected hand), agraphia
(inability to write with that hand), and impaired ability
to imitate the gestures of one hand with the other except
under visual guidance. The alien-hand signs included
inability to recognize one hand as the patients own
when grasped by his other hand, both out of view behind
his back (Case 1); denial of hand ownership (Case 2);
after writing with the left hand and then viewing the
result, denial of having written it and belief that it came
from outside of him (Case 3); and that what was written
did not reflect what the patient told the hand to write
(Case 4). Similar signs had been described by Goldstein
(1908) in his original case.
More recently, Geschwind et al. (1995) described the
case of a woman who, roughly 1 week after a coronary
artery bypass grafting, reported a loss of control over her
left hand, which seemed to her to be acting on its own.
She awoke several times with her left hand choking her.
The hand would unbutton her gown and fight with the
right hand to answer the phone. She had to physically
subdue her left hand with her right (self-restriction, a
common tactic to prevent involuntary movements). The
left hand also displayed significant ideomotor apraxia.
Although she could mimic actions with the left hand,
this could be done only along with mirror movements of
the right (enabling synkinesis). On examination 8 hr
later, all unusual manual activity of the left hand had disappeared with the exception of remaining ideomotor
apraxia. Magnetic resonance imaging (MRI) scan
showed an area of infarction involving the posterior half
of the callosal body, sparing the anterior half and the
splenium (the most posterior part of the corpus callosum), with slight extension into the white matter underlying the right cingulate cortex (see Figure 1 for an illustration of basic callosal anatomy). The alien-hand signs
in this case were transient, as in Akelaitiss (1945) case.
Giroud and Dumas (1995) reported two more cases of
callosal infarction (one with genu and body plus frontal
pathology, the other much of the entire corpus callosum), most with alien-hand signs similar to those

described above. The patients presented by Nagumo


a n d Ya m a d o r i (1 9 9 5 ) a n d S u w a n w e l a a n d
Leelacheavasit (2002) likewise had callosal infarction
and alien-hand signs typical of the disconnection
syndrome.
Most callosal alien-hand cases report the nondominant hand as the alien hand. As known from lesion studies and imaging studies, in right-handed individuals, the
left hemisphere is dominant for complex or fine motor
activities (reviewed in Geschwind et al., 1995). A disconnection between the left and right hemispheres in these
individuals caused by damage to the corpus callosum
results in the left hands being controlled only by the
right hemisphere, without the direction of the motordominant left hemisphere.
CASES OF DAMAGE TO THE
MEDIAL FRONTAL LOBES
Most cases of alien-hand signs are seen in patients
who have suffered damage to the medial frontal cortex
with accompanying damage to the corpus callosum. The
most common cause of this damage is unilateral or bilateral infarction of the anterior cerebral artery or associated arteries. The anterior cerebral artery supplies
blood to medial frontal areas and to the anterior two
thirds of the corpus callosum (Gasquoine, 1993; Giroud
& Dumas, 1995), and infarction may therefore result in
damage to multiple areas (see Figure 2). As mentioned
above, frontal-type cases of alien hand are characterized
by additional behaviors not typically seen in callosal
alien-hand cases.
Banks et al. (1989) reported two cases of right-handed
individuals with injuries to medial-frontal and callosal
brain areas. One case was a woman who suffered a gunshot wound to the head that caused bilateral rupture of
the anterior cerebral arteries. Within a few months of
surgical clipping of the arteries, the patient noticed that
her left arm began to move on its own. Her left hand
would grope for and grab any object nearby, pull at her
clothes, and grasp her throat while she slept, prompting
her to sleep with her arm tied. She denied that the limb
was hers. Damage to bilateral medial frontal areas and
the anterior portion of the corpus callosum were confirmed by a computerized tomography (CT) scan.
Neuropathological examination upon her death 13
years after the injury revealed general callosal
demyelination (loss of the fatty sheath that speeds information relay along axons) and infarction of the genu
and splenium of the corpus callosum.
The second case was a right-handed man who suffered a large aneurysm (ballooning and consequent
weakening) of the anterior communicating artery at the

Scepkowski, Cronin-Golomb / THE ALIEN HAND


junction of the right anterior cerebral artery. During the
month after surgery to clip the aneurysm, the patient
began complaining of difficulty in getting his left arm
and hand to obey him during tasks requiring bimanual
cooperation. He reported that his left hand would frequently grasp and manipulate objects while he was
attempting to perform a task with his right hand. Other
difficulties fell in the category of intermanual conflict.
On one occasion, the left hand repeatedly made moves
in a game of checkers that the patient would have to correct with the right hand. At other times, one hand turned
the pages of a book while the other tried to close it. A CT
scan performed at 4 months showed bilateral medialfrontal cortical damage as well as damage to the genu
and anterior portion of the body of the corpus callosum.
Goldberg and Bloom (1990) described four cases of
frontal-lobe and callosal damage with resulting extravolitional hand movements. Their first case was a righthanded woman whose MRI scan showed abnormalities
of the right medial frontal lobe and the body of the corpus callosum. She displayed a grasp reflex in her left
hand and an inability to release objects once grasped.
She also showed compulsive tool manipulation in the
left hand. Her left hand would wander involuntarily if
left unrestrained. She demonstrated frequent intermanual conflict. For example, when she would try to
light a cigarette with her right hand, her left hand would
remove the cigarette from her mouth. One year later,
her alien-hand behaviors had become very infrequent.
The second case was a right-handed woman with a
large left anterior cerebral infarction of the medial surface of the left frontal lobe. Interestingly, no callosal
damage was reported, nor were there any signs of
callosal disconnection. This patient displayed a grasp
reflex in her right hand with an inability to release
objects. Her right hand was noted to reach out for
nearby objects and compulsively grasp objects and even
her own body parts. At times, her right hand injured her
leg by excoriating the skin with perseverative grasping
movements, to the extent that an orthotic device was
required to restrict this harmful action. The hand was
highly reactive to verbal suggestion and would perform
acts mentioned in casual conversation.
Goldberg and Blooms (1990) third case was a righthanded man with symptoms of right-hemisphere stroke
whose MRI scan revealed abnormalities of the medial
surface of the right frontal lobe extending into the corpus callosum (specific area of the corpus callosum not
reported). Once he regained strength in his left hand, it
was noted to frequently reach out and grasp objects without his volition. By contrast, voluntary attempts to move
the hand were relatively slow and uncoordinated. In one
telling episode, the man burned his left palm by crush-

