- Email: [email protected]
“Naturalization” of the alien hand: Case report
113
PROSTHETIC/ORTHOTIC DEVICES
"Naturalization" of the Alien Hand: Case Report John J. Nicholas, MD, Monica H. Wichner, DO, Philip B. Gorelick, MD, Michael M. Ramsey, MD A B S T R A C T . Nicholas JJ, Wichner MH, Gorelick PB, Ramsey MM. "Naturalization" of the alien hand: case report. Arch Phys Med Rehabil 1998;79:113-4. The alien hand syndrome is an involuntary motor phenomenon that occurs infrequently and mostly in stroke patients. A case is reported of a 67-year-old man with left hemiparesis whose hand crept and crawled, especially at night, which caused him to awaken by grasping his collar. The disturbing nocturnal activity of the hand was stilled by placing it in a common oven mitt.
© 1998 by the American Congress of Rehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation HE A L I E N H A N D syndrome is a motor phenomenon in which the hand escapes total voluntary motor control. 1 It usually presents in association with mild hemiparesis. The hand develops " a life of its o w n , " and its movements may be bothersome or distressing to the patient, The condition was originally described by Goldstein in 1908, 2 and the term "alien hand syndrome" (la main 6trang~re) was designated by Brion and Jedynak in 1972. 3 We report a 67-year-old man who was noted to have uncontrolled movements of his left hand after an ischemic stroke with left hemiparesis. The spontaneous activity of the hand was distressing to the patient, especially at night, but was controlled by wearing a common oven mitt when sleeping. The phenomenon occurs mostly in right-handed patients who have a left hemiparesis, and the corpus callosum and supplementary motor area 6 is almost always involved. No satisfactory rehabilitation of alien hand syndrome has been reported.
T
CASE REPORT The patient was a 67-year-old man with a history of coronary artery disease (coronary artery bypass graft [CABG] in 1983) and non-insulin-dependent diabetes mellitus. He was hospitalized with weakness of the left face, arm, and leg on May 30, 1996, at an outlying hospital. Initial computed tomography (CT) scan was unremarkable, as was a carotid ultrasound study. The patient was transferred June 3, 1996, to the rehabilitation service. Repeat CT of the head showed a subacute right anterior cerebral artery territory infarction. When little improvement occurred, the patient's family asked for transfer to the rehabilitation service of the institution where his primary care physician practiced. On examination, the patient had a left central facial palsy. No motion was noted in his left arm or leg, with the exception From the Department of Physical Medicine and Rehabilitation (Drs. Nicholas, Wichner), the Center for Stroke Research, Department of NeurologicalSciences (Dr. Gorelick), and Department of Internal Medicine (Dr. Ramsey), Rush-Presbyterian-St. Luke's Medical Center, Chicago, IL. Submitted for publication June 4, 1997. Accepted in revised form July 14, 1997. No commercialparty having a direct or indirect interest in the subject matter of this articlehas or will confera benefitupon the authors or upon any organization with which the authors are associated. Reprint requests to John J. Nicholas, MD, Rush Medical Center, 1653 West Congress Parkway, Chicago, IL 60612. © 1998 by the AmericanCongressof Rehabilitation Medicineand the American Academy of Physical Medicine and Rehabilitation 0003-9993/98/7901-453253.00/0
of flexion and extension movements of the fingers of his left hand and slight function of the intrinsic hand muscles. There was no Hoffman sign, Babinski sign, or clonus. Pinprick was diminished in the left arm and leg. The patient was alert and oriented. He cried frequently and became disoriented at night. The remainder of the findings from his physical examination were normal. Routine blood laboratory data were unremarkable. Magnetic resonance imaging (MRI) with gadolinium on June 24th revealed evidence of subacute hemorrhage and edema in the territory of the anterior cerebral artery involving the corpus callosum. A chest X-ray showed surgical clips from the CABG, Plain films of the lumbar spine showed a large osteophyte on the left at L1 and L2 and smaller osteophytes throughout the spine. A transesophageal echocardiogram showed a 1.0- to 2.0mm calcification on the aortic valve, and a patent foramen ovale with significant "right to left shunting." The electrocardiogram revealed normal sinus rhythm with occasional supraventricular premature beats and an inferior wall infarction of uncertain age. Warfarin was administered after three blood cultures were unremarkable. The patient participated in his therapies