Alien Hand Sign Research Articles

Rehabilitation of two patients presenting with alien hand sign

Rehabilitation of two patients presenting with alien hand sign

Posterior variant alien hand sign with prominent focal dystonia: A case report

Background: Alien hand sign (AHS) is classified into frontal, callosal, and posterior variants. Posterior variant has been rarely documented.

A case of Parry–Romberg syndrome and alien hand

Parry–Romberg syndrome (PRS) is a rare condition characterised by progressive hemi-facial atrophy. Here we present a PRS case with alien-hand syndrome, which has not previously been described in adult onset disease. On the basis of the presumed auto-immune pathology of PRS we justify the treatment strategy we successfully used in this patient. A review of the literature was extensively done for understanding the history of alien hand sign over the years.

The alien hand sign

We report a case of alien hand sign in a male with stroke and briefly discuss the pathogenesis of this rare condition symptom.

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.

病初期に他人の手徴候がみとめられたＣｒｅｕｔｚｆｅｌｄｔ−Ｊａｋｏｂ病の１例

A 68-year-old man was admitted to our hospital in the mid-October of 2006 because of a one-month history of peculiar movements of the left hand, which had been preceded by one month by awkward motions in the left leg. Upon neurological examination, spontaneous involuntary movement of the left hand was found. His left hand with his index finger stretched moved toward his right side spontaneously. He could not control his left leg freely. Although he showed mild ataxia in his left hand, there was no weakness, no dystonia, and no apraxia. No sensory abnormality was detected except for mild deep-sensation impairment in his left foot. The results of magnetic resonance imaging (MRI) including diffusion-weighted imaging (DWI) were normal; however, single-photon emission computed tomography (SPECT) showed hypoperfusion in the right hemisphere. At the time of admission, although a clinical diagnosis could not be made, we considered that the involuntary movements of his left hand