Right Sensory Alien Hand Phenomenon from a Left Pontine Hemorrhage

Background

Acute onset of a sensory alien hand phenomenon has been observed only from a supratentorial lesion involving the non-dominant hand, mostly from a right posterior cerebral artery infarction. A single acute vascular lesion resulting in a dominant hand sensory alien hand syndrome has not been previously documented.

Case Report

A 78-year old right-handed woman exhibited right sensory alien hand phenomenon from a left pontine hemorrhage. Disturbance of proprioceptive input and visuospatial perception are likely to play a role in manifesting the sign.

Conclusions

Dominant-hand sensory alien hand phenomenon may occur in an acute setting from a left pontine hemorrhage.     

Moving with or without will: functional neural correlates of alien hand syndrome

Alien hand syndrome is a rare neurological disorder in which movements are performed without conscious will. By using functional magnetic resonance imaging in a patient with alien hand syndrome after right parietal lesion, we could identify brain regions activated during involuntary or voluntary actions with the affected left hand. Alien hand movements involved a selective activation of contralateral primary motor cortex (M1), presumably released from conscious control by intentional planning systems. By contrast, voluntary movements activated a distributed network implicating not only the contralateral right M1 and premotor cortex but also the left inferior frontal gyrus, suggesting an important role of the dominant hemisphere in organizing willed actions.     

Callosal alien hand sign following a right parietal lobe infarction

Callosal alien hand syndrome is characterized primarily by intermanual conflict and is associated with an anterior callosal lesion. We report a patient who presented with topographical disorientation and the callosal type alien hand sign. An MRI of the brain showed a right parietal lobe infarction. This is a rare example of callosal alien hand sign associated with a right parietal lesion. The right parietal lobe appeared to be responsible for the callosal hand sign in this patient, possibly due to interference with peristriate outflow pathways toward the parietal zones, where visual somatosensory interactions are likely to occur.     

Alien hand syndrome in left posterior stroke

Alien hand syndrome is a rare neurological disorder characterized by involuntary and uncontrollable motor behaviour, usually of an arm or hand. The patient perceives the affected limb as alien, and may personify it. The case of a 61-year-old right-handed woman who developed right posterior AHS after ischaemic stroke in the left posterior cerebral artery territory is reported. Neuroimaging studies disclosed no frontal or parietal involvement, while a posterior thalamic lesion was detected. A possible role of the thalamus in the genesis of AHS is discussed.     

Transient left-sided alien hand with callosal and unilateral fronto-mesial damage: A case study

A patient who showed a transient left alien hand after a subarachnoid haemorrhage, due to a ruptured aneurysm of the anterior communicating artery, is described. No left agraphia and left apraxia could be found. A CT-scan including coronal sections showed a right medial frontal hypodensity and different ischaemic lesions in the anterior part of the corpus callosum. The possible anatomic site of the lesion responsible for the permanent form of alien hand is discussed.     

A case of alien hand syndrome after right posterior cerebral artery territory infarction]

We report a 63-year-old right-handed man who presented an alien hand syndrome (AHS). He complained of clumsiness of his left hand and admitted to our hospital. On the first examination, he presented left homonymous hemianopia, left spatial neglect and left limb ataxia, but neither paralysis nor sensory impairment. A few days after, he complained that his left hand was controlled by someone else, and we considered this phenomenon as AHS. At that time, he lost sensation of almost all modalities including deep sensation on his left upper and lower limb. Magnetic resonance image examination was performed, and it showed acute cerebral infarction at right posterior cerebral artery territory including right thalamus (ventral posterior lateral nucleus). Generally, AHS is caused by left mediofrontal and callosal lesion (frontal type AHS), or by callosal with bilateral frontal or without frontal lesion (callosal type AHS). However, some cases were reported that they presented AHS after damage of the basal ganglia, right thalamus, right occipital or inferior parietal lobe. Some authors described this phenomenon as "sensory" or "posterior" type AHS. In such cases, included our case, we speculate that sensory impairment causes AHS. Especially in our case, AHS might be caused by not only the sensory impairment but also by left homonymous hemianopia and left spatial neglect. So, because of these symptoms, our patient could not recognize the motion of the left hand, and presented AHS. We think that this "sensory" or "posterior" type AHS should be distinguished from frontal and callosal type AHS.     

