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533

SHORT REPORT

Isolated corpus callosal infarction secondary to
pericallosal artery disease presenting as alien hand
syndrome
N C Suwanwela, N Leelacheavasit
.............................................................................................................................

J Neurol Neurosurg Psychiatry 2002;72:533–536

Two patients are described with the callosal type of alien
hand syndrome. Both presented with abnormal feelings in
the left upper limb and intermanual conflict without clinical
evidence of callosal apraxia or frontal lobe dysfunction
such as motor deficit or reflexive grasping. Imaging studies
disclosed subacute infarction in the body and splenium of
the corpus callosum due to pericallosal artery disease.
These patients were unique in their presentation as a
callosal type of alien hand syndrome secondary to ischaemic stroke.

A

lien hand syndrome is a fascinating but controversial
clinical entity. Brion and Jedynak, in 1972, first
described three patients with tumour of the corpus callosum who failed to recognise ownership of one hand when it
was placed into the other hand behind the back and
introduced the concept of the alien hand (la main
étrangere).1 Subsequently, the term has been applied to a
motor phenomena of apparently purposeful actions of one
hand against the patient’s intention.2 Perception disturbance,
which makes this syndrome unique, is always verbally
expressed, even expression of astonishment at the autonomous activities of the alien hand may occur. Neuroimaging
and pathological studies demonstrated that the frontal lobe
and corpus callosum are the most common anatomical lesions
responsiible for this distinctive phenomenon.3 4 Here, we
describe the clinical, neuroanatomical, and radiological
findings of two patients who presented with the callosal type
of alien hand syndrome. The isolated manifestation of
intermanual conflict without evidence of callosal apraxia and
forced grasping makes our patients substantially different
from those previously described. On neuroradiological studies,
both patients had infarction restricted to the corpus callosum
secondary to stenosis of the supracallosal segment of the pericallosal artery.

CASE REPORTS
Patient 1
This patient was a 54 year old right handed Thai man who
presented with abnormal feelings in his arms. His medical
history included diabetes mellitus and hypertension which
had been poorly controlled. The symptoms had started while
he was sitting, when he suddenly experienced transient
weakness of the lower limbs, which lasted for only 5 minutes.
Ten hours later, he noticed some strange feelings in his arms.
For example, when he crossed his arms behind his back, he
felt as if the right hand was touched by a foreign hand. The
feeling disappeared when he placed both hands in the front.
When he reached into his pocket with his left hand, he felt as
if there was another, non-existing, hand touching it. When he

held a paper with both hands, the left hand would try to pull
the paper against the right. Some actions indicating mirror
movement were also seen. For example, when he moved his
right hand backwards, he felt that the left hand was pulled
back in the same manner. On examination, he was alert.
Motor power of the arms and legs was full. There was no pinprick sensory loss or inattention, on double simultaneous
stimulation test. Proprioceptive sense was normal. He could
not identify his left hand fingers or objects placed in his left
hand with his eyes closed, but was able to do so under visual
observation. There was no apraxia of the left hand on verbal
command, in imitation, and in actual object use. Frontal lobe
releasing signs such as reflexive grasping, palmomental reflex,
and snout reflex were absent.
Brain MRI on admission showed lesions with hyposignal
intensity on T1 weighted and hypersignal intensity on T2
weighted and FLAIR images in the body and splenium of the
corpus callosum. There was no associated frontal lobe lesion.
The diffusion weighted study showed increased signal
intensity of the involved area (fig 1 A and B). Magnetic resonance angiography of the cerebral vessels showed a variation
of the anterior cerebral artery, with only a single trunk of
pericallosal branches. Stenosis and irregularity of the supracallosal segment of the right pericallosal artery which could
have been responsible for the callosal infarction was also
demonstrated (fig 1 C).
Case 2
This patient was a 50 year old right handed Thai woman with
a history of diabetes mellitus, dyslipidaemia, and coronary
artery disease. She was admitted for coronary artery bypass
grafting. The operation went uneventfully without any immediate complications. On the 7th day postoperatively, she was
noted by nursing staff to have mild slurring of speech. She also
complained of loss of bimanual coordination. For example, she
could not tie a rope by herself as the left hand would not help
the right and at times even tried to inhibit the action. When
she held a piece of paper with both hands, each hand would
pull against each other. She also reported that the left hand
would not release the telephone when she wanted to use it
with her right hand. The intermanual conflict was so
pronounced that she felt as if the left hand was under someone else’s control. On neurological examination, she was alert
and oriented. There was only mildly dysarthric speech. No
evidence of motor aphasia was demonstrated. Hypaesthesia of
the left side of her body and face was also noted. The motor
power and tone were normal. She was able to release objects
held in the left hand when asked by the examiner. Forced
grasping and other frontal release signs were absent. Apraxia
was absent, both on verbal command and patomime of object
use. There was no tactile agnosia, agraphia, or alexia. Stereognosis was normal. Other general physical examination was
within normal limits. Regarding the aetiology of stroke, there