and began to improve. On June 21, it was noted that the left hand had become " a c t i v e " and was "crawling around," grasping his belt, his robe, bed sheets, and his pockets in an uncontrolled manner. On June 27, it was noted he could flex his left elbow, and on July 1, there was motion in the left biceps and anterior deltoid, as well as wrist flexion and extension. By July 8, the patient could abduct his left shoulder 180 ° and asserted he felt pinprick sensation in the left arm. The patient's balancing skills improved, and by July 17, he could flex his left hip, stand, and pivot transfer with the aid of only one person. He had poor-grade strength in left hip flexion and knee extension. With the aid of a walker and a plastic ankle-foot orthosis, he learned to transfer and walk, although he was unsteady. As early as June 21, the patient had voluntary control of his left hand; however, the hand would become active, crawling around and grasping objects in an involuntary manner. At these times, the patient could not voluntarily control his hand, but would have to grab the left hand with the right one to hold it still. The hand awakened him at night as it clutched at his collar and the neck of his gown. Because the patient was distressed by this involuntary activity, he was provided a common oven mitt in the hope that this would inhibit the hand's activity. This was completely successful, and the hand stopped crawling and grasping while it was encased in the mitt. When attending therapies without the oven mitt, the patient performed bimanual activities skillfully. His left hand was dexterous and useful. Nonetheless~ when he was not paying attention or became excited, his hand began the annoying involuntary movements. The patient was discharged in the care of his wife on warfarin therapy on July 19. On follow-up 8 months later, the patient reported continued involuntary activity of his alien hand with suppression of the activity by use of the oven mitt. DISCUSSION Alien hand syndrome usually presents as a motor phenomenon accompanying a right or left hemiparesis and involving the
Arch Phys Med Rehabil Vol 79, January 1998
114
"NATURALIZATION" OF THE ALLEN HAND, Nicholas
hand or, on rare occasions, the leg. It is generally believed to be associated with lesions of the corpus callosum and associated supplementary motor areas. 1 Goldstein is generally credited with the first descriptive narrative of the phenomenon2; however, it was Brion and Jedynek3 who coined the term "the alien hand" (la main 6trang6re). Leiguarda et ale reported three cases of patients with hemorrhage into the corpus callosum. Multiple abnormalities of the corpus callosum were described, and two cases featured oppositional involuntary antagonistic behavior of the involved left hand. Leiguarda attributed the alien hand syndrome to involvement of the mid and posterior portions of the corpus callosum. McNabb and colleagues5 described three cases with ischemic infarction in the territory of the anterior cerebral artery with alien hand syndrome. In the first case, the hand groped, grabbed, and interfered. In the second and third cases, the hand interfered, but did not grope, grasp, or grab. The authors asserted that involvement of the supplementary motor area, as well as the corpus callosum, was responsible for the abnormal motor activity. Banks and colleagues6 described two cases of alien hand syndrome. In the first case, a patient's left hand exhibited grasping, groping, and bothersome movements following a gunshot wound. In the second case, a patient who had had cerebral aneurysmal rupture noted left-handed oppositional movements. Both patients had frontal corpus callosum lesions. In the first case, the patient was able to sleep at night by tying the hand. Gottlieb and colleagues7 described two patients with ischemic frontoparietal lesions and involuntary movements of the left hand. One patient's left hand performed mirrorlike movements that interfered with the patient's right hand. The other patient's left or hemiparetic hand "did the opposite" of the patient' s right hand. Gottlieb and colleagues showed electroencephalographic activity of the bilateral supplementary motor areas occurring with voluntary unilateral movement. They maintained that this was part of the development of motor control that was subsequently suppressed, and cited as evidence contralateral homologous muscle contractions that occurred in both normal and hemiplegic subjects when unilateral-willed muscle contractions were performed. They suggested that the supplementary motor area controls and suppresses primitive bilateral activity, and when it is injured, this suppression is removed. "As yet the