265

ing a blazing napkin, reporting that he had tried to


refrain from this behavior but the alien drive was overpowering. The patient eventually developed the ability
to suppress some of the alien movements by issuing verbal commands to the hand. Despite this growing ability,
the hand would still grasp objects and not be able to
release them. Over a period of 7 months, all
extravolitional manual activity slowly diminished and
then disappeared. Recently, Kumral (2001) reported a
similar case of compulsive grasping in the absence of
groping, mirror movements, or utilization behavior in a
patient with hemorrhage of the medial frontal areas and
the genu, body, and splenium of the corpus callosum.
The fourth case described by Goldberg and Bloom
(1990) was a right-handed woman with infarcts of the
corpus callosum (site not specified) and the medial posterior surface of the left frontal lobe, with symptoms consistent with stroke. She had severely impaired bimanual
coordination and a grasp reflex in her right hand. The
right hand would reach out and grasp objects and would
be unable to release them and would consistently interfere with the actions of the left hand. The patient indicated that she did not feel that she was moving the
affected hand. She had a tendency to perform bimanual
tasks symmetrically. Movement of the right alien hand
was highly responsive to verbal suggestion. These extravolitional manual activities diminished over time.
Chan, Chen, and Ng (1996) described the case of a
right-handed man who presented with sudden bilateral
leg weakness, indicative of medial frontal disturbance.
After 2 weeks, his hypokinesia (reduced movement)
began to resolve, and abnormal left-arm movements
became noticeable, including intermanual conflict and
spontaneous unwilled goal-directed movements such as
turning the pages of his newspaper before he intended
to. He exhibited a strong grasp reflex in his left hand as
well as spontaneous reaching to grope nearby objects.
MRI scan revealed an area of infarction in the territory of
the right anterior cerebral artery including the right corpus callosum from the genu to the isthmus (narrowest
part of the body), the right supplementary motor area,
anterior cingulate gyrus, and medial prefrontal cortex.
The alien-hand signs in this patient markedly diminished after 6 months.
McNabb, Carroll, and Mastaglia (1988) discussed
three patients with alien-hand signs and infarction of the
territory of the anterior cerebral artery. In one of the
patients, there was CT confirmation of lesion to the left
superior and medial frontal-parietal cortices and the
genu and body of the corpus callosum. This patient had
right-handed impulsive groping and grasp reflex, to
which actions she would express astonishment. She
attempted to control the right hands interference by

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BEHAVIORAL AND COGNITIVE NEUROSCIENCE REVIEWS

wedging it between her legs or holding or slapping it


with the left hand. Motor potential recordings in this
patient suggested abnormalities of the supplementary
motor area and possible loss of the normal asymmetry of
activation of motor centers in the two hemispheres.
Kischka, Ettlin, Lichtenstern, and Riedo (1996)
described the case of a right-handed patient with left
anterior cerebral artery infarction and consequent right
alien hand. The infarct included the pericallosal artery
and its territory of the anterior corpus callosum, sparing
the splenium. This man exhibited grasp reflex, public
masturbation, significant utilization behavior, and
intermanual conflict. A memorable example provided
by the authors was of the patients inability to stop hammering a nail with his alien right hand. The left hand
pushed the board with the nail away but the right hand
kept pursuing it until the patient was leaning forward over the table. Weeping, he told his hand, Please
stop it.
Somewhat similar symptoms were exhibited in a case
of hemorrhage spanning the entire corpus callosum,
whose CT scan showed no evidence of extracallosal
involvement. This right-handed woman experienced
lower limb hemiparesis, right-hand grasp reflex, and
compulsive manipulation of tools, suggesting transient
frontal involvement (Lavados et al., 2002). Her presentation was unique in that either hand was apraxic when
instructed to perform an action, whereas the other,
uninstructed hand compulsively performed the action
more rapidly and accurately. The authors termed this
phenomenon agonistic dyspraxia. The case was also
remarkable in that 7 months after her callosal hemorrhage, during which time her left hand became oppositional and felt foreign, she also occasionally experienced
having two left hands, with one feeling smaller than and
external to the other.
It is noteworthy that despite the sometimes horrific,
distressing behavior of the alien hand reported in these
cases, clinical interventions are rarely mentioned. There
is occasional reference to verbal cajoling and personification and restraint of the alien hand with the normal
hand. Individual cases described above report sleeping
with the arm tied, use of an orthotic device to restrict
perseverative grasping, wedging the hand between the
legs, or slapping it. One rather ingenious and noninvasive intervention was the nighttime use of a common
oven mitt to prevent unwanted grasping and groping
(Nicholas, Wichner, Gorelick, & Ramsey, 1998).
The cases described above capture the main symptoms, signs, and neuropathological correlates of alienhand phenomena arising from medial frontal injury.
Additional cases, each of interest for the variations in
behavioral manifestation and neuropathology, are
noted in Table 1.

FRONTAL VERSUS CALLOSAL SUBTYPES?


We have described above a number of cases illustrating alien-hand signs in patients with lesions of the corpus
callosum, frontal lobes, or both. Attempts to define subtypes of the syndrome have been few. Although callosal
and frontal substrates of alien-hand signs were noted in
the past (e.g., Banks et al., 1989; Bogen, 1979; Brion &
Jedynak, 1972; Goldberg & Bloom, 1990), Feinberg et al.
(1992) suggested that there are two subtypes distinguished not only by site of damage but also by patterns of
behavior. Their subtype descriptions arose from their
review of 20 published cases, which they divided into
four groups: (a) unilateral left-hemisphere lesions, (b)
unilateral right-hemisphere lesions, (c) bilateral hemisphere lesions, and (d) lesions confined to the corpus
callosum. The cases were evaluated in terms of the
patients handedness, the hand afflicted, and presence
or absence of grasp reflex, impulsive groping, compulsive tool manipulation, and intermanual conflict.
According to these investigators, all unilateral lefthemisphere lesions were frontal and involved the medial
premotor region including supplementary motor area,
anterior cingulate, medial prefrontal cortex, and the
anterior corpus callosum. These patients exhibited
alien-hand signs of the dominant (right) hand and typically demonstrated reflexive grasping, impulsive groping, and compulsive tool manipulation. By contrast,
patients with lesions confined to the corpus callosum
had alien-hand signs in the nondominant (left) hand
and exhibited mostly intermanual conflict. Patients with
unilateral right-hemisphere damage also had callosal
lesions, but according to the authors, they tended to be
more posterior than those of patients with unilateral lefthemisphere damage. This latter conclusion was based
on only two cases of unilateral right hemisphere damage, one with damage to the genu and body of the corpus
callosum and one with damage to the splenium. Clinical
manifestation of alien-hand signs in the unilateral right
cases resembled those of patients with callosal-only
lesions. Patients with bilateral hemisphere damage had
lesions of medial frontal areas, anterior cingulate, and
anterior corpus callosum (genu and body). Alien-hand
signs in this group most closely resembled those of
patients with callosal-only lesions and generally did not
include signs associated with left unilateral frontal damage. The clinical similarity between the right hemisphere
and bilateral groups suggests that the right-hemisphere
damage in both groups accounted for the observed
signs; it is not clear why the bilateral cases should not
rather have resembled those with unilateral left-hemisphere damage.
Feinberg et al. (1992) concluded from their review
that there are two main subtypes of alien-hand syn-

