Case Report

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 , Lujiang County Hospital of TCM, Anhui, PR China.

A senile Chinese female patient with gion involving the cortex and adjacent white matter. Follow-up MRI in a week post-onset showed chronic ischemic lesion in the right tem- of vascular etiology is reported. This case exhibited contra- poral-parietal-occipital gray and white matter, but callosal involve- dictive movement, left-lateral paresis and disorder of color ment was not seen. She complained often that her left arm “doesn’t discrimination, which might be a new subtype of the alien do what its supposed to do. It always is controlled by a ghost or god.” limb syndrome. The nature of the involuntary movement in the left arm was prima- Key Words: Alien hand syndrome, Involuntary movement, rily uncontrolled levitation with intermittent writhing of her fingers. No exploratory or self-stimulating behavior (grasping skin, cloth- Cerebrovascular diseases, Disorder of color discrimination. 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 Introduction drug abuse. Neurological examination revealed that her tongue was turned to- Alien limb syndrome or alien limb phenomenon is summa- wards the left. There were no . The tendon reflexes in all the four limbs were exaggerated. The patient showed right-left disorien- rized as: the patient complains that an arm or leg has a ‘life of tation and did not exhibit grasp reflex or apraxic symptoms in either its own’ and is almost always asymmetrical; on examination, upper limb. The revised Hasegawa’s dementia scale showed her score the limb may move and even grasp objects involuntarily; best as 9, indicating that her intelligence was below normal. The routine assessed by distracting the patient with some other task and serum biochemical tests were normal. Her spontaneous verbal pro- observing the limb; often seen in corticobasal degeneration duction was nearly normal in rate and frequency of utterances, but (CBD).1-3 It is related with a cluster of symptoms character- she had mild dysarthria. She exhibited pronounced left visual ne- ized by the involuntary movement of a single upper limb in glect on line bisection and target cancellation tasks. conjunction with the experience of estrangement from or per- and kinaesthesia were both severely impaired bilaterally, worse in the left than the right upper limb. Tactile sensory and proprioceptive sonification of the movements of the limb itself. However, a impairment prevented meaningful testing of the patient’s ability to reliable, anatomically derived definition of the alien hand syn- differentiate between her affected hand and the examiner’s hand drome has been elusive. A reason for this broadened accept- when held out of sight. Stereognosis and tactile object naming were able application has been the identification of theoretically not possible with the left hand, and were impaired with the right. dissociable subtypes.4 We present a case of a specific variety After 12 weeks of rehabilitation and the preventive management or subtype of the alien hand syndrome, and discuss its etiology of cerebral vascular risks and a small dose of tiapride, the levitation and possible anatomical reasoning. and complaints of avolitional movement had reduced considerably in frequency, though visual neglect, slight lower limb weakness, and Case Report hemianesthesia remained. Throughout, the patient seemed perplexed rather than angered or depressed by her contradictive movement.

A 74-year-old right-handed woman suddenly developed left-sided Discussion 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 The alien hand syndrome was originally used to describe hemi-inattention was also noted. Initial brain CT without contrast cases involving the anterior part of corpus callosal lesions (split- showed probable infarct in the right temporal-parietal-occipital re- brain person) producing involuntary or contradictive move-

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]