treatment of this handicap (alien hand syndrome) is deficient," the authors noted, and suggested, "Holding a can or some object in the problematic hand diminishes its tendency to reach voluntarily for other objects." Doody and Jankovic 8 described seven cases of AHS culled from hospital records. Five had corticobasal ganglionic degeneration. One patient had bilateral, insidious-onset, involuntary movements. All patients complained that their hand moved involuntarily and that they also were not able to consistently move the hand voluntarily. Grasping and groping movements of the hand were reported by 6 of the 7 patients. Several felt that the hand was "not theirs." In addition, there were mirror and oppositional interfering movements. The authors pointed out that there was no callosal involvement in corticobasal ganglionic degeneration. They distinguished two types of alien hand syndrome, a "frontal type" in which the groping, grasping involuntary movements were seen and a "callosal type" in which mirror imaging and oppositional conflicting activities occurred. They suggested that "although no specific therapy
Arch Phys Med Rehabil Vol 79, January 1998
exists for the alien hand, experimental neurosurgical approaches to modulating the corpus callosum or supplementary motor area activity remain a p o s s i b i l i t y . . . " Geschwind et al I described a patient with left hemiparesis after CABG who on the first day after surgery noted that his left hand choked him, unbuttoned his shirt, and fought with the right hand] Mirror movements were also described. There was a precise lesion of the posterior body of the posterior callosum seen on MRI. Geschwind and associates suggested this type of alien hand syndrome was distinguishable anatomically from the frontal type. They also cited evidence to suggest that the nondominant hand was most frequently involved, and reported that MRI and positron-emission tomography demonstrated bilateral activities during left-handed movements in right-handeddominant persons. When dextrals used their right hand, however, only the left hemisphere supplementary motor areas were active. This suggested that lesions of the corpus callosum would most likely disconnect motor control to the right hand completely, whereas in dextrals, the left hand was under more bilateral control and was not suppressed completely by right-sided lesions. Additional insight into the mechanism of the alien hand syndrome comes from the work of Shahani and associates 9 who described a 59-year-old patient with degenerative cerebral disease and a right-handed spontaneous grasp reflex. Electromyographic data support their theory that the grasp reflex is a cutaneous reflex mediated at a subcortical or spinal cord level. Our patient demonstrated groping, grasping, and bothersome movements of the left hand. He did not, however, demonstrate mirror activities or oppositional interfering movements. In addition, he was able to control his left hand voluntarily at all times except when the hand acted involuntarily and requited control by grasping with the right hand. We were successful in eliminating the bothersome nocturnal activity of the alien hand by enclosing it in a common oven mitt. It is possible that the oven mitt provide sufficient sensory stimulation to the sensory spinal grasp reflex so that accommodation was reached and the motor activity ceased. References
1. Geschwind DH, Iacoboni M, Mega MS, Zaidel DW, Cloughesy T, Zaidel E. Alien hand syndrome: interhemispheric motor disconnection due to a lesion in the midbody of the corpus callosum. Neurology 1995;45:802-8. 2. Goldstein K. Zur Lehre der motorischen Apraxie. J Ftir Psychologie und Neurologie 1908;11:169-87. 3. Brion S, Jedynak CP. Troubles du transfert interh6misph6rique(callosal disconnection)apropos de 3 observations de tumeurs du corps calleux: le signe de la main 6trangbre. Rev Neurol (Paris) 1972;126: 257-66. 4. Leiguarda R, Starkstein S, Berthier M. Anterior callosal haemorrhage. A partial interhemispheric disconnection syndrome. Brain 1989; 112:1019-37. 5. McNabb AW, Carroll WM, Mastaglia FL. "Alien hand" and loss of bimanual coordinationafter dominant anterior cerebral artery territory infarction. J Neurol Neurosurg Psychiatry 1988;51:218-22. 6. Banks G, Short P, Martinez J, Latchaw R, Ratcliff G, Phil D, et al. The alien hand syndrome. Arch Neurol 1989;46:456-9. 7. Gottlieb D, Robb K, Day B. Mirror movements in the alien hand syndrome. Case report. Am J Phys Med Rehabil 1992;71:297-300. 8. Doody RS, Jankovic J. The alien hand and related signs. J Neurol Neurosurg Psychiatry 1992;55:806-10. 9. Shahani B, Burrows P, Whitty CWM. The grasp reflex and preservation. Brain 1970;93:181-92.