Scepkowski, Cronin-Golomb / THE ALIEN HAND

267

TABLE 1: Cases of Alien Hand Signs Observed Following Frontal or Callosal Damage
Reference

Case

Area of Damage

Hand

DD/IC

Akelaitis (1945)

1
2

BCC, GCC
Complete section of CC

L
R

X
X

Goldberg, Mayer, and Toglia (1981)

1
2

L medial FC
L medial FC

R
R

L superior and medial FC and parietal

McNabb, Carroll, and Mastaglia (1988)


Banks et al. (1989)

1
2

Anterior CC, bilateral medial FC and subcortical


GCC, bilateral medial FC, R gyrus rectus

L
L

Goldberg and Bloom (1990)

1
2
3
4

BCC, R medial FC, SMA, ACG


L medial FC, SMA, ACG
CC, R medial FC, SMA, ACG
CC, L medial posterior FC, SMA, ACG

L
R
L
R

CC, L medial FC, CG

Feinberg, Schindler,
Flanagan, and Haber (1992)

CC, L medial FC

1
2

Possible CC, R frontoparietal cortex


BCC, bilateral medial FC

L
L

1
2

L medial FC
R medial FC

R
L

Trojano, Crisci, Lanzillo,


Elefante, and Caruso (1993)

Ant CC, R medial FC, SMA, ACG

Geschwind et al. (1995)

BCC, R cingulate cortex

CC, frontoparietal ischemia


R anterior CC, R FC

L
L

Nagumo and Yamadori (1995)

L BCC, GCC

Papagno and Marsile (1995)

Anterior CC, R medial FC

Chan, Chen, and Ng (1996)

BCC, GCC, ICC, R SMA, ACG, bilateral medial PFC

Kischka, Ettlin, Lichtenstern,


and Riedo (1996)

Anterior CC, L medial FC, parieto-occipital, SMA

1
2
3
4
5
7

Post BCC, anterior SCC, L CG, temporoparietal


BCC, R CG, L ACG, bilateral medial FC
BCC, GCC, L ACG
Anterior BCC, L medial FC, ACG
Anterior BCC, L medial FC
Anterior BCC, R medial FC, ACG

L
L
L
R
R
L

X
X
X

1
2
3

GCC, ICC, L medial FC, SMA, ACG


BCC, GCC, R medial FC, SMA, ACG
GCC-ICC, R PFC, SMA, ACG

R
L
L

X(L)
?
X

R frontotemporal cortex

Feinberg, Roane, and Cohen (1998)


Nicholas, Wichner, Gorelick,
and Ramsey (1998)

CC

Tow and Chua (1998)

L medial FC and posterior parietal cortex

Bakchine, Slachevsky, Tourbah,


Serres, and Abdelmounni (1999)

BCC, SCC, L internal capsule

L, R

Hanakita and Nishi (1991)

Chan and Ross (1997)

Tanaka, Yoshida, Kawahata,


Hashimoto, and Obayashi (1996)

R
R

7
8

BCC, GCC, bilateral anterior medial FC, SMA


Anterior CC, bilateral FC, L medial FC, SMA

Giroud and Dumas (1995)

X
X
X
X

Della Sala, Marchetti, and Spinnler (1991)

Leiguarda, Starkstein, Nogues,


Bertheir, and Arbelaiz (1993)

X
X

Kuhn, Shekar, Schuster,


Buckler, and Couch (1990)

Gottlieb, Robb, and Day (1992)

GR Groping CTM

X
X
X

X
X

X
X

X
X
?

X
X
X
X
X

X
X
X
X

X
X
X

X
X
X

X
X

X
X

X
(continued)

268

BEHAVIORAL AND COGNITIVE NEUROSCIENCE REVIEWS

TABLE 1 (continued)
Reference

Case

Area of Damage

Fisher (2000)

Hand

DD/IC

GR Groping CTM

Ong Hai and Odderson (2000)

Anterior CC, R medial FC

Kumral (2001)

BCC, GCC, SCC, R medial FC?

BCC, GCC, SCC


Anterior BCC, SCC, L parietal cortex

L
R

Nishikawa et al. (2001)

1
3

Lavados et al. (2002)


Suwanwela and Leelacheavasit (2002)

CC, F medial (inferred)


1
2

BCC, SCC
BCC

L
X
(alien) (AgD)
L
L

X
(R)

X
(R)

X
(R)

X
X

NOTE: ACG = anterior cingulate gyrus; AgD = agonistic dyspraxia; BCC = body of corpus callosum; CC = corpus callosum; CG = cingulate gyrus;
CTM = compulsive tool manipulation; DD/IC = diagonistic dyspraxia or intermanual conflict; FC = frontal cortex; GCC = genu of corpus callosum;
GR = grasp reflex; ICC = isthmus of corpus callosum; SCC = splenium of corpus callosum; L = left; PFC = prefrontal cortex; R = right; SMA = supplementary motor area; X = sign present; ? = unclear. Focus is on cases published since the review of Feinberg et al. (1992) and representative earlier
cases.

dromes: a callosal type and a frontal type. The callosal


type affects the nondominant hand and results from
callosal damage with or without bilateral or right frontal
lesions. These patients rarely exhibit a grasp reflex or
compulsive tool manipulation but frequently exhibit
intermanual conflict. Movements of the alien hand are
elicited by actions of the dominant (nonalien) hand.
The frontal type, in contrast, affects the dominant hand
and results from left medial frontal and callosal damage.
These patients commonly exhibit a grasp reflex and
compulsive tool manipulation and rarely display
intermanual conflict. Alien movements are activated by
visual or tactile stimulation.
Table 1 summarizes many of the cases described thus
far, using the categories of Feinberg et al. (1992) in
regard to the site of anatomical damage and specific
alien-hand signs present. The focus is on cases reported
since the review of Feinberg et al. with reference as well
to some representative earlier cases. The cases do not
sort themselves neatly into the proposed subtypes. The
types of behaviors exhibited by individuals with different
areas of brain damage overlap considerably. Behavioral
distinctions associated with right versus left versus
bilateral damage are not easily made.
Two conclusions can be drawn legitimately from these
published cases. First, patients with damage only to the
corpus callosum almost exclusively show intermanual
conflict as the primary alien-hand sign, in accord with
the summary of Feinberg et al. (1992). In addition,
intermanual conflict, as defined as one hand acting at
cross-purposes to the other, is primarily seen in the
nondominant hand. The suggestion by Feinberg et al.
that intermanual conflict is rarely seen in cases with lefthemisphere frontal damage is challenged by its presence
in a number of cases reviewed here: Chan and Ross

