Alien hand syndrome: Contradictive movement and disorder of color discrimination

NI_JanMar_04.pmdCMYK 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: [email protected]
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 contra- dictive 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 summa-
rized 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 character-
ized by the involuntary movement of a single upper limb in
conjunction with the experience of estrangement from or per-
sonification of the movements of the limb itself. However, a
reliable, anatomically derived definition of the alien hand syn-
drome has been elusive. A reason for this broadened accept-
able 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.
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 re-
gion involving the cortex and adjacent white matter. Follow-up MRI
in a week post-onset showed chronic ischemic lesion in the right tem-
poral-parietal-occipital gray and white matter, but callosal involve-
ment 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 prima-
rily uncontrolled levitation with intermittent writhing of her fingers.
No exploratory or self-stimulating behavior (grasping skin, cloth-
ing) 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 to-
wards the left. There were no tremors. The tendon reflexes in all the
four limbs were exaggerated. The patient showed right-left disorien-
tation 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 pro-
duction was nearly normal in rate and frequency of utterances, but
she had mild dysarthria. She exhibited pronounced left visual ne-
glect 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
cases involving the anterior part of corpus callosal lesions (split-
brain person) producing involuntary or contradictive move-
110 Neurology India March 2004 Vol 52 Issue 1
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 domi-
nant medial frontal cortex, and the temporal or/and parietal
cortical/sub-cortical areas, and rarely, non-dominant hemi-
sphere 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 vas-
cular 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 move-
ments, 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 cal-
losal disconnection. Despite the right temporal-parietal-oc-
cipital 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 im-
pairment. 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 pro-
duced by body schema distortion and hemineglect secondary
to the non-dominant, the right temporal-parietal-occipital re-
gion lobe infarct. The fine etiology of her involuntary move-
ment, however, is not as clear. Another possibility is of basal
ganglia dysfunction. That the involuntary movements are not
specifically associated with any particular, theoretically criti-
cal neuropathology represents a fundamental difference be-
tween posterior alien hand syndrome and the callosal and cal-
losal-frontal varieties. Remission of the alien hand syndrome
of this type may occur with improvement of either the invol-
untary 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) re-
mained. It may be reasonable to expect that, given this con-
text, any future occurrence of sustained involuntary move-
ments 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 “al-
ien hand syndrome”. This patient and other examples sug-
gest 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-pa-
rietal-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 symp-
toms may occur independently of one another and may occur
from either single or multiple lesions. The alien hand syn-
drome, compared to “frozen feet”, a kind of contradictive phe-
nomenon seen often in Parkinsonism, may also be a symptom
of movement disorder so attention should be drawn to basal
ganglia dysfunction.
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