Alien hand syndrome

Neurology India March 2004 Vol 52 Issue 1 109
CMYK 109
Case Report
Xiao-Ping Wang
Dr. Laboratory of Neuro-degenerative diseases, School of Life Science University of Science & Technology of China, Hefei, PR China. 230026.
E-mail: wangxp@mail.hf.ah.cn
Alien hand syndrome: Contradictive movement and disorder of
color discrimination
X. P. Wang,*,** C. B. Fan,*** J. N. Zhou*
*Laboratory of Neuro-degenerative diseases, College of Life Science, University of Science & technology of China, Hefei, PR China;
**Department of Psychology, University of Montreal, Qc. Canada; ***Department of Neurology, Lujiang County Hospital of TCM, Anhui, PR
China.
A senile Chinese female patient with alien hand syndrome
of vascular etiology is reported. This case exhibited contradictive
movement, left-lateral paresis and disorder of color
discrimination, which might be a new subtype of the alien
limb syndrome.
Key Words: Alien hand syndrome, Involuntary movement,
Cerebrovascular diseases, Disorder of color discrimination.
Introduction
Alien limb syndrome or alien limb phenomenon is summarized
as: the patient complains that an arm or leg has a ‘life of
its own’ and is almost always asymmetrical; on examination,
the limb may move and even grasp objects involuntarily; best
assessed by distracting the patient with some other task and
observing the limb; often seen in corticobasal degeneration
(CBD).1-3 It is related with a cluster of symptoms characterized
by the involuntary movement of a single upper limb in
conjunction with the experience of estrangement from or personification
of the movements of the limb itself. However, a
reliable, anatomically derived definition of the alien hand syndrome
has been elusive. A reason for this broadened acceptable
application has been the identification of theoretically
dissociable subtypes.4 We present a case of a specific variety
or subtype of the alien hand syndrome, and discuss its etiology
and possible anatomical reasoning.
Case Report
A 74-year-old right-handed woman suddenly developed left-sided
slight weakness, feeling as if the “left hand was controlled by a ghost”
and sighted a gray wall as having five-six colors on its surface. Nine
weeks after this attack, she became dysphagic and dysarthric. Left
hemi-inattention was also noted. Initial brain CT without contrast
showed probable infarct in the right temporal-parietal-occipital region
involving the cortex and adjacent white matter. Follow-up MRI
in a week post-onset showed chronic ischemic lesion in the right temporal-
parietal-occipital gray and white matter, but callosal involvement
was not seen. She complained often that her left arm “doesn’t
do what its supposed to do. It always is controlled by a ghost or god.”
The nature of the involuntary movement in the left arm was primarily
uncontrolled levitation with intermittent writhing of her fingers.
No exploratory or self-stimulating behavior (grasping skin, clothing)
was ever noted to occur, and she often restrained her left arm
with her right hand. She could not perform bimanual tasks. The
patient had no formal education, had no positive personal or family
history or any mental disorder. There was no history of alcohol or
drug abuse.
Neurological examination revealed that her tongue was turned towards
the left. There were no tremors. The tendon reflexes in all the
four limbs were exaggerated. The patient showed right-left disorientation
and did not exhibit grasp reflex or apraxic symptoms in either
upper limb. The revised Hasegawa’s dementia scale showed her score
as 9, indicating that her intelligence was below normal. The routine
serum biochemical tests were normal. Her spontaneous verbal production
was nearly normal in rate and frequency of utterances, but
she had mild dysarthria. She exhibited pronounced left visual neglect
on line bisection and target cancellation tasks. Proprioception
and kinaesthesia were both severely impaired bilaterally, worse in
the left than the right upper limb. Tactile sensory and proprioceptive
impairment prevented meaningful testing of the patient’s ability to
differentiate between her affected hand and the examiner’s hand
when held out of sight. Stereognosis and tactile object naming were
not possible with the left hand, and were impaired with the right.
After 12 weeks of rehabilitation and the preventive management
of cerebral vascular risks and a small dose of tiapride, the levitation
and complaints of avolitional movement had reduced considerably in
frequency, though visual neglect, slight lower limb weakness, and
hemianesthesia remained. Throughout, the patient seemed perplexed
rather than angered or depressed by her contradictive movement.
Discussion
The alien hand syndrome was originally used to describe
cases involving the anterior part of corpus callosal lesions (splitbrain
person) producing involuntary or contradictive move110
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110 CMYK
ments and a concomitant inability to distinguish the affected
hand from an examiner’s hand when these were placed in the
patient’s unaffected hand.1 Some varieties or subtypes of the
alien hand syndrome have been reported, involving lesions of
the corpus callosum alone, the corpus callosum and the dominant
medial frontal cortex, and the temporal or/and parietal
cortical/sub-cortical areas, and rarely, non-dominant hemisphere
thalamic infarcts.5-7 The pathogenic chart of the alien
hand syndrome has often been found to contain CBD
mainly,3,5,8 it has also been reported in the acute cerebral vascular
diseases,4,9 herpes viral encephalitis(Avrahami-Heller),
Alzheimer’s disease, progressive supranuclear palsy,10
epilepsies11 and Creutzfeldt –Jacob’s disease,12 which have been
seen in Europe, North and South America, and Asia.