Neurology India March 2004 Vol 52 Issue 1 109 CMYK 109 Wang XP, et al: Alien limb syndrome ments and a concomitant inability to distinguish the affected of this type may occur with improvement of either the invol- hand from an examiner’s hand when these were placed in the untary movements, alterations in the body schema, or both. patient’s unaffected hand.1 Some varieties or subtypes of the Our patient’s involuntary movements had largely resolved on alien hand syndrome have been reported, involving lesions of her discharge, whereas her alterations in body schema (visual the alone, the corpus callosum and the domi- neglect, hemianesthesia, and proprioceptive impairment) re- nant medial frontal cortex, and the temporal or/and parietal mained. It may be reasonable to expect that, given this con- cortical/sub-cortical areas, and rarely, non-dominant hemi- text, any future occurrence of sustained involuntary move- sphere thalamic infarcts.5-7 The pathogenic chart of the alien ments could produce a recurrence of the alien hand syndrome, hand syndrome has often been found to contain CBD or vice versa. mainly,3,5,8 it has also been reported in the acute cerebral vas- At present, there seem to be some broad clusters of behavioral cular diseases,4,9 herpes viral encephalitis(Avrahami-Heller), and subjective symptoms subsumed under the diagnosis “al- Alzheimer’s disease, progressive supranuclear palsy,10 ien hand syndrome”. This patient and other examples sug- epilepsies11 and Creutzfeldt –Jacob’s disease,12 which have been gest that the alien hand syndrome may not only be as closely seen in Europe, North and South America, and Asia. associated with focal, critical lesion sites as its callosal and Given this patient’s involuntary movements and her verbal callosal-frontal counterparts, but also with the temporal-pa- expressions of perplexity and estrangement from the move- rietal-occipital region as well as with the single temporal or ments, she clearly manifested the alien hand syndrome as single parietal region in the dominant . defined in recent studies. She did not, however, exhibit the Rather, it seems to be a disorder of involuntary movement in mutism, apathy, exploratory behavior, groping, compulsive the context of alterations in body schema sufficient to cause manipulation of objects and tools, or grasp reflex reported in feelings of estrangement from those movements. These symp- callosal-frontal cases.1 She likewise exhibited the intermanual toms may occur independently of one another and may occur conflict seen in callosal cases and exhibited no signs of cal- from either single or multiple lesions. The alien hand syn- losal disconnection. Despite the right temporal-parietal-oc- drome, compared to “frozen feet”, a kind of contradictive phe- cipital region abnormalities on MRI, this patient was similar nomenon seen often in Parkinsonism, may also be a symptom to the reported cases of the alien hand syndrome associated of movement disorder so attention should be drawn to basal with posterior lesions. Such cases have involved multiple loci ganglia dysfunction. of cerebral dysfunction (e.g., cortical and subcortical) caused by single or multiple infarcts, which perhaps independently, References produced the subjective and behavioral symptoms of the alien hand syndrome. Two previously reported cases of the alien 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. hand syndrome involved non-dominant hemisphere thalamic 2. Aleem MA. Paroxysmal alien hand syndrome. J Assoc Physic Ind 2000;48:1035-6. infarcts in conjunction with additional cortical, sensory im- 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 pairment. Cases of posterior alien hand syndrome arising from posterior alien hand syndrome. J Neurol Neurosurg Psychiatr 2000;68:83-5. corticobasal degeneration may be similarly multi-determinate. 5. Carrilho PE, Caramelli P, Cardoso F, Barbosa ER, Buchpiguel CA, Nitrini R. Involuntary hand levitation associated with parietal damage: another alien hand What we should mention is the symptom of the disorder of syndrome. Arq Neuropsiquiatr 2001;59:521-5. color discrimination—our patient sighted gray as five-six 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 colors, which suggested considerable injuries in her occipital 2000;10:70-2. region and a possible new subtype of alien hand syndrome. 7. Kischka U, Ettlin TM, Lichtenstern L, Riedo C. Alien hand syndrome of the dominant hand and ideomotor of the nondominant hand. Eur Neurol We speculate that our patient’s feelings of estrangement from 1996;36:39-42. her non-dominant upper limb and its movements were pro- 8. Kompoliti K, Goetz CG, Boeve BF, Maraganore DM, Ahlskog JE, Marsden CD, et al. Clinical presentation and pharmacological therapy in corticobasal duced by body schema distortion and hemineglect secondary degeneration. Arch Neurol 1998;55:957-61. to the non-dominant, the right temporal-parietal-occipital re- 9. Ong Hai BG, Odderson IR. Involuntary masturbation as a manifestation of -related alien hand syndrome. J Phys Med Rehabil 2000;79:395-8. gion lobe infarct. The fine etiology of her involuntary move- 10. Gunal DI, Agan K, Aktan S. A case of spontaneous arm levitation in progres- ment, however, is not as clear. Another possibility is of basal sive supranuclear palsy. Neurol Sci 2000;21:405-6. 11. Frattalic M, Grafman J, Patronas N, Makhlouf F, Litvan I. Language distur - ganglia dysfunction. That the involuntary movements are not bances in corticobasal degeneration. Neurology. 2000;54:990-2. specifically associated with any particular, theoretically criti- 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. cal neuropathology represents a fundamental difference be- Neurologia 2001;16:223-6. tween posterior alien hand syndrome and the callosal and cal- losal-frontal varieties. Remission of the alien hand syndrome Accepted on 01.10.2002.

110 Neurology India March 2004 Vol 52 Issue 1 110 CMYK