Opticosensory ataxia and alien hand syndrome after posterior cerebral artery territory infarction

A right-handed woman developed left homonymous hemianopia and left hemianesthesia from infarction due to right posterior cerebral artery occlusion. Ataxia of the left arm and leg was severe and was not improved by vision. The patient often interpreted spontaneous movements of the left arm as alien in origin. The ataxia may have been caused by a combination of sensory ataxia, resulting from right thalamic infarction, and crossed-optic ataxia, resulting from posterior disconnection of the hemispheres due to right temporo-occipital infarction. This opticosensory ataxia was associated with a new form of "alien hand" syndrome.     

Right-sided anarchic (alien) hand: a longitudinal study.

A patient with a bilateral frontal vascular lesion, encroaching upon the mesial cortex on the left and damage of the corpus callosum showed the 'alien hand' phenomenon on the right. The various hypotheses as to the nature of the lesion for the alien hand phenomenon to appear are discussed. It is proposed that an acute clinical condition, following a lesion of the corpus callosum only, should be differentiated from a chronic condition resulting from the additional fronto-mesial lesion.     

Slowly progressive apraxia in Alzheimer's disease.

Slowly progressive apraxia due to Alzheimer's disease was encountered in a 66 year old, right handed man whose initial impairments included coordinated movements of the left hand and some features of the alien hand syndrome. Over four years, the patient developed progressively worsening deficits of memory and language. A biopsy of his right temporal lobe showed numerous plaques and neurofibrillary tangles. Pronounced right parietal lobe hypoperfusion on serial SPECT suggests involvement of this region in contralateral praxis.     

Recurrent alien hand syndrome in a multiple sclerosis case

Alien hand syndrome is the strange feeling of one's hand behaving independently. This syndrome has rarely been reported in multiple sclerosis (MS) patients. Herein, we present a 34-year-old female MS patient who had recurrent symptoms of alien hand syndrome that were evaluated as MS attacks based on cranial magnetic resonance imaging that showed demyelinating lesions in the corpus callosum. Alien hand syndrome is classified according to the location of the lesion and the presenting symptoms. As such, our patient can best be classified as a callosal alien hand case.     

The alien hand syndrome. Clinical and postmortem findings

Two patients had automatonlike movements of their left hands and arms (alien hand syndrome) following damage to the brain. Autopsy findings in one patient demonstrated gunshot wound damage to the medial frontal white matter bilaterally, as well as the corpus callosum, right basal ganglia, internal capsule, and thalamus. The other patient had a ruptured anterior communicating aneurysm, with subsequent resection of the right frontal gyrus rectus. We postulate that this syndrome is due to the combination of a partial callosectomy and mesial frontal lesions.     

Disconnection syndrome in a right-handed patient with right hemispheric speech dominance

The callosal disconnection syndrome is characterized by unilateral (left) tactile anomia, unilateral (left) agraphia and unilateral (left) ideomotor apraxia. We report on a right-handed patient, who developed a callosal disconnection syndrome due to the rupture of an anterior communicant aneurysm. As he had right hemisphere dominance for language and praxis, the signs described above were crossed. He also featured a left alien hand sign and motor aprosody. The authors believe this is the first case of callosal disconnection in a right-hander with crossed language and praxis functions.     

Involuntary masturbation and hemiballismus after bilateral anterior cerebral artery infarction

Ischemia of the areas supplied by the anterior cerebral artery is relatively uncommon. In addition, combined hemiballismus and masturbation have rarely been reported in patients with cerebrovascular disease. We describe herein a 62-year-old right-handed man simultaneously exhibiting right side hemiballismus and involuntary masturbation with the left hand after bilateral infarction of the anterior cerebral artery territory. Right side hemiballismus was related to the disruption of afferent fibers from the left frontal lobe to the left subthalamic nucleus. Involuntary masturbation using the left hand was exclusively linked to a callosal type of alien hand syndrome secondary to infarction of the right side of the anterior corpus callosum. After 2 weeks, these abnormal behaviours were completely extinguished. This report stresses the wide diversity of clinical manifestations observed after infarction of the anterior cerebral artery territory.     

Cheiro-oral syndrome due to lesions in the corona radiata.

BACKGROUND AND PURPOSE: We describe three patients with cheiro-oral syndrome caused by a small lesion in the corona radiata confirmed by high-resolution magnetic resonance imaging. CASE DESCRIPTIONS: Case 1: A 56-year-old hypertensive man who developed hypesthesia and paresthesia in the left perioral area and hand was found to have a small hematoma just lateral to the right internal capsule. Case 2: A 67-year-old man noticed hypesthesia around the left mouth angle and thumb and index finger. Magnetic resonance imaging revealed a lesion in the right corona radiata. Case 3: A 45-year-old hypertensive man developed numbness in his perioral region and left hand that later spread to his shoulder. Magnetic resonance imaging revealed a recent small infarct in the lower lateral aspect of the right corona radiata. CONCLUSIONS: A small lesion in the corona radiata can cause cheiro-oral syndrome, whose pathogenetic mechanism in such patients may be explained by the somatotopical location or by the differing vulnerability of the neuropils in the corona radiata.     

