A. Pappalardo
10.1177/0888439004269031
Posterior
AlienetHand
al. Syndrome

CASE REPORT

Posterior Alien Hand Syndrome:
Case Report and Rehabilitative Treatment
A. Pappalardo, M. R. Ciancio, E. Reggio, and F. Patti

Alien hand syndrome (AHS) is a rare syndrome characterized by involuntary, uncontrollable, and purposeless movement of one upper limb, which is considered as extraneous
by the patient. The term AHS was previously used to describe
a syndrome due to lesions in the anterior corpus callosum.
Successively, some authors reported cases of AHS in patients
due to posterior cerebral lesions, without lesions of the corpus callosum. Thus, it was possible to distinguish the posterior variant of AHS from the anterior form. The authors
report an unusual case of posterior AHS due to a unique
parieto-occipital lesion of the dominant hemisphere. However, the patient showed clinical findings that were similar
to the anterior AHS. The rehabilitative treatment, individualized and targeted toward the specific needs of the patient,
allowed the improvement of the patient’s participation in
activities of daily living.
Key Words: Alien hand syndrome—Rehabilitation.

T

he alien hand syndrome (AHS) is a condition
characterized by involuntary and uncontrollable motor activity of an upper limb, associated
with sense of extraneousness of the same limb.1 Such
movements are aimless and differentiable from those
choreic and athetosic (mioclonic). Criticism is
another specific condition of AHS, in which the
patient criticizes the behavior of his affected limb,
with a feeling of frustration.2

From Dipartimento di Scienze Neurologiche, Università degli Studi
di Catania, Italy (AP, ER, FP), and U.O. Medicina Fisica e Riabilitazione, Ospedale Gravina e S. Pietro, Caltagirone, Italy (MRC).
Address correspondence to A. Pappalardo, MD, PhD, Dipartimento
di Scienze Neurologiche, Università di Catania, Azienda
Policlinico—Edificio 20, Via S. Sofia 78, Catania, Italy. E-mail:
pange.bel@virgilio.it.
Pappalardo A, Ciancio MR, Reggio E, Patti F. Posterior alien hand
syndrome: case report and rehabilitative treatment. Neurorehabil
Neural Repair 2004;18:176–181.
DOI: 10.1177/0888439004269031

176

Initially AHS was used to indicate cases with
anterior corpus callosum lesions, determining
involuntary movements. 3 Afterward, some
authors described other cases of AHS involving
lesions of the corpus callosum plus dominant
medial frontal cortex4 and posterior corticalsubcortical areas.5
Goldstein first described the syndrome in a
discussion of apraxia in 1908. 6 Brion and
Jedynak introduced the term “main etrangere”
to describe a feeling of foreignness of an arm in
patients with lesions of the corpus callosum.7
Goldberg applied the term to unintended motor
actions of the hand opposite to medial frontal
lobe lesions.8
Actually 3 varieties of AHS are reported:
1) AHS provoked by a lesion of the corpus callosum alone, where the most important feature is
the intermanual conflict9;
2) AHS due to a lesion of the dominant medial
frontal cortex plus corpus callosum, characterized by compulsive manipulation of tools,
associated with grasping and groping signs10;
3) AHS due to a lesion in cortical region supplied
by the right posterior cerebral artery and characterized by stereotyped simple hand.11

Further cases with thalamic injury were also
described.12 AHS was due to posterior lesions
resulting either from cortico-basal degeneration
involving primarily posterior cortical degeneration or from cerebrovascular accidents.13 In this
subtype of AHS movement, disorders are less
specific.
The first 2 types of AHS are classified as an
“anterior” or “motor” form of AHS, whereas the
posterior AHS (PAHS) is also defined as a “sensory” form.

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Posterior Alien Hand Syndrome

Table 1.

Neuropsychological Evaluation of Visual-Spatial Cognition and Constructive Abilities

Description of the Task

Number of Errors T0/T1

Length of 10 different lines
Angle between 2 lines (10 tasks)
Spatial orientation of 10 lines
Mental rotation of 10 two-dimensional complex geometric figures
Identification of 10 complex figures
Identification of 10 hidden figures
Mental construction of 10 complex figures

8/2
8/2
7/1
10/3
8/2
8/2
10/3

T0 = start of rehabilitation; T1 = 6 months after start of rehabilitation.