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534

Figure 1 (A and B) Axial diffusion weighted images of patient 1
show a high signal intensity lesion compatible with acute infarction
in the body and splenium of the corpus callosum. (C) Magnetic
resonance angiography shows irregularity and narrowing of the
supracallosal branch of the pericallosal artery (arrows).

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Suwanwela, Leelacheavasit

Figure 2 (A) Axial FLAIR image of patient 2 demonstrates a high
signal intensity lesion in the body of the corpus callosum and multiple
small lesions in the subcortical white matter. (B) Diffusion image shows
high signal intensity lesion in the body of the corpus callosum indicating
acute infarction. The multiple old lacunar infarctions in the frontal and
parietal subcortical white matter seen in FLAIR are not demonstrated.
(C) Magnetic resonance angiography demonstrates multiple sites of
atherosclerotic narrowing of the intracranial vessels; both cavernous
part of the internal carotid arteries and both middle cerebral arteries.
Narrowing of the right pericallosal artery is also shown (arrow).

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Callosal alien hand syndrome secondary to pericallosal artery disease

535

was no atrial fibrillation. An ECG showed a moderate aortic
regurgitation without intracardiac clot. All of the neurological
symptoms disappeared within 48 hours.
Brain MRI showed a region of low signal intensity in the
right side of the body of the corpus callosum on the T1
weighted image and high signal intensity in the same area on
T2 weighted and FLAIR images (fig 2 A). Diffusion weighted
images also showed increased signal intensity at the same
region (fig 2 B). Evidence of old lacunar infarction in the
frontal and parietal subcortical white matter was noted in the
T2 weighted and FLAIR images but not on the diffusion studies. There was no evidence of a frontal lobe lesion. Magnetic
resonance angiography showed an absence of the A1 segment
of the right anterior cerebral artery. Atherosclerotic narrowing
of the cavernous portion of both internal carotid arteries and
the proximal part of both middle cerebral arteries were noted.
There was a significant stenosis of the supracallosal segment
of the right pericallosal artery (fig 2 C).

tion is responsible for less than 3% of all ischaemic strokes,15
and alien hand syndrome is considered to be an uncommon
manifestation.15 Among reported patients, the alien hand syndrome after anterior cerebral artery infarction is clinically
frontal type with prominent reflexive grasping of the
dominant hand and motor deficits suggesting frontal lobe
dysfunction.7 13–17 The responsible anatomical lesions are
generally located in the corpus callosum and the neighbouring
frontal lobe especially in the supplementary motor area.
According to Giroud and Dumas, alien hand syndrome was
found in only two out of eight patients with callosal infarction
and both of these had hemiparesis and forced grasping which
is characteristic of frontal alien hand syndrome.15 Our two
patients each displayed the characteristic pattern of callosal
alien hand syndrome. Their symptoms appeared in the
non-dominant upper limbs and there was no obvious motor
deficit or reflexive grasping.
These patients are unique in their manifestations and the
imaging findings. Both demonstrated isolated corpus callosal
lesions without evidence of cortical involvement on MRI during the acute phase. However, they initially displayed
symptoms consistent with frontal lobe dysfunction. The first
patient reported a transient weakness of both lower limbs
whereas the second case had mild dysarthria. Therefore, it is
possible that these patients sustained some degree of cortical
damage in addition to the callosal lesion. Temporary ischaemia
of the frontal lobe resulting in limited area of residual infarction on MRI may explain this phenomena. We were able to
show the responsible vascular lesions at the pericallosal
branch of anterior cerebral artery by magnetic resonance
angiography. To our knowledge, there have been no previous
reports documenting pericallosal artery disease as a cause of
alien hand syndrome. In the first patient, variation of the
pericallosal artery as a single trunk supplying both sides of the
corpus callosum causing bilateral infarction was shown on
MRI.18 The second patient had lesions in the right pericallosal
artery resulting in infarction of the right side of the corpus
callosum.

DISCUSSION
The term “alien hand syndrome” has been used to describe
two distinctive phenomena. Originally, it was proposed by
Brion and Jedynac as a sensory phenomenon characterised
by the denial of ownership of one hand despite no sensory
loss when it was held by the other hand behind the back,
secondary to lesions of the parietal lobe and posterior part of
corpus callosum.1 Subsequently, the same term has become
more recognised as a psychomotor disorder characterised by
the patient’s perception of alienation coexisting with observable involuntary, but seemingly purposeful, movement of the
affected limb.5 6 Motor phenomena of the alien hand
syndrome vary from simple to complex and apparently
purposeful movements. Recently, the term “anarchic hand”
has been proposed to distinguish the motor phenomena of
alien hand syndrome from the original description of sensory
misperception.6 Both patients in this report presented with
a predominantly motor type of alien hand syndrome.
However, an associated sensory phenomenon similar to that
originally described by Brion and Jedynak was shown in the
first case.1
Two types of predominantly motor alien hand syndrome
were proposed by Della Sala et al in 1991.7 The first type is an
acute or transient form due to callosal lesions. The other more
common form is a chronic condition resulting from both callosal and additional mesial frontal lesions. Feinberg et al
reviewed the clinical characteristics and neuroanatomy of 20
reported cases of alien hand syndrome and categorised the
alien hand syndrome into two distinct syndromes.8 The “frontal alien hand syndrome” results from damage to the supplementary motor area, anterior cingulate gyrus, medial prefrontal cortex of the dominant hemisphere, and anterior corpus
callosum. The symptoms of frontal alien hand syndrome
always occur in the dominant hand, with prominent motor
phenomena including reflexive grasping, groping, and compulsive manipulation of tools. The second type or “callosal
alien hand syndrome” requires only a callosal lesion and is
characterised primarily by intermanual conflict and the nondominant hand is usually affected. Clinically, our patients fulfilled the criteria for the callosal type of alien hand syndrome.
The feeling of alienation, including intermanual conflict and
mirror movement, was found in the left upper limb in both
cases. Neuroimaging studies confirmed the responsible lesions
for the interhemispheric motor disconnection syndrome in
the corpus callosum. However, both patients did not manifest
the profound callosal apraxia which has been previously
reported with lesions in the body of the corpus callosum.6 8
Many medical and surgical conditions can cause alien hand
syndrome.9–17 For patients with alien hand syndrome secondary to ischaemic stroke, the anterior cerebral artery is almost
always the affected vessel.7 13–17 Anterior cerebral artery infarc-

.....................

Authors’ affiliations
N C Suwanwela, N Leelacheavasit, Neurological Unit, Department of
Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok
10330, Thailand
Correspondence to: Dr N C Suwanwela, Neurological Unit, Department
of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok
10330, Thailand; fmednsu@md2.md.chula.ac.th
Received 19 June 2001
In revised form 17 September 2001
Accepted 5 November 2001

REFERENCES
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disconnection). A propos de trois observation de tumeurs du corps
calleux. Le signe de la main étrangere. Rev Neurol (Paris)
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2 Goldberg G, Mayer N, Toglia JU. Medial frontal cortex infarction and
the alien hand sign. Arch Neurol 1981;38:683–6.
3 Banks G, Short P, Martinez J, et al. The alien hand syndrome: clinical
and post mortem findings. Arch Neurol 1989;46:456–9.
4 Benson DF, Geschwind N. The aphasias and related disturbances. In:
Baker AB, Joynt RJ, ed. Clinical neurology. Philadelphia:Harper and Row
1985:1–85.
5 Doody RS, Jankovic J. The alien hand and related signs. J Neurol
Neurosurg Psychiatry 1992;55:806–10.
6 Geschwind DH, Iacoboni M, Mega MS, et al. Alien hand syndrome:
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the corpus callosum. Neurology 1995;45:802–8.
7 Della Sala S, Marchetti C, Spinner H. Right sided anarchic (alien) hand:
a longtitudinal study. Neuropsychologia 1991;29:1113–27.
8 Feinberg TE, Schindler RJ, Flanakan NG, et al. Two alien hand
syndromes. Neurology 1992: 42:19–24.

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9 Jeeves MA, Simpson DA, Geffen G. Functional consequences of the
transcallosal removal of intraventricular tumors. J Neurol Neurosurg
Pshychiatry 1979;42:134–42.
10 Leiguarda R, Starkstein S, Bethier M. Anterior callosal hemorrhage: a
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12 Gibb WRG, Luther PJ, Marsden CD. Corticobasal degeneration. Brain
1989;112:1171–92.
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Suwanwela, Leelacheavasit
14 Chan JL, Ross ED. Left hand mirror writing following right anterior
cerebral artery infarction: evidence for non-mirror transformation of motor
programs by right supplementary motor area. Neurology
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15 Giroud M. Dumas R. Clinical and topographical range of callosal
infarction: a clinical and radiological correlation study. J Neurol
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NEUROLOGY IN LITERATURE ........................................................................
A RAYMOND CHANDLER TRIO
The big sleep: Hamish Hamilton 1939
Her hand holding the empty gun began to shake violently. The gun fell
out of it. Her mouth began to shake. Her whole face went to pieces.
Then her head screwed up towards her left ear and froth showed on
her lips. Her breath made a whining sound. She swayed.

Farewell my lovely: Hamish Hamilton 1940
But naturally there are certain elements which would like to show me
in a bad light. Psychiatrists, sex specialists, neurologists and nasty
little men with rubber hammers and shelves loaded with the literature
of aberrations.

The high window: Hamish Hamilton 1943
Her mouth was in a tight line at the corners, but the middle part of her
upper lip kept lifting off her teeth, upwards and outwards as if fine
threads attached to the edge of the lip were pulling it. It would go up
so far that it didn’t seem possible, and then the entire lower part of her
face would go into a spasm and when the spasm was over her mouth
would be tight shut, and then the process would slowly start all over
again. In addition to this there was something wrong with her neck.
So that very slowly her head was drawn around to the left about
forty-five degrees. It would stop there, her neck would twitch, and her
head would slide back the way it had come.

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Curiously Carmen Sternwood’s epilepsy as described in The big sleep
does not appear in the film starring Humphrey Bogart and Lauren
Bacall. Nor indeed does the recent remake of Farewell my lovely refer to
the rather deprecatory remarks made by Jules Anthor in that book
regarding neurologists. Jules Anthor is described as a psychic consultant. Reading the book one has to say he seems to be more troubled by
aberrations than most neurologists that I have met.
The description of Merle Davis’ movement disorder appears in The
high window. Reading between the lines one is probably meant to infer
that the movements were due to psychological stress. The description
of the facial component of her movement disorder is intriguing and
one that I perhaps would not immediately identify. It does not really
fit with the clinical features of Meige’s syndrome. The neck
movements are more straightforward in suggesting a spasmodic torticollis. The book doesn’t indicate the eventual outcome of the problem
although the psychological issues are probably eventually settled.
G D Perkin
Imperial College School of Medicine, Charing Cross hospital, Fulham Palace
Road, London W6 8RF, UK

Downloaded from http://jnnp.bmj.com/ on May 28, 2015 - Published by group.bmj.com

Isolated corpus callosal infarction secondary to
pericallosal artery disease presenting as alien
hand syndrome
N C Suwanwela and N Leelacheavasit
J Neurol Neurosurg Psychiatry 2002 72: 533-536

doi: 10.1136/jnnp.72.4.533
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