Given this patient’s involuntary movements and her verbal
expressions of perplexity and estrangement from the movements,
she clearly manifested the alien hand syndrome as
defined in recent studies. She did not, however, exhibit the
mutism, apathy, exploratory behavior, groping, compulsive
manipulation of objects and tools, or grasp reflex reported in
callosal-frontal cases.1 She likewise exhibited the intermanual
conflict seen in callosal cases and exhibited no signs of callosal
disconnection. Despite the right temporal-parietal-occipital
region abnormalities on MRI, this patient was similar
to the reported cases of the alien hand syndrome associated
with posterior lesions. Such cases have involved multiple loci
of cerebral dysfunction (e.g., cortical and subcortical) caused
by single or multiple infarcts, which perhaps independently,
produced the subjective and behavioral symptoms of the alien
hand syndrome. Two previously reported cases of the alien
hand syndrome involved non-dominant hemisphere thalamic
infarcts in conjunction with additional cortical, sensory impairment.
Cases of posterior alien hand syndrome arising from
corticobasal degeneration may be similarly multi-determinate.
What we should mention is the symptom of the disorder of
color discrimination—our patient sighted gray as five-six
colors, which suggested considerable injuries in her occipital
region and a possible new subtype of alien hand syndrome.
We speculate that our patient’s feelings of estrangement from
her non-dominant upper limb and its movements were produced
by body schema distortion and hemineglect secondary
to the non-dominant, the right temporal-parietal-occipital region
lobe infarct. The fine etiology of her involuntary movement,
however, is not as clear. Another possibility is of basal
ganglia dysfunction. That the involuntary movements are not
specifically associated with any particular, theoretically critical
neuropathology represents a fundamental difference between
posterior alien hand syndrome and the callosal and callosal-
frontal varieties. Remission of the alien hand syndrome
of this type may occur with improvement of either the involuntary
movements, alterations in the body schema, or both.
Our patient’s involuntary movements had largely resolved on
her discharge, whereas her alterations in body schema (visual
neglect, hemianesthesia, and proprioceptive impairment) remained.
It may be reasonable to expect that, given this context,
any future occurrence of sustained involuntary movements
could produce a recurrence of the alien hand syndrome,
or vice versa.
At present, there seem to be some broad clusters of behavioral
and subjective symptoms subsumed under the diagnosis “alien
hand syndrome”. This patient and other examples suggest
that the alien hand syndrome may not only be as closely
associated with focal, critical lesion sites as its callosal and
callosal-frontal counterparts, but also with the temporal-parietal-
occipital region as well as with the single temporal or
single parietal region in the dominant cerebral hemisphere.
Rather, it seems to be a disorder of involuntary movement in
the context of alterations in body schema sufficient to cause
feelings of estrangement from those movements. These symptoms
may occur independently of one another and may occur
from either single or multiple lesions. The alien hand syndrome,
compared to “frozen feet”, a kind of contradictive phenomenon
seen often in Parkinsonism, may also be a symptom
of movement disorder so attention should be drawn to basal
ganglia dysfunction.
References
1. Bakchine S, Slachevsky A, Tourbah A, Serres I, Abdelmounni H. Four “alien” hands
for two hands after a lesion in corpus callosum. Rev Neurol 1999;155:929-34.
2. Aleem MA. Paroxysmal alien hand syndrome. J Assoc Physic Ind 2000;48:1035-6.
3. Wang XP. Corticobasal degeneration. Chin J Pract Inter Med 2000;20:755-7.
4. Bundick T Jr Spinella M. Subjective experience, involuntary movement, and
posterior alien hand syndrome. J Neurol Neurosurg Psychiatr 2000;68:83-5.
5. Carrilho PE, Caramelli P, Cardoso F, Barbosa ER, Buchpiguel CA, Nitrini R.
Involuntary hand levitation associated with parietal damage: another alien hand
syndrome. Arq Neuropsiquiatr 2001;59:521-5.
6. Marti-Fabregas J, Kulisevsky J, Baro E, Mendoza G, Valencia C, Marti-Vilalta
JL. Alien hand sign after a right parietal infarction. Cerebrovasc Dis
2000;10:70-2.
7. Kischka U, Ettlin TM, Lichtenstern L, Riedo C. Alien hand syndrome of the
dominant hand and ideomotor apraxia of the nondominant hand. Eur Neurol
1996;36:39-42.
8. Kompoliti K, Goetz CG, Boeve BF, Maraganore DM, Ahlskog JE, Marsden
CD, et al. Clinical presentation and pharmacological therapy in corticobasal
degeneration. Arch Neurol 1998;55:957-61.
9. Ong Hai BG, Odderson IR. Involuntary masturbation as a manifestation of
stroke-related alien hand syndrome. J Phys Med Rehabil 2000;79:395-8.
10. Gunal DI, Agan K, Aktan S. A case of spontaneous arm levitation in progressive
supranuclear palsy. Neurol Sci 2000;21:405-6.
11. Frattalic M, Grafman J, Patronas N, Makhlouf F, Litvan I. Language distur -
bances in corticobasal degeneration. Neurology. 2000;54:990-2.
12. Colomer Rubio E, Sanchez R oy R, Pareja Martinez A, Perla C, Villarroya T,
Cerda Nicolas M et al. Alien hand syndrome in Creutzfeldt- Jakob disease.
Neurologia 2001;16:223-6.
Accepted on 01.10.2002.
Wang XP, et al: Alien limb syndrome