(1997), Case 1; Goldberg, Mayer, and Toglia (1981),


Case 1; and Hanakita and Nishi (1991). In a related case
described by Nicholas et al. (1998), a patient exhibited
crawling and groping signs in his left hand following a
subacute right anterior cerebral artery infarction involving the corpus callosum. There was no intermanual conflict and no report of lesioning of cortical areas surrounding the artery. One may infer from his left
hemiparesis, however, that there was involvement of surrounding medial cortex. In contrast to Feinberg et al.s
(1992) proposal that tool manipulation arises from lefthemisphere dysfunction, compulsive tool manipulation
is seen in cases in which the right or left hemisphere is
involved. Tanaka et al. (1996) have suggested that compulsive tool use is associated with lesions to the anterior
sections of the body of the corpus callosum, whereas
diagonistic dyspraxia (including intermanual conflict)
arises from lesions of the posterior body of the corpus
callosum.
Although Feinberg et al. (1992) stated that unilateral
right-frontal cases tended to have lesions of the corpus
callosum that were more posterior than those seen in unilateral left-frontal cases, there were only two unilateralright cases, and one of them had damage to anterior sections of the corpus callosum. In the absence of more
detail of the anatomical lesions in each case, it is difficult
to decide whether this patients callosal lesion was in fact
posterior to those seen in the left-frontal cases. The suggestion that bilateral frontal cases resemble callosal-only
cases in terms of behavior, including lack of a grasp
reflex and other frontal-type signs, is called to account
by the bilateral frontal cases described by Chan et al.
(1996) and Doody and Jankovic (1992) who showed various frontal-type signs, such as grasp reflex, impulsive
groping, and utilization behaviors.

Scepkowski, Cronin-Golomb / THE ALIEN HAND


An important point was made by Chan and Ross
(1997) about frontal-type alien hand. Specifically, this
behavioral subtype has been associated with the dominant (right) hand, not necessarily because the nondominant (left) hand is uninvolved in the syndrome but
rather because right-hemisphere lesions lead to motor
neglect in the left hand. Neglect, a well-studied consequence of right-hemisphere damage, would suppress or
mask any tendency of the left hand to reach or grasp,
resulting in a very different presentation of alien-hand
signs than those seen in right-hand cases. Investigators of
alien hand could erroneously conclude that only lesions
of the left hemisphere result in the impulses to reach,
grasp, and manipulate objects. Feinberg et al. (1992)
were aware of this possibility, stating that left limb akinesia (lack of movement) may have prevented the
appearance of alien-hand signs of the left frontal type
after right frontal lesions.
The subtypes proposed by Feinberg et al. (1992) capture some aspects of alien-hand phenomena. Specifically, they are useful in describing the callosal-only subtype. They are less successful in relating individual signs
to anatomical substrates when frontal lesions are implicated. In fairness to Feinberg et al., numerous difficulties become apparent in the quest to identify subtypes of
alien-hand phenomena through case review. The number of published cases on which to base generalizations
is small. More critically, the definitions of the various
reported behaviors, or even what behaviors constitute
alien-hand phenomena, are not completely consistent
from study to study. Because there is no standard procedure for evaluating patients with extravolitional arm
movements, it is probable that certain behaviors are
overlooked if they do not appear during clinical evaluation. A good example is the rarity of mirror movements
and enabling synkinesis in published cases, both of
which are elicited only by specific assessments and may
not be noticed or reported by the patient. In addition,
very few cases include the results of detailed neuropsychological assessment, which can aid in pinpointing
brain areas subject to dysfunction even in the absence of
a clear picture of anatomical change on CT or MRI scan,
as often occurs with lesions of large territories as in anterior cerebral artery stroke. Adding to the difficulty in elucidating anatomical-behavioral correlations are more
recently published cases of alien hand that result from
damage to more posterior cortical areas, as we describe
in a subsequent section of this review.
Frontal and Callosal Pathology:
The Dual Premotor System Theory
Alien-hand signs occurring in patients with callosal
and medial frontal damage have been explained largely
by the dual premotor system theory (Goldberg & Bloom,

269

1990). This theory posits that the alien-hand signs are


caused by damage to the supplementary motor area
(SMA) and the disruption of communication between
the right and left SMA caused by callosal damage. The
SMA, located on the medial surface of each hemisphere
anterior to the primary motor area, projects bilaterally to
the primary motor cortex (see Figure 2). Goldberg and
Bloom discussed two premotor systems, a medial system
involving the SMA and cingulate gyrus from which anticipatory movements emanate, and a lateral system involving the arcuate premotor area from which reactive
movements emanate. The medial and lateral systems are
thought to be mutually inhibitory and highly interactive.
When the left or right SMA is damaged, the lateral, or
reactive, premotor system becomes disinhibited and can
give rise to the avolitional movements seen in alien-hand
syndrome. SMA stimulation has long been known to
evoke complex movements of the contralateral limb,
including impulsive groping, grasp reflex, and impaired
bimanual coordination (Penfield & Welch, 1951). The
callosal lesion in many cases may function to produce
alien movements by dissociating the damaged SMA from
contralateral input (Gasquoine, 1993).
Little has been said about cases in which only the corpus callosum has been damaged. One possibility raised
by Bogen (1985) is that in individual cases of callosal
resection, the medial frontal cortex may have been
mildly damaged when the two hemispheres were
retracted. The less extensive frontal-lobe damage may
explain why these cases of alien-hand syndrome tend to
be more transient than cases with more severe frontallobe involvement, because contralateral SMA may play a
critical role in mediating recovery after unilateral SMA
injury (Eccles, 1982). An alternative explanation is that
alien-hand phenomena may arise from the disconnection of intact right and left SMAs. Strong evidence for
this possibility comes from the case reported by
Geschwind et al. (1995), in which MRI scan revealed a
discrete lesion in the posterior body of the corpus callosum, with no frontal-lobe pathology. As these investigators pointed out, the infarct occurred at the very site of
interhemispheric connection of the left and right SMAs
and primary motor cortices.
Dual premotor systems is the leading theory for alienhand syndrome, and it is useful as a general treatment.
Like most theories of the brain bases of complex disorders, it does not explain the extensive variations in
behaviors from person to person. The appearance of
alien-hand signs in patients without damage to the SMA
or corpus callosum also presents a problem for this theory. In the following section, we discuss in detail several
cases in which posterior brain damage gives rise to alienhand phenomena.