Recently, the term “anarchic hand” has been
proposed for unintended actions to distinguish
them from the “alien hand” in its original sense.14
We report a rare case of AHS due to left parietooccipital damage from ischemic stroke. However,
the clinical findings were typical of the anterior
form. We also highlight that in our patient the
PAHS involved the dominant limb. Contrarily, the
reported cases of PAHS affected the nondominant
limb.5

CASE REPORT
MA, male, 60 years old, right-handed, with a
high education level, was injured by an ischemic
stroke, due to an occlusion of the left posterior
cerebral artery. At the hospital, the neurological
examination highlighted a moderate right upper
motor neuron facial paresis, associated with mild
weakness of both upper and lower limbs, Babinski
on the right side. Verbal comprehension, repetition, and reading were moderately impaired, as
well as object naming to visual and tactile
presentation in both the hands.
Initial brain CT without contrast evidenced
probable infarct in the left cortical-subcortical
parieto-occipital region. Routine blood laboratory
data were unremarkable. The electrocardiogram
revealed normal sinus rhythm with occasional
supraventricular premature beats and a previous
inferior wall infarction. Follow-up MRI at 15 days
poststroke onset showed low density in the left
parieto-occipital gray and white matter, with
sparing of the corpus callosum.
Three months after stroke onset, neurological
examination highlighted complete recovery of the
right facial-brachial-crural hemiparesis; word comprehension, repetition, and reading were normal,
as well as object naming to visual and tactile presentation in both the hands.
Examination of the motor behavior revealed the
following signs in the right upper arm: unwanted,

uncontrollable, and purposeless motor activity,
well distinguishable from other known movement
disturbances such as myoclonus, athetosis, chorea, and so on; intermanual conflict; rare involuntary synchronous movements during voluntary
unilateral movements of the nonaffected limb; and
difficulty releasing objects.
MA did not recognize his right arm and right
hand as being part of his own body. Autocriticism
toward the affected arm was present too; MA
defined his right hand as “handicapped.”
Activities of daily living (ADLs) were severely
impaired. MA was unable to use a knife or a fork
while eating, dress himself, do up buttons, shave
himself, drink a glass of water, catch, and manipulate and release various tools. Also the bimanual
coordination was seriously impaired. The total
score of a Functional Independence Measure
(FIM)15 was 70/126.
The patient dramatically suffered from his incapability to control his hand. Furthermore, attempts
to execute regular repetitive self-paced flexionextension movements of individual fingers of the
right hand resulted in errors of timing and
sequencing, which were not observed in the left
hand.
A new brain MRI, 3 months postonset, demonstrated a large left cortical-subcortical parietooccipital ischemic injury (Figure 1). Acoustic P-300
wave with odd-ball paradigm 1 6 showed a
morphologic alteration of P-300 wave and a
marked increase in its latency.
The neuropsychological evaluation demonstrated severe impairment of both perception and
spatial thought, deficit of visual-spatial cognition,
and constructive disorders (Table 1). MA did not
evidence neglect in spatial exploration tasks (line
and letter cancellation, sentence reading).
The patient was asked to
1) measure the length of 10 different lines,
2) measure the angle between 2 lines (10 tasks),
3) recognize the spatial orientation of 10 lines,

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Figure 1. Brain NMR, showing a large left parieto-occipital ischemic injury.

4) perform the mental rotation of 10 two-dimensional complex geometric figures,
5) perform the identification of 10 complex figures,
6) perform the identification of 10 hidden figures,
and
7) perform the mental construction of 10 complex
figures.17

We started the rehabilitative treatment 3 months
after the stroke onset.18,19 In total, the rehabilitative
therapy went on for 6 months, 6 times a week for
the 1st month, 3 times a week from months 2 to 6.
As intermanual conflict interfered with intended
movements, we initially positioned the affected
arm on the patient’s back when the patient was
away from the rehabilitative setting. In this way,
intermanual conflict was inhibited and the patient
could use the nonaffected arm in many ADLs.
Obviously, the motor training was focused on the
affected arm within the rehabilitative setting. Afterward, we gradually encouraged the use of the right
arm in some ADLs.
Rehabilitation was planned by the physiatrist in
collaboration with the neurologist, the
neuropsychologist, the therapist, and members of
the patient’s family. His family lived in close contact with the patient and provided useful contributions and suggestions on strategies that could be
adopted.
The rehabilitation program put active and conscious participation at the forefront. We focused
our attention to environmental factors such as the
manner in which health care professionals
informed the patient and overprotection by family