Alzheimer's disease presenting as corticobasal syndrome.

A 60-year-old man presented with slowly progressive left hemi-Parkinsonism, left hand apraxia, myoclonus, dystonia, visuospatial disturbances, and alien limb phenomenon, resembling corticobasal syndrome. Eight years later, neuropathology revealed features of Alzheimer's disease, with asymmetrical (right more than left) cortical tau burden with image analysis. The videotaped clinical features, neuropsychological aspects, and neuropathological correlates are presented and discussed.     

First-episode 'coenesthetic' schizophrenia presenting with alien hand syndrome and partial agenesis of the corpus callosum

We describe the case of a 23-year-old Caucasian woman who presented with alien hand syndrome and a first episode of the coenesthetic subtype of schizophrenia. 'Alienness' of her non-dominant hand was intimately phenomenologically associated with the onset of first-psychosis. Cerebral MRI revealed a partial agenesis of the corpus callosum with a complete absence of the rostrum, hypoplastic anterior and inferior genu, and a hypoplastic splenium. This case suggests that this syndrome can occur with the development of a functional disconnection syndrome involving the anterior callosum, and in this case the 'second hit' proposed to occur in early adulthood in schizophrenia may have interacted with her earlier neurodevelopmental lesion to result in a combination of psychosis and alien hand syndrome.     

Posterior alien hand syndrome after a right thalamic infarct

The alien hand syndrome, as originally defined, should be reserved for cases in which the hand feels foreign "together with" observable involuntary motor activity. These involuntary movements are unusual during or after acute stroke. Three varieties of alien hand syndrome have been reported, involving lesions of the corpus callosum alone, the corpus callosum plus dominant medial frontal cortex, and posterior cortical and subcortical areas. A patient with posterior alien hand syndrome of vascular aetiology is reported. Imaging studies disclosed an isolated infarction of the right thalamus sparing other cerebral regions.     

Controlling the alien hand through the mirror box. A single case study of Alien Hand Syndrome

Disruption of motor control in the alien hand syndrome might result from a dissociation between intentions and sensory information. We hypothesized that voluntary motor control in this condition could improve by restoring the congruency between motor intentions and visual feedback. The present study shows that, in one patient with right alien hand syndrome, the use of a mirror box paradigm improved motor speed. We speculate that the visual feedback provided by the mirror increases the sense of congruence between intention and sensory feedback, leading to motor improvement.     

Mixed alien hand syndrome coexisting with left-sided extinction secondary to a left corpus callosal lesion: a case report.

Alien hand syndrome (AHS) is actually two distinct syndromes with distinct clinical and anatomic features, that is, a frontal type and a callosal type. Frontal AHS occurs in the dominant hand; is associated with reflexive grasping, groping, and compulsive manipulation of tools. Callosal AHS is characterized primarily by intermanual conflict. We report a case of right frontal AHS and left callosal AHS (mixed AHS) secondary to ischemic stroke of the left corpus callosum (lesion extending from the genu to splenium) and right corpus callosum (minimal lesion in the splenium) in a 67-year-old male patient who also presented with left-sided tactile extinction. To our knowledge, rare reports have documented mixed AHS coexisting with nondominant side extinction secondary only to unilateral (left) callosal lesion, as in our case.     

Alien hand syndrome

In Stanley Kubrick's movie Dr Strangelove, the main character is described as "erratic" and displays a bizarre movement disorder. His right hand seems to be driven by a will of its own, at times clutching his own throat and at other times raising into a Nazi salute. Dr Strangelove must try to restrain this wayward limb with his left hand. Bizarre as this fictional character is, a similar movement disorder can occur in neurologic disease. The complex phenomenon associated with this disorder falls under the rubric of alien hand syndrome. This syndrome is characterized by a limb that seems to perform meaningful acts without being guided by the intention of the patient. Patients find themselves unable to stop the alien limb from reaching and grabbing objects, and they may be unable to release these grasped objects without using their other hand to pry open their fingers. These patients frequently express astonishment and frustration at the errant limb. They experience it as being controlled by an external agent and often refer to it in the third person. This article outlines the origins of the terminology used in describing this syndrome, early observations, and studies regarding its functional neuroanatomy.