270

BEHAVIORAL AND COGNITIVE NEUROSCIENCE REVIEWS

CASES OF POSTERIOR DAMAGE


A number of cases of alien-hand signs have been
described in patients with infarction in the area of the
posterior cerebral artery (see Figure 3). Levine and Rinn
(1986) described the case of a right-handed woman with
left-sided neglect and left arm ataxia of a type suggesting
involvement of the right thalamus (sensory ataxia) and
the occipito-temporal areas together with damage to the
corpus callosum (crossed optic ataxia; optic ataxia is
misreaching due to loss of visual guidance of movement). CT scan confirmed damage to these areas.
Although the patient did not deny ownership, she perceived the left arm to be an alien presence with hostile
intentions. The arm struck her and tried to choke her,
pinched her, and knocked her glasses off. There was no
grasp reflex or intermanual conflict. The investigators
suggested that the patients movements were perceived
as alien because normally automatic, unnoticed movements were exaggerated and ill-controlled (ataxic) and
she lacked sensory feedback that would have enabled
her to determine that she had generated the movements, prompting the authors to suggest a new form of
alien-hand syndrome. As also discussed by Goldberg and
Bloom (1990), this lack of sensory feedback may be
equivalent to a disorder of corollary discharge, the anticipatory flow of information from motor regions back to
sensory regions, which provides the sensory system with
advanced notice of self-generated movements. This information allows the sensory consequences of the motor
action to be interpreted as arising from self-movement.
The concept of corollary discharge was introduced by
Teuber (1972), who described it as a physiologic marker
for the voluntariness of self-initiated movement.
An interesting side note to Levine and Rinns (1986)
case is the treatment of the symptoms of alien hand. The
patient was encouraged to make friends with her arm
by talking to it. The authors reported that she came to
treat it as a misbehaving child, fondling it and talking to
it: There, there, behave yourself now. . . . Dont be
naughty. The patient of Marey-Lopez, Rubio-Nazabal,
Alonso-Magdalena and Lopez-Facal (2002) (described
below) also regarded the left arm as a misbehaving child.
In another case in which the patient was instructed to try
talking to the hand, the strategy apparently was ineffectual (Della Sala et al., 1991). There are several other
cases in which the patient talked to the arm in a cajoling
way or attempted to reason with it. For example,
Nagumo and Yamadoris (1995) case called her alien
arm Grandma and asked it to please let me go when it
interfered with a task. Unlike in the case of Levine and
Rinn (1986), talking to the arm in these other cases was
not a prescribed treatment but rather the patients own
coping strategy.

Figure 3: Posterior Cerebral Artery, Right Hemisphere, Midsagittal


View.
NOTE: Alien-hand phenomena may arise from lesions to brain areas
including the thalamus, parietal and occipital lobes (depicted), and
the temporal lobes.

Ay, Buonanno, Price, Le, and Koroshetz (1998)


described a case of alien hand in a woman following
occlusion of the right posterior cerebral artery. MRI scan
showed infarction in the right thalamus, hippocampus,
inferior-medial temporal lobes, splenium of the corpus
callosum, and occipital lobe. Positron emission tomography (PET) scan revealed reduced metabolism in the
infarcted areas, as well as in right posterior frontoparietotemporal regions (more specific locations not
reported). She exhibited left-sided neglect with impairment of coordinated movements and reaching (sensory,
cerebellar, and optic ataxia). The patient initially complained that her left hand was acting as if it were under
someone elses control. The hand repeatedly attempted
to strangle and hit her throughout the day, and she
stated that she was afraid of him. She had dysfunction
of multiple cognitive domains. The alien-hand signs
diminished over 2 weeks.
The authors pointed out that most lesions that cause
spatial neglect, ataxia, and sensory loss also often cause
hemiparesis or motor neglect, which would preclude
movements such as those seen in their patient. They proposed that what appeared to be purposeful actions of the
alien hand (hitting, choking, etc.) may have been uncoordinated, spontaneous, self-stimulatory arm movements that were misinterpreted by the patient, who also
made errors of identification and causation of her other
symptoms unassociated with the alien hand. Although
the investigators did not draw attention to the frontal
aspect of the decrease of F-fluorodeoxyglucose uptake in

Scepkowski, Cronin-Golomb / THE ALIEN HAND


the right posterior-frontoparietotemporal areas found
on PET scan, it is of course possible that there was sufficient frontal pathology in this patient to contribute to
the seemingly purposeful movements of the alien hand.
Ball et al. (1993) described an unusual case of alienhand phenomena in a patient found at autopsy to have
had the neuropathology of Alzheimers disease. He presented with a left grasp reflex and frequent uncooperative movements of his left hand, described as involuntary
wandering. The movements of the left hand appeared
to be purposeless and did not interfere with actions of
the right hand. He showed no forced groping or utilization behavior. There was significant sensory inattention
to the left side of space. Many amyloid plaques and
neurofibrillary tangles, the neuropathological hallmarks of Alzheimers disease, were found in the medial
frontal cortex as well as a small old infarct in that area.
Neuropathology was not specifically reported in the
parietal lobes, but neuropsychological testing together
with the left-side sensory inattention suggested at least
right and probably bilateral parietal-lobe dysfunction.
Elements of alien-hand syndrome have been noted in
other patients with Alzheimers disease, including grasp
reflex and utilization behavior (Doody and Jankovic,
1992), but these may just be frontal release signs arising
from degeneration of multiple brain areas in this disease, including frontal cortex. One study reported a case
of biopsy-confirmed Alzheimers disease with a presenting sign of left-hand apraxia and behaviors reminiscent
of alien hand syndromes but gave no examples other
than one of intermanual conflict. There was mild frontal
and pronounced right parietal hypoperfusion in this
case (Green et al., 1995). Another case of alien hand with
Alzheimers disease is described below; in this case, the
signs did not suggest any frontal-lobe involvement
(Carrilho et al., 2001).
Because the patient of Ball et al. (1993) clearly had
frontal-lobe pathology, one might decide that here,
alien-hand phenomena arose from frontal-lobe involvement although with an unusual etiology. Behavioral and
anatomical aspects, however, distinguish this case from
most with alien hand. First, because there was no
intermanual conflict but also few frontal-type signs such
as purposeful movements of the alien hand, the case
looks like neither a callosal nor frontal type. Moreover,
the patient showed left-side inattention, a feature of
parietal-lobe damage. The probability of parietal dysfunction was supported by his neuropsychological profile. As we shall see from the next set of cases, alien hand
in patients with lesions of the parietal lobes may be an
entity distinct both behaviorally and anatomically from
the usual callosal and frontal cases.
Ventura, Goldman, and Hildebrand (1995) reported
the case of a woman who manifested left alien-hand signs