178

members and nurses. All the rehabilitative treatment was directed to meet the needs of the patient,
who was gradually motivated to use the acquired
abilities in his daily activities. At the beginning of
therapy, the patient failed to recognize the special
position of his limbs while watching the mirror.
Such errors were not evident without a mirror. This
sign may be due to the impairment of spatial
abilities.
We trained the patient to use the temporal
sequence assignments with himself (catching a
bottle of water, unscrewing the cap, and pouring
out the water in a glass), as well as visual feedback,
to minimize the intermanual conflict.
The patient regained enough competence in the
performance of some therapeutic practices just
based on daily activities; in this way, he was gradually able to cut most types of food and use implements with the right hand. Although there was
functional improvement, his hand motor behavior
was not fluid and was poorly coordinated. We also
used a modified pen that, being larger than a normal one, facilitated both handling and pinching
movements. MA gradually regained the ability to
write with his right hand. The manipulation of
tools was performed by wearing gloves; this task
was facilitated by handling smooth round tools. In
this way, the patient could either catch or release
the tools.
Because returning to work constituted a main
goal in MA’s life, we tried to create a special rehabilitative setting with particular focus on job activities (calling on and answering the telephone, writing for public relations, etc.).

RESULTS
A new neuropsychological, neurophysiological,
and motor examination was performed 6 months
after the beginning of rehabilitative therapy.
The patient was still obliged to practice the various tasks feedback with both sustained attention
and effort. He managed to identify both complex
and hidden figures, as well as measure both length
and space orientation. Furthermore, he could carry
out the mental construction of complex figures,
even if he sometimes failed the mental rotation of
bidimensional complex geometric figures (Table 1).
The total score of the FIM was 112/126.
The motor examination evidenced the reduction of the involuntary and purposeless activity.
Manipulation of tools became less compulsive
than at the beginning of rehabilitation; MA was

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Posterior Alien Hand Syndrome

able to release objects. Even the intermanual conflict had subsided, but the sense of alienness persisted as well as the autocriticism. Nevertheless,
the patient was still obliged to practice the various
tasks feedback with both sustained attention and
effort.
The second electrophysiologic evaluation demonstrated a normalization in either the latency or
the morphology of the P-300 wave. As known, the
P-300 wave may be considered as a neuropsychophysiologic marker of cognitive process involving
attention and skills, generated by the activity of
posterior cerebral areas (parietal and occipital
lobes mainly).
In the follow-up examination, 24 months after
the stroke event, we found neither compulsive
manipulation of tools nor intermanual conflict.
Specific rehabilitative treatment had not been
executed between 6 months and 2 years after start
of the therapy.

DISCUSSION
We present a rare case of PAHS, unusual because the patient exhibited
— intermanual conflict, which is usually particular
to callosal lesion,
— compulsive manipulation and difficulty in
releasing tools, which are typical features of a
frontal lesion, and
— sense of extraneousness of the right arm and
hand, as being part of his own body.

These pathological findings involved the dominant limb.
Our case of PAHS differs substantially from
those reported in the literature. Levine and Rinn20
described a case of PAHS associated with
opticosensory ataxia. According to them, alien
hand movements are caused from posterior disconnection of the hemisphere, due to right
temporo-occipital infarction.
Doody and Jancovic21 reported a case of a
patient with a large right posterior infarct involving
the corpus callosum and posterior internal
capsule.
Bundick and Spinella5 issued the case of a
woman presenting uncontrolled levitation in the
left arm, without intermanual conflict. The lesion
was located in the right parietal-temporal-occipital
region. They speculated that the sense of estrangement from the nondominant upper limb and its