PROSTHETIC/ORTHOTIC DEVICES
"Naturalization" of the Alien Hand: Case Report John J. Nicholas, MD, Monica H. Wichner, DO, Philip B. Gorelick, MD, Michael M. Ramsey, MD A B S T R A C T . Nicholas JJ, Wichner MH, Gorelick PB, Ramsey MM. "Naturalization" of the alien hand: case report. Arch Phys Med Rehabil 1998;79:113-4. The alien hand syndrome is an involuntary motor phenomenon that occurs infrequently and mostly in stroke patients. A case is reported of a 67-year-old man with left hemiparesis whose hand crept and crawled, especially at night, which caused him to awaken by grasping his collar. The disturbing nocturnal activity of the hand was stilled by placing it in a common oven mitt.
© 1998 by the American Congress of Rehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation HE A L I E N H A N D syndrome is a motor phenomenon in which the hand escapes total voluntary motor control. 1 It usually presents in association with mild hemiparesis. The hand develops " a life of its o w n , " and its movements may be bothersome or distressing to the patient, The condition was originally described by Goldstein in 1908, 2 and the term "alien hand syndrome" (la main 6trang~re) was designated by Brion and Jedynak in 1972. 3 We report a 67-year-old man who was noted to have uncontrolled movements of his left hand after an ischemic stroke with left hemiparesis. The spontaneous activity of the hand was distressing to the patient, especially at night, but was controlled by wearing a common oven mitt when sleeping. The phenomenon occurs mostly in right-handed patients who have a left hemiparesis, and the corpus callosum and supplementary motor area 6 is almost always involved. No satisfactory rehabilitation of alien hand syndrome has been reported.
T
CASE REPORT The patient was a 67-year-old man with a history of coronary artery disease (coronary artery bypass graft [CABG] in 1983) and non-insulin-dependent diabetes mellitus. He was hospitalized with weakness of the left face, arm, and leg on May 30, 1996, at an outlying hospital. Initial computed tomography (CT) scan was unremarkable, as was a carotid ultrasound study. The patient was transferred June 3, 1996, to the rehabilitation service. Repeat CT of the head showed a subacute right anterior cerebral artery territory infarction. When little improvement occurred, the patient's family asked for transfer to the rehabilitation service of the institution where his primary care physician practiced. On examination, the patient had a left central facial palsy. No motion was noted in his left arm or leg, with the exception From the Department of Physical Medicine and Rehabilitation (Drs. Nicholas, Wichner), the Center for Stroke Research, Department of NeurologicalSciences (Dr. Gorelick), and Department of Internal Medicine (Dr. Ramsey), Rush-Presbyterian-St. Luke's Medical Center, Chicago, IL. Submitted for publication June 4, 1997. Accepted in revised form July 14, 1997. No commercialparty having a direct or indirect interest in the subject matter of this articlehas or will confera benefitupon the authors or upon any organization with which the authors are associated. Reprint requests to John J. Nicholas, MD, Rush Medical Center, 1653 West Congress Parkway, Chicago, IL 60612. © 1998 by the AmericanCongressof Rehabilitation Medicineand the American Academy of Physical Medicine and Rehabilitation 0003-9993/98/7901-453253.00/0
of flexion and extension movements of the fingers of his left hand and slight function of the intrinsic hand muscles. There was no Hoffman sign, Babinski sign, or clonus. Pinprick was diminished in the left arm and leg. The patient was alert and oriented. He cried frequently and became disoriented at night. The remainder of the findings from his physical examination were normal. Routine blood laboratory data were unremarkable. Magnetic resonance imaging (MRI) with gadolinium on June 24th revealed evidence of subacute hemorrhage and edema in the territory of the anterior cerebral artery involving the corpus callosum. A chest X-ray showed surgical clips from the CABG, Plain films of the lumbar spine showed a large osteophyte on the left at L1 and L2 and smaller osteophytes throughout the spine. A transesophageal echocardiogram showed a 1.0- to 2.0mm calcification on the aortic valve, and a patent foramen ovale with significant "right to left shunting." The electrocardiogram revealed normal sinus rhythm with occasional supraventricular premature beats and an inferior wall infarction of uncertain age. Warfarin was administered after three blood cultures were unremarkable. The patient participated in his therapies and began to improve. On June 21, it was noted that