271

following a right thalamic hemorrhage with midbrain


extension but no involvement of the corpus callosum.
PET scans revealed reduced glucose metabolism in the
right posterior thalamus and the right frontal, parietal,
and temporal cortices, with the most pronounced
hypometabolism in the sensorimotor area (site unspecified). She exhibited mirror movements and spontaneous left-arm movements perceived by her as levitation.
She described the movement as being effected by someone else. There was no grasp reflex and no report of
impulsive groping, utilization behavior, or intermanual
conflict. In fact, she was able to cross-localize tactile stimuli from the left hand to the right and vice versa, indicating adequate interhemispheric transfer and hence corpus callosum function. She did show the classic alienhand sign of being unable to recognize her hand as her
own in the absence of visual cues. She exhibited tactile
neglect on the left side and visuoconstructive impairment, both consistent with right parietal dysfunction.
Her neuropsychological examination was otherwise normal. Approximately 10 weeks after onset, the symptoms
diminished. In considering the behavioral signs in this
case, Ventura et al. suggested that the parietal lesion produced left-arm neglect, which could account for the
alien feel of the arm, whereas the lesion to thalamic and
subthalamic areas accounted for the involuntary motor
activity.
A somewhat similar case of alien hand after right
thalamic infarction was reported by Marey-Lopez et al.
(2002). This was a circumscribed lesion with no additional infarction seen in the corpus callosum or other
cerebral regions. Like the case of Ventura et al. (1995)
above, this patient exhibited spontaneous left-arm
movements, including levitation, the inability to recognize her hand as her own without visual cues, sensory
ataxia, and a variety of spatial impairments including
mild left spatial neglect, astereognosia (inability to recognize an object by touch), and significant hemisensory
defects. Also like the previous case, she showed no
intermanual conflict, consistent with sparing of the corpus callosum, but unlike that case, she did not exhibit
mirror movements. Her initial reaction to the alien feeling and involuntary movements of the hand was astonishment and fear, the latter arising from the arms propensity to hit her in the fact and on the body. After 1
week, she was able to suppress some involuntary movement under visual guidance. Her fear consequently subsided and she treated the arm as a misbehaving child.
The feeling of foreignness and involuntary movements
persisted to her death 1 year later.
The posterior cases reported by Ventura et al. (1995)
and Marey-Lopez et al. (2000) were characterized by
behaviors not seen in more anterior lesions. In these posterior cases, the corpus callosum was spared. Of interest

272

BEHAVIORAL AND COGNITIVE NEUROSCIENCE REVIEWS

in this context is a 23-year-old patient described by


Nishikawa et al. (2001) who showed similar behaviors
(right hand levitation and purposeless movements) and
damage to posterior areas (left parietal cortex and
subcortex) but also had lesions of much of the body and
splenium of the corpus callosum; the parietal damage
occurred through transcortical operation on a callosal
tumor. Her additional symptoms of groping and compulsive tool use presumably arose from the callosal damage, with no evidence of lesions of the cingulate cortex
or supplementary motor areas. Her most unusual sign
was what the authors described as conflict of intention,
a whole-body freezing for minutes to hours reflecting an
inability to act on one of competing desires. The authors
reported similar exhibitions of conflict of intention in
two other cases with lesions to the posterior body of the
corpus callosum without cortical damage; these cases
did not show alien-hand signs.
Groom, Ng, Kevorkian, and Levy (1999) described an
especially novel case in which the patient perceived his
alien hand as positive rather than hostile or bothersome.
He introduced his arm to his examiner as his little
buddy and remarked on the interesting behaviors it
engaged in, such as massaging his head (albeit
ataxically). His left arm exhibited impulsive reaching
and groping as well as mirror movements when writing.
MRI scan showed an ischemic lesion near the posterior
cerebral artery comprising the medial and posterior
parts of the right temporal lobes and right posterior parietal and occipital lobes but not the corpus callosum.
Neuropsychological tests of posterior cortical function
were abnormal. Frontal-lobe executive function was difficult to assess because of the patients difficulties in
visual search. At 10-week follow-up, the alien-hand signs
were absent, but he still referred to the arm in the third
person.
This patient showed anosognosia (denial of illness),
left-sided spatial neglect, and la belle indifference, all characteristic of significant right-hemisphere stroke. He was
described as friendly, talkative and lighthearted . . . smiling as he made puns and told stories to staff on the
unit . . . and had difficulty appreciating the need for continued rehabilitation. This man had alien-hand signs
that resembled those seen with frontal-lobe lesions in
terms of purposeful movement. Whereas most patients
with alien-hand signs perceive them with astonishment,
fear, horror, frustration, or at least distaste, in this parietal case, the signs were ego-syntonic. The authors suggested that the patient had little incentive to stop personifying the arm because it was never ego-dystonic.
A similar case of ego-syntonic alien hand was
described by Pack, Stewart, Diamond, and Gale (2002)
resulting from a right lateral thalamic infarct. The
patient named his arm George and appeared very fond

of it and amused by its behavior despite its tendency to


strike him in the face. The authors reported that the
uncontrolled movements of his arm diminished
throughout a standard course of inpatient rehabilitation, although the details of the rehabilitation were not
offered.
Two cases of alien-hand signs resulting from posterior
damage were reported by Leiguarda, Starkstein,
Nogues, Berthier, and Arbelaiz (1993). The first was a
right-handed man who had a left parietal arteriovenous
malformation surgically removed. Following surgery, he
was left with right hemiplegia that eventually recovered,
but he suffered a focal seizure a year later resulting in a
right alien hand. His arm moved spontaneously in a purposeless way. These movements were noted to occur only
when the seizure recurred twice, but the patient frequently experienced the feeling that his arm did not
belong to him, prompting him on one occasion to shout
at his arm. Leiguarda et al.s other patient was a righthanded woman with an intracerebral right parietal
hematoma secondary to a ruptured arteriovenous malformation. Eighteen months following evacuation of the
hematoma and surgical removal of the malformation,
the patient experienced two transient alien-hand episodes involving her left arm. It was noted to elevate and
move toward her involuntarily. During the first episode,
the woman was so terrified that she begged the examiner
to stop this monster, fearing that it would kill her. No
intermanual conflict, grasp reflex, or groping was noted
in either case. Similar levitation-like signs were noted in
a case by Rohde, Weidauer, Lanfermann, and Zanella
(2002), in which a right-handed woman gradually lost
control over her right hand due to atrophy of the
precentral and postcentral gyri. She felt that her right
hand did not belong to her, that it doesnt do what its
supposed to, and it was noted to elevate uncontrollably,
sometimes provoked by sudden noises or coughs.
In an unusual case, alien-hand symptoms were experienced by a right-handed man suffering from a transient
ischemic attack (Andre & Domingues, 1996). On waking, he could not inhibit groping and grasping
automatonlike gestures of his left hand. The movements resolved within 20 min and he recovered fully.
These symptoms, plus the pronounced leg weakness
experienced during the attack, suggested a dysfunction
of the medial frontal lobe, although there was no direct
evidence of frontal pathology. CT scan instead revealed
older lesions in the left internal capsule, right occipital
lobe, and left cerebellar hemisphere in this patient, who
had multiple risk factors for stroke.
Bundick and Spinella (2000) recently described
another case of posterior alien-hand phenomena in a
patient with CT- and MRI-confirmed infarct in the right
parieto-temporal-occipital cortex and white matter, spar-