movements were similarly produced by body
schema distortion and hemineglect secondary to
the nondominant parietal lobe infarct.
Ay et al.22 described a case of left AHS, characterized by involuntary movement disorders of primary sensation and sensory processing, due to an
infarction in the right thalamus, hippocampus,
inferior-medial temporal lobes, and splenium of
the corpus callosum. Furthermore, the patient
exhibited a combination of sensory, optic, and cerebellar ataxia (triple ataxia).
Pack et al.23 published a case of PAHS with primary sensory loss and hemispatial neglect, different from our case, because in our patient, primary
sensation was spared.
Recently, Rohde et al.24 reported a case of PAHS
involving the dominant right hand. Clinical features consisted of uncontrollable elevation of the
right arm, associated with a sense of alienness. MRI
showed left central-parietal cortical and
subcortical lesions and extensive atrophy of the
pre- and postcentral gyri. The authors postulated
(as Ay et al.22) that the sensation of estrangement
was due to a distortion of the body image secondary to the postcentral cortical lesions, even though
this phenomenon is usually observed in the
nondominant limb. Surely the case described by
Rohde is the most similar to ours, even if it differs
in anatomical location.
From a clinical point of view, MA’s case should
be classified as a “motor” or “anterior” form of
AHS, despite the fact that both corpus callosum
and frontal lobe were not directly involved. The
“posterior” form is often characterized by the
impairment of the sensitive system, which was
spared in our case. In any case, some authors disagree with the classical criteria adopted to correlate clinical aspects with anatomical localization in
AHS. According to Jiu-Lech Chan and Ross,25 the
significance of the intermanual conflict as a sign of
callosal disconnection is not well validated. But if
we consider the clinical and anatomical findings,
we can classify our patient as neither posterior or
anterior.21-24 As affirmed by Aboitiz et al.,26 the
above-mentioned categorization might be insufficient to distinguish all clinical forms reported for
this syndrome. So Aboitiz et al. recently proposed
dividing these syndromes into at least 4 broad
categories:
1) intermanual conflict and diagnostic dyspraxia, in
which one hand (usually the left one) interferes
with the movements of the other hand;
2) alien hand sign, in which the hand is perceived as
extraneous by the patient;

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A. Pappalardo et al.

3) syndrome of the anarchic hand, which concerns
denial of the patient’s own intentions for actions of
the hand; and
4) supernumerary hands, in which the patient complains of feelings of having an extra extremity.

ing to hypothesize that the severe impairment of
bimanual coordination could have taken advantage from the improvement of visual-spatial
disorders.
An individualized rehabilitation program targeted to the specific requirements of the patient,
aimed either at inhibiting the pathological aspects
of AHS (intermanual conflict) or at stimulating the
correct manipulation of tools, may be indicated as
a possible therapy in AHS.

These different forms may coexist in 1 patient. The
case of MA may best fit the 1st 2 categories.
However, it is difficult to explain why a parietaloccipital lesion would produce the intermanual
conflict. One possible explanation might be an
outflow from the parietal cortex into the frontal
cortex or fronto-striatal system. For the reasons
described above, there is perhaps a potential need
to reconsider the correlation between the clinical
features and the anatomical localization of AHS.1
Literature data on rehabilitative treatment of
AHS is lacking. Few authors have addressed this
issue. Wu et al.9 described a potentially effective
technique involving biofeedback for patients with
AHS. Groom et al.2 suggested the masking and/or
fastening of the affected hand. Pack et al.23 underlined the excellent prognosis of PAHS, with resolution of associated neurological impairment during
the course of rehabilitation.
We proposed a rehabilitative treatment,
planned on the real needs of the patient and corroborated by ecological contents. It allowed an
evident improvement of the patient’s participation
in daily living activities. The neuropsychological
rehabilitation targeting the selective treatment of
both visual-spatial and constructive disorders
allowed the recovery of the ability to recognize
geometric forms, spatial representation with generalization, and the “transfer” of every reacquired
skill in routine daily activities.
The patient achieved a level of complete independence (112/126 FIM score). He returned to
work as expected by the rehabilitative aims. He is
now completely independent in taking care of
himself, but the harmonious flow of the gesture
has slowed down.
There was a clear reduction in intermanual conflict, so that the patient could carry out many activities, like writing, dressing, and lifting objects. This
recovery was partly due to spontaneous improvement, which usually occurs in the 1st months following stroke onset or a brain injury. Most important, however, the recovery was due to the
rehabilitative project, which was based on selective treatment of both the spatial cognition and
constructive apraxia disorders. Nevertheless, we
were not able to clarify whether the recovery of
spatial cognition positively influenced the
improvement of motor aspects of AHS. It is tempt-

