80686ournal ofNeurology, Neurosurgery, and Psychiatry 1992;55:806-810 The alien hand and related signs J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.55.9.806 on 1 September 1992. Downloaded from Rachelle Smith Doody, Joseph Jankovic Abstract contralateral thalamic sensory lesion;1' and Alien limb sign includes failure to recog- bifrontal penetrating cerebral injury.8 nise ownership of one's limb when visual More recently, alien limbs have been des- cues are removed, a feeling that one body cribed in patients with proven and presumed part is foreign, personification of the corticobasal ganglionic degeneration affected body part, and autonomous (CBD).2`-4 In addition to "alien-ness" and activity which is perceived as outside the other original features, patients often expe- voluntary control. Although the hand is rienced involuntary complex motor activities. most frequently affected, any limb or Observations include grasp reflexes and motor combination of limbs may fulfil the alien preservation, usually on the same side as the limb criteria. Alien hand sign should be involuntary movements; hemiparesis and reserved for cases in which the hand feels hemisensory changes; a wide range of involun- foreign together with observable involun- tary complex motor behaviour, from grasping tary motor activity. To characterise this and groping to self destructive activity or phenomenon, seven patients with alien intermanual conflict; and subtle speech and hand sign and other motor or behavioural language abnormalities. A review ofthe various manifestations are described. Aetiologies usages of alien hand and its evolving definition included multiple infarcts and cortobasal as a syndrome may be found in another ganglionic degeneration (CBD). In this publication. 5 study, all patients had apraxia in response Despite numerous clinical descriptions, no to verbal commands and problems with systematic approach has been proposed for bimanual coordination. Most displayed comprehensive examination of patients with non-goal directed involuntary motor the alien hand sign. The reports suggest that activities, and two had self destructive more than one anatomical lesion or patho- motor behaviours. Grasp reflex occurred logical process accounts for alien limb phe- with alien hand due to either aetiology. nomena, but no consistent anatomic correlates Cortical reflex myoclonus was frequendy have emerged. We therefore studied patients seen in CBD patients. The phen- with alien hand and related signs in order to omenological spectrum is reviewed, a suggest a comprehensive assessment and possi- diagnostic protocol proposed, and possi- ble anatomical mechanisms. ble anatomical bases of alien hand dis- http://jnnp.bmj.com/ cussed. Methods (7 Neurol Neurosurg Psychiatry 1992;55:806-8 10) We reviewed the hospital and clinic records of patients presenting to the neurology depart- ment with alien hand sign or related sensor- The alien hand sign, first described by Brion imotor phenomena. The study period spanned and Jedynak in 1972, referred to denial ofhand from 1988 to the present. Out of the seven ownership and the inability to transfer func- patients in the study, videotapes are available on September 27, 2021 by guest. Protected copyright. tions between the hemispheres in patients with for five, all ofwhom had CBD. All patients had corpus callosum tumours.' Some patients complete histories and physical examinations, thought that one hand felt foreign or alien; they neuroimaging studies and laboratory investiga- could not imitate hand postures of one hand tions to rule out metabolic disorders. with the other and could not write. Other Patients were included if they complained of patients had tactile anomia and apraxia in a foreign or alien limb, or carried a diagnosis of Baylor College of response to verbal commands of the involved alien limb provided they also had complex, Medicine, Department hand (verbal apraxia): they tended to con- autonomous motor activity that was involun- of Neurology, to When visual Patients were excluded ifthey had apraxia Houston, Texas, USA fabulate responses questions. tary. R S Doody* cues were removed (one hand held behind the or an identifiable movement disorder, for J Jankovic back), these patients could not differentiate example, hemiballismus that fully explained Alzheimer's Disease their own hand from the examiner's by feeling the involuntary motor phenomenon (see Research Center and Houston VA Medical with their normal hand. appendix). Center,* Houston, Since the initial report, several authors have USA described alien limbs in association with con- Correspondence to: tralateral frontal corpus callosum Results Dr Doody, Department of strokes;24 Neurology, Baylor College of infarction;5 anterior communicating artery Four patients were women and all were right Medicine, 6550 Fannin, combina- handed except patient 7 who was ambidex- Suite 1801, Houston, Texas rupture;6-9 corpus callosectomy;"' 77030, USA tion of a posterior corpus callosum lesion and trous. Age of onset ranged from 57 to 85. Four The alien hand and related signs 807 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.55.9.806 on 1 September 1992. Downloaded from Table I The alien hand and related signs, clinicalfeatures and progression Patient Age of onset (years) Sex Aetiology Handedness Onset side Progression 1 63 F CBD R LUE Wihin 2 years,LLE & RLE 2 60 F CBD R L UE Within 2 years, RUE 3 57 F CBD R R UE Within 2 years, LUE & RLE 4 72 M CBD R R UE Within 4 years RLE 5 69 M CBD +/- infarction R R UE Within 4 years, RLE & LUE 6 85 F infarction R R UE Within days, RLE 7 63 M infarction Mixed Both UEs Resolved CBD = corticobasal ganglionic degeneration; F = female; L = left; LE = lower extremity; M = male; R = right; UE = upper extremity. patients had CBD and two had multiple We excluded one patient who had several strokes (table 1). Patient 5 had a presentation elements of the alien hand sign but did not and course similar to what has been described meet the full criteria which we proposed. This for CBD, but also had multiple lesions on CT presumed CBD patient presented four years and a left slow-wave focus on EEG, raising the into an illness which began with speech apraxia possibility of multiple infarcts alone or in and right sided clumsiness. When examined, combination with CBD. His symptoms, how- she was completely mute, had trouble with ever, predated his CT findings (he had an somaesthetic transfer (cross replicating hand earlier, normal study), and were slowly pro- postures) and bimanual coordination. She gressive, which makes a stroke related aetiol- frequently held her right arm with her left ogy less likely. hand. When asked to touch her nose, she The alien limb initially involved a hand in all rubbed the side of her head or her face before seven patients, and progressed to involve other ultimately touching the nose. Comprehension limbs in six of the seven (table 1). Patient 7, for three step commands not involving the who was ambidextrous, had a bilateral onset. right limbs was good. We suspect that alien His condition resolved within a few days of an limb could have been diagnosed earlier in her acute right frontal infarct, but the alien limbs disease, when she could have told us whether persisted in all other patients until lost to or not her limb activity felt foreign or alien. follow up or until the limb became stiff and useless. In the CBD patients, the illness either began with development of an alien hand or Discussion with Parkinsonism, later complicated by an The term alien hand has been used to describe alien hand sign. The two patients with cerebral a wide variety of sensory impressions and infarcts varied: one had the sudden onset of motor behaviours. Minimal diagnostic criteria alien hand and a right parietal syndrome, while have not been set. We define alien limb as a the other had the insidious onset of interman- feeling that one limb is foreign or "has a will of ual conflict. For example, he noted that one its own", together with observable involuntary hand tried to turn left when the other hand motor activity. Our study and review of publi- tried to turn right while driving a car. shed reports support this definition for all All patients complained, at some time in cases of alien hand, regardless of cause. The their illness, of involuntary complex motor feeling of alien-ness can range from a percep- activity and the perception that their limb tion that the limb belongs to the examiner http://jnnp.bmj.com/ either did not obey them or that it did not rather than the patient to personification of the belong to them. All had difficulty carrying out limb. Such personifications can be quite elabo- verbal commands with the affected limb(s). Six rate and patients might be mistakenly thought of the seven patients displayed some non-goal psychotic: patient 6 believed that her left arm directed motor activity, such as posturing, was a baby called Joseph, and that its actions grasping and groping, or action induced pat- against other parts of her body (like pinching terned, rhythmical movements (table 2). Two her nipples) were mischievous behaviours (bit- patients had self destructive behaviours which ing while nursing). The autonomous motor on September 27, 2021 by guest. Protected copyright. were autonomous but purposeful, in addition activities seen constitute a spectrum of com- to the non-goal directed behaviours. Four of plexity from action induced patterned and seven, all with CBD, had cortical reflex myo- rhythmical movement to non-goal directed clonus. At the time of examination, four grasping and groping behaviour, goal directed patients had difficulty with bimanual coordina- activities like utilisation behaviour (the com- tion, on tasks such as alternating fist closure pulsive manipulations of tools) or even self and opening or taking off glasses, folding the destructive acts.