Scepkowski, Cronin-Golomb / THE ALIEN HAND


ing the corpus callosum, and additional abnormalities in
right medial and lateral frontal cortex and white matter.
The patient complained that her left arm had a mind of
its own, and she spoke about it as if it were a separate
entity. She reported often that she could not find her left
arm, a difficulty compounded by uncontrolled levitation
of the arm combined with finger writhing that would
occur while awake and asleep. These were the only alien
signs exhibited by the patient, who showed no grasping,
groping, or intermanual conflict, and they greatly
reduced in frequency by 75-day follow-up. She retained a
pronounced left-sided neglect and severe visuospatial
deficits even after the clearing of her alien-hand signs.
Although the possible role of the frontal lesion cannot be discounted in this case, the arms behaviors
appeared similar to those of the other posterior cases
described here, including levitation and other
nonpurposeful movements. Other candidate components of the posterior syndrome, if we may call it that, are
left-sided spatial neglect in one or more sensory modalities and neuropsychological or neurophysiological evidence of parietal-lobe dysfunction. Two additional
reports of parietal lesions of various etiologies document
this same pattern of behaviors. The patient described by
Dolado, Castrillo, Urra, and Varela de Seijas (1995) had
purposeless movements, tactile neglect, and optic
ataxia, although also some purposeful movements and
intermanual conflict; there was no evidence of callosal
or frontal lesions. The authors suggested that the parietal lesion interfered with normal visual-somatosensory
interactions. Carrilho et al. (2001) found alien-hand levitation in one patient with parietal stroke, one patient
with Alzheimers disease, and two patients with corticobasal ganglionic degeneration. The case of Marey-Lopez
et al. (2000) exhibited levitation, left spatial neglect, and
other signs suggestive of right parietal dysfunction,
although imaging scan revealed a circumscribed right
thalamic infarct.
Cortico-basal ganglionic degeneration is a disorder
characterized neuropathologically by fronto-parietal
atrophy with distinctive swelling of neuronal cell bodies
and resistance to staining. Behaviorally, it appears as a
parkinsonian syndrome, with slowness and awkwardness
of voluntary movements together with involuntary
movement (Kompoliti et al., 1998). A recent review of
147 cases by Kompoliti et al. documented alien-hand
phenomena in 63 patients (42%), although the types of
signs were not described. Transcranial magnetic stimulation of the brain of patients with cortico-basal ganglionic
degeneration and alien-hand signs (type not specified)
indicated that these patients had enhanced excitability
or reduced inhibition of the motor areas stimulated,
making for an abnormal cortical representation of the
hand muscles (Valls-Sole et al., 2000). Although there

273

was insufficient information provided to evaluate the


types of alien-hand signs that occur in this disorder, the
results of this study suggest the exciting possibility that
new techniques such as transcranial magnetic stimulation may shed light on the pathophysiology of alienhand syndrome associated not only with cortico-basal
ganglionic degeneration but also with other etiologies.
These etiologies would include the relatively common
types arising from lesions of the frontal lobe and corpus
callosum as well as behaviorally similar cases with
unusual etiologies such as Creutzfeldt-Jakob disease
(MacGowan et al., 1997).
We have evaluated the symptoms and signs of alienhand syndrome associated with posterior pathology and
present in Table 2 a summary of relevant studies in which
arm levitation and groping were frequent features.
A POSTERIOR SUBTYPE?
It is debatable whether the cases of alien hand associated with posterior lesions represent a subtype distinct
from those with medial-frontal and callosal damage.
Feelings of the arm wandering, instances of levitation,
and in general movements that are purposeless rather
than purposeful appear to be more common in these
cases with posterior and subcortical involvement than in
frontal and callosal cases. The purposeless/purposeful
distinction is seen even in transient cases arising from
seizure disorders of the posterior parietal cortex
(Leiguarda et al., 1993) and the medial frontal cortex,
respectively (Feinberg, Roane, & Cohen, 1998;
Leiguarda et al., 1993). The case of Groom et al. (1999)
raises the question of whether damage to the frontal
lobe or corpus callosum is necessary for the emergence
of alien-hand signs of groping, grasping, intermanual
conflict, and mirror movements, in that this patient without frontal damage or callosal infarct exhibited groping
and mirror movements. The cases of Marey-Lopez et al.
(2002) and Levine and Rinn (1986) also exhibited groping behaviors. Because several of the posterior cases
manifested additional frontal if not callosal damage
(except splenium), however, these appearances of alienhand signs need not be assigned a completely novel anatomical substrate. The right thalamic case described by
Marey-Lopez et al. (2002) did not show these putatively
frontal-type signs but nevertheless exhibited the basic
features of alien hand (involuntary movement and feeling of foreignness) in the absence of direct damage to
the corpus callosum, frontal lobes, or other cerebral
regions. These two cases in particular, and any future
cases of alien-hand signs arising exclusively from posterior damage sparing the corpus callosum and frontal
lobes, may be pivotal in helping researchers to develop a

274

BEHAVIORAL AND COGNITIVE NEUROSCIENCE REVIEWS

TABLE 2: Cases of Alien Hand Signs Observed Following Posterior Pathology


Reference

Case

Levine and Rinn (1986)

Area of Damage

Hand

Arm Levitation Groping

R temporal/occipital cortex, posterior thalamus

L posterior parietal cortex


R parietal/temporal cortex

R
L

X
X

Bilateral parietal

Green et al. (1995)

AD, R parietal cortex, mild frontal

Ventura, Goldman, and Hildebrand (1995)

R capsulothalamic area, R frontal/temporal/


parietal cortex

Andre and Domingues (1996)

L internal capsule, R occipital cortex, L cerebellum

SCC, R posterior frontal/temporal/occipital/


parietal cortex, thalamus

Groom, Ng, Kevorkian, and Levy (1999)

R medial posterior temporal cortex, posterior


parietal/occipital cortex

Bundick and Spinella (2000)

R posterior parietal/temporal cortex,


medial frontal cortex

CBGD, R temporal/parietal cortex


CBGD, R temporal/parietal cortex
AD, L parietal cortex
Stroke, L parietal cortex

L
L
R
L

X
X
X
X

Marey-Lopez, Rubio-Nazabal,
Alonso-Magdalena, and Lopez-Facal (2002)

R thalamus

Pack, Stewart, Diamond, and Gale (2002)

R lateral thalamus

Rohde, Weidauer, Lanferman, and


Zanella (2002)

L pre/post central gyri (parietal), subcortical

Leiguarda, Starkstein, Nogues,


Berthier, and Arbelaiz (1993)
Dolado, Castrillo, Urra, and
Varela de Seijas (1995)

3
4

Ay, Buonanno, Price, Le, and


Koroshetz (1998)

Carrilho et al. (2001)

1
2
3
4

X?
R

NOTE: AD = Alzheimers disease; CBGD = cortico-basal ganglionic degeneration; L = left; R = right; SCC = splenium of corpus callosum. Focus is on
cases exhibiting levitation or groping.

nosology of the alien hand based on specific behaviors


and their neuropathological underpinnings.
It should be noted that the available case examples of
alien hand resulting from posterior damage generally
offer less detail on observed behaviors and other neuropsychological testing than do the cases of frontal and
callosal damage. It is possible that other alien-hand signs
or relevant behaviors in these cases were present but
were either unobserved or unreported.
Posterior Cases, Disconnection, Ataxia,
and Abnormalities in Body Schema
An important contribution of Groom et al. (1999) was
in postulating a possible anatomic substrate for the several behaviors exhibited in their case and possibly in others as well. Specifically, they suggested that a disconnection syndrome might have been at work, though not
through the usual route of corpus callosum and other
forebrain commissures. They provided the example of
the medial paralimbic system that contributes to the perception of volitional control of action. As noted by

Goldberg and Bloom (1990), damage to this system may


lead to loss of feeling of the intentionality of action and
the feeling of foreignness of the afflicted arm. The fiber
tracts originate in the hippocampal area and the basal
ganglia and project to the anterior cingulate. The anterior cingulate and adjacent supplementary motor area
often are affected in alien-hand syndrome, as mentioned
in the cases already described and discussed in more
detail in the next section. The right and left SMA connect with each other through the corpus callosum. In
light of this organization, it is conceivable that a lesion
anywhere in the network may yield symptoms like those
seen with direct callosal section.
There is ample precedent in the literature for noncallosal, corticocortical disconnection syndromes. Norman Geschwinds (1965a, 1965b) influential recasting of
classic behavioral deficits as disconnection syndromes
included consideration of right-hand apraxia, visual agnosia (inability to recognize objects visually), and alexia
(inability to read). More recently, disconnection has
been discussed as giving rise to perceptual and cognitive

Scepkowski, Cronin-Golomb / THE ALIEN HAND


deficits in neurodegenerative disorders, such as the corticocortical interruptions of Alzheimers disease (Hof &
Bouras, 1991) and breaks in the cortico- striatothalamic
and corticocortical circuitry in Parkinsons disease
(Cronin-Golomb & Amick, 2001; Cronin-Golomb and
Braun, 1997). Moreover, as Groom et al. (1999) noted,
the ability of high-order cognitive information to transfer interhemispherically in the absence of the corpus callosum and other forebrain commissures indicates the
presence of alternative, subcortical pathways between
the right and left cerebral hemispheres (CroninGolomb, 1986; Cronin-Golomb, Gabrieli, & Keane,
1996).
Novel conceptualizations of the pathology underlying alien-hand phenomena in cases of posterior damage
center on changes in body schema overlaid with involuntary arm movement (e.g., Ay et al., 1998; Bundick &
Spinella, 2000; Marey-Lopez et al., 2002). Patients with
dysfunction of cortical or subcortical posterior structures may experience sensory loss, neglect, and ataxia on
the body side of the alien hand. Lack of sensory feedback
would result in the perception of the arms involuntary
movements as having a foreign source. Marey-Lopez
et al. (2002) further noted that thalamic lesions are
known to sometimes have remote cortical effects.
It is presently unclear whether there is a single, ultimate etiology of alien-hand syndrome in posterior cases.
As detailed above, in some instances alien-hand symptoms may have arisen from additional direct pathology
of the frontal cortex. In others, disconnection of frontal
areas such as the SMA from the posterior site of direct
damage may have sufficed to produce alien-hand phenomena. In both types of cases, the dual premotor system theory of alien hand could still validly explain the
development of symptoms.
An alternative explanation is the development of
alien-hand phenomena as a response to involuntary,
ataxic movement without accurate sensory feedback.
This type of case would be truly posterior in origin, both
neuropathologically and symptomatically. Superficial
resemblance to frontal and callosal types of alien-hand
syndrome would not be supported by common sites of
pathology. The question then arises: Why do only some
cases of posterior insult that are associated with sensory
loss or neglect, loss of sensory feedback, and arm ataxia
in addition experience alien hand? Is the key to understanding the posterior cases embedded in the extent of
disruption of body schema, or extent of sensory ataxia?
As suggested by some investigators (Bogen, 1979; Levine
& Rinn, 1986), certain aspects of personality contribute
to the tendency to personify the alien hand. Might this
be true especially for the genuine posterior cases in their
propensity to interpret ataxic movement as derived from
a foreign source? These questions await responses from

275

investigators who are committed to carefully examining


sensory and motor function, spatial abilities through
detailed neuropsychological assessment and imaging,
and personality factors in patients with alien-hand
symptoms of putatively posterior origin.
FUTURE DIRECTIONS
One of the difficulties in systematically studying the
alien hand is its rarity relative to other neurological disorders, forcing reliance on case reviews. Another is its
transience in most cases, and a third is the coincidental
occurrence of other behavioral dysfunctions that hinder
assessment, such as hemiparesis, motor neglect, or
neglect of visual hemispace. As well, there are general
limitations to extensive assessment in patients who have
suffered a severe brain disorder such as stroke or tumor.
This combination of features makes it difficult for
researchers to apply the same clinical and neuropsychological assessments to a large number of patients.
Elucidating the nature of the alien-hand syndrome
may depend on the ability to devise a standard assessment that examines the full range of alien-hand signs,
together with the use of ever-improving imaging technology. Gasquoine (1993) suggested that a full assessment include standardized unimanual and bimanual
motor tests; an index of self-awareness of deficits; Brion
and Jedynaks (1972) test of alienation; measures of utilization behavior and grasp reflexes; neuropsychological
evaluation, especially of frontal-lobe function; quantification of motor weakness, visual and somatosensory loss;
examination for dyspraxia and other signs of callosal
and noncallosal disconnection; and a standard state
measure for anxiety (presumably to evaluate personification and affect, though not so stated). We would add a
measure of personality and, for posterior-type cases,
neuropsychological evaluation with an emphasis on
parietal-lobe function.
With regard to advances in technology, there is as yet
no report to our knowledge of a case of alien hand studied with functional MRI, and cases described with any
type of functional technology (e.g., PET, transcranial
magnetic stimulation) are quite rare. A combination of
standard symptom assessment and functional imaging
would be a powerful means of addressing the general
value of the dominant dual premotor system theory as
well as addressing the question of whether frontal,
callosal, and posterior are necessary and sufficient as
subtypes of alien-hand syndrome. Future efforts in
behavioral assessment and neuroimaging will be important contributors in the quest for a rational classification
of possible subtypes defined by clinical signs and areas of
brain injury, and they may help unravel the mystery of
the individual patients alien hand.

276

BEHAVIORAL AND COGNITIVE NEUROSCIENCE REVIEWS

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