1. Smith Doody R, Jankovic J. The alien hand and related
signs. J Neurol Neurosurg Psychiatry 1992;55:806-10.
2. Groom KN, Ng WK, Kevortin CG, Levy JK. Ego syntonic
alien hand syndrome after right posterior cerebral artery
stroke. Arch Phys Med Rehabil 1999;80(2):162-5.
3. Suwanwela NC, Leelacheavasit N. Isolated corpus callosal
infarction secondary to pericallosal artery disease presenting as alien hand syndrome. J Neurol Neurosurg Psychiatry
2002;72(4):533-6.
4. Chan JL, Liu AB. Anatomical correlates of alien hand syndromes. Neuropsychiatr Neuropsychol Behav Neurol
1999;12(3):149-55.
5. Bundick LT Jr, Spinella M. Subjective experience, involuntary movement, and posterior alien hand syndrome. J
Neurol Neurosurg Psychiatry 2000:68(1):83-5.
6. Goldstein K. Zur Lehre von der motorischen Apraxie. J
Psychol Neurol 1908;11:169-86.
7. B r i o n S , J e d y n a k C P. Tr o u b l e s d e t r a n s f e r t
interhemispherique (callosal disconnection). A propos de
trois observations de tumeurs du corps calleux. Le sign de la
main etrangère. Rev Neurol 1972;126:257-66.
8. Goldberg G, Mayer NH, Toglia JU. Medial frontal cortex
infarction and the alien hand sign. Ar ch Neur ol
1981;38:683-6.
9. Wu FY, Leong CP, Su TL. Alien hand syndrome: report of
two cases. Changgeng Yi Xue Za Zhi 1999;22(4):660-5.
10. Persaud RS. Sensory alien hand syndrome. J Neurol
Neurosurg Psychiatry 1999;67(1):130-1.
11. Marey-Lopez J, Rubio-Nazabal E, Alonso-Magdalena L,
Lopez-Facal S. Posterior alien hand syndrome after a right
t ha l a m i c i nf a r c t . J N eur o l N eur o sur g P sy chi a t r y
2002;73(4):447-9.
12. Gibb WR, Luthert PJ, Marsden CD. Cortcobasal degeneration. Brain 1989;112:1171-92.
13. Keith RA, Granger CV, Hamilton BB, Sherwin FS. The functional independence measure: a new tool for rehabilitation.
Adv Clin Rehabil 1987;1:6-18.
14. Marchetti C, Della Sala S. Disentangling the alien and anarchic hand. Cognit Neuropsychiatr 1998;3:191-207.
15. Linacre JW, Heinemann AW, Wright BD, Granger C, Hamilton BB. The structure and stability of the functional independence measure. Arch Phys Med Rehabil 1994;75:127-32.

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ACKNOWLEDGMENTS
The authors wish to thank Prof. Arturo Reggio
and Prof. Francesco Le Pira for the precious
collaboration.

REFERENCES

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Posterior Alien Hand Syndrome
16. Pfefferbaum A, Ford JM, Wenegrat BG, Roth WT, Kopell BS.
Clinical applications of the P-300 component of eventrelated potentials. Norm Aging Electroencephalogr Clin
Neurophysiol 1984:59:85-103.
17. Angelici R, Correra G, Calice C, D’Aurria V, Grossi D. La
Terapia Razionale dei Disordini Costruttivi. Centro di
Riabilitazione Neuromotoria S. Lucia – Sezione Ricerca “i
quaderni”, n° 10, appendice 2, Roma 1991.
18. Nudo RJ, Friel KM. Cortical plasticity after stroke: implications for rehabilitation. Rev Neurol (Paris) 1999;155(9):71317.
19. Hallett M. Plasticity of the human motor cortex and recovery from stroke. Brain Res Rev 2001;36(2-3):169-74.
20. Levine DN, Rinn WE. Opticosensory ataxia and alien hand
syndrome after posterior cerebral artery territory infarction.
Neurology 1986;36:1094-7.
21. Doody RS, Jancovic J. The alien hand and related signs. J
Neurol Neurosurg Psychiatry 1992;55:806-10.

22. Ay H, Buonanno FS, Price BH, Le DA, koroshetz WJ. Sensory alien hand syndrome: case report and review of the literature. J Neurol Neurosurg Psychiatry 1998;65(3):366-9.
23. Pack BC, Stewart KJ, Diamond PT, Gale SD. Posterior-variant
alien hand syndrome: clinical features and response to rehabilitation. Disabil Rehabil 2002;24(15):817-8.
24. Rohde S, Weidauer S, Lanfermann H, Zanella F. Posterior
alien hand syndrome: case report. Neuroradiology
2002;44(11):921-3.
25. Chan JL, Ross ED. Alien hand syndrome: influence of
neglect on the clinical presentation of frontal and callosal
variants. Cortex 1997;33(2):287-99.
26. Aboitiz E, Carrasco X, Schroter C, Zaidel D, Zaidel E,
Lavados M. The alien hand syndrome: classification, of
forms reported and discussion of a new condition. Neurol
Sci 2003;24:252-7.

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