the left hand had become " a c t i v e " and was "crawling around," grasping his belt, his robe, bed sheets, and his pockets in an uncontrolled manner. On June 27, it was noted he could flex his left elbow, and on July 1, there was motion in the left biceps and anterior deltoid, as well as wrist flexion and extension. By July 8, the patient could abduct his left shoulder 180 ° and asserted he felt pinprick sensation in the left arm. The patient's balancing skills improved, and by July 17, he could flex his left hip, stand, and pivot transfer with the aid of only one person. He had poor-grade strength in left hip flexion and knee extension. With the aid of a walker and a plastic ankle-foot orthosis, he learned to transfer and walk, although he was unsteady. As early as June 21, the patient had voluntary control of his left hand; however, the hand would become active, crawling around and grasping objects in an involuntary manner. At these times, the patient could not voluntarily control his hand, but would have to grab the left hand with the right one to hold it still. The hand awakened him at night as it clutched at his collar and the neck of his gown. Because the patient was distressed by this involuntary activity, he was provided a common oven mitt in the hope that this would inhibit the hand's activity. This was completely successful, and the hand stopped crawling and grasping while it was encased in the mitt. When attending therapies without the oven mitt, the patient performed bimanual activities skillfully. His left hand was dexterous and useful. Nonetheless~ when he was not paying attention or became excited, his hand began the annoying involuntary movements. The patient was discharged in the care of his wife on warfarin therapy on July 19. On follow-up 8 months later, the patient reported continued involuntary activity of his alien hand with suppression of the activity by use of the oven mitt. DISCUSSION Alien hand syndrome usually presents as a motor phenomenon accompanying a right or left hemiparesis and involving the
Arch Phys Med Rehabil Vol 79, January 1998
114
"NATURALIZATION" OF THE ALLEN HAND, Nicholas
hand or, on rare occasions, the leg. It is generally believed to be associated with lesions of the corpus callosum and associated supplementary motor areas. 1 Goldstein is generally credited with the first descriptive narrative of the phenomenon2; however, it was Brion and Jedynek3 who coined the term "the alien hand" (la main 6trang6re). Leiguarda et ale reported three cases of patients with hemorrhage into the corpus callosum. Multiple abnormalities of the corpus callosum were described, and two cases featured oppositional involuntary antagonistic behavior of the involved left hand. Leiguarda attributed the alien hand syndrome to involvement of the mid and posterior portions of the corpus callosum. McNabb and colleagues5 described three cases with ischemic infarction in the territory of the anterior cerebral artery with alien hand syndrome. In the first case, the hand groped, grabbed, and interfered. In the second and third cases, the hand interfered, but did not grope, grasp, or grab. The authors asserted that involvement of the supplementary motor area, as well as the corpus callosum, was responsible for the abnormal motor activity. Banks and colleagues6 described two cases of alien hand syndrome. In the first case, a patient's left hand exhibited grasping, groping, and bothersome movements following a gunshot wound. In the second case, a patient who had had cerebral aneurysmal rupture noted left-handed oppositional movements. Both patients had frontal corpus callosum lesions. In the first case, the patient was able to sleep at night by tying the hand. Gottlieb and colleagues7 described two patients with ischemic frontoparietal lesions and involuntary movements of the left hand. One patient's left hand performed mirrorlike movements that interfered with the patient's right hand. The other patient's left or hemiparetic hand "did the opposite" of the patient' s right hand. Gottlieb and colleagues showed electroencephalographic activity of the bilateral supplementary motor areas occurring with voluntary unilateral movement. They maintained that this was part of the development of motor control that was subsequently suppressed, and cited as evidence contralateral homologous muscle contractions that occurred in both normal and hemiplegic subjects when unilateral-willed muscle contractions were performed. They suggested that the supplementary motor area controls and suppresses primitive bilateral activity, and when it is injured, this suppression is removed. "As yet the treatment of this handicap (alien hand syndrome) is deficient," the authors noted, and suggested, "Holding a can or some object in the problematic hand diminishes its tendency to reach voluntarily for other objects." Doody and Jankovic 8 described seven cases of AHS culled from hospital records. Five had corticobasal ganglionic degeneration. One patient had bilateral, insidious-onset, involuntary movements. All patients complained that their hand moved involuntarily and that they also were not able to consistently move the hand voluntarily. Grasping and groping movements of the hand were reported by 6 of the 7 patients. Several felt that the hand was "not theirs." In addition, there were mirror and oppositional interfering movements. The authors pointed out that there was no callosal involvement in corticobasal ganglionic degeneration. They distinguished two types of alien hand syndrome, a "frontal type" in which the groping, grasping involuntary movements were seen and a "callosal type" in which mirror imaging and oppositional conflicting activities occurred. They suggested that "although no specific therapy
Arch Phys Med Rehabil Vol 79, January 1998
exists for the alien hand, experimental neurosurgical approaches to modulating the corpus callosum or supplementary motor area activity remain a p o s s i b i l i t y . . . " Geschwind et al I described a patient with left hemiparesis after CABG who on the first day after surgery noted that his left hand choked him, unbuttoned his shirt, and fought with the right hand] Mirror movements were also described. There was a precise lesion of the posterior body of the posterior callosum seen on MRI. Geschwind and associates suggested this type of alien hand syndrome was distinguishable anatomically from the frontal type. They also cited evidence to suggest that the nondominant hand was most frequently involved, and reported that MRI and positron-emission tomography demonstrated bilateral activities during left-handed movements in right-handeddominant persons. When dextrals used their right hand, however, only the left hemisphere supplementary motor areas were active. This suggested that lesions of the corpus callosum would most likely disconnect motor control to the right hand completely, whereas in dextrals, the left hand was under more bilateral control and was not suppressed completely by right-sided lesions. Additional insight into the mechanism of the alien hand syndrome comes from the work of Shahani and associates 9 who described a 59-year-old patient with degenerative cerebral disease and a right-handed spontaneous grasp reflex. Electromyographic data support their theory that the grasp reflex is a cutaneous reflex mediated at a subcortical or spinal cord level. Our patient demonstrated groping, grasping, and bothersome movements of the left hand. He did not, however, demonstrate mirror activities or oppositional interfering movements. In addition, he was able to control his left hand voluntarily at all times except when the hand acted involuntarily and requited control by grasping with the right hand. We were successful in eliminating the bothersome nocturnal activity of the alien hand by enclosing it in a common oven mitt. It is possible that the oven mitt provide sufficient sensory stimulation to the sensory spinal grasp reflex so that accommodation was reached and the motor activity ceased. References
1. Geschwind DH, Iacoboni M, Mega MS, Zaidel DW, Cloughesy T, Zaidel E. Alien hand syndrome: interhemispheric motor disconnection due to a lesion in the midbody of the corpus callosum. Neurology 1995;45:802-8. 2. Goldstein K. Zur Lehre der motorischen Apraxie. J Ftir Psychologie und Neurologie 1908;11:169-87. 3. Brion S, Jedynak CP. Troubles du transfert interh6misph6rique(callosal disconnection)apropos de 3 observations de tumeurs du corps calleux: le signe de la main 6trangbre. Rev Neurol (Paris) 1972;126: 257-66. 4. Leiguarda R, Starkstein S, Berthier M. Anterior callosal haemorrhage. A partial interhemispheric disconnection syndrome. Brain 1989; 112:1019-37. 5. McNabb AW, Carroll WM, Mastaglia FL. "Alien hand" and loss of bimanual coordinationafter dominant anterior cerebral artery territory infarction. J Neurol Neurosurg Psychiatry 1988;51:218-22. 6. Banks G, Short P, Martinez J, Latchaw R, Ratcliff G, Phil D, et al. The alien hand syndrome. Arch Neurol 1989;46:456-9. 7. Gottlieb D, Robb K, Day B. Mirror movements in the alien hand syndrome. Case report. Am J Phys Med Rehabil 1992;71:297-300. 8. Doody RS, Jankovic J. The alien hand and related signs. J Neurol Neurosurg Psychiatry 1992;55:806-10. 9. Shahani B, Burrows P, Whitty CWM. The grasp reflex and preservation. Brain 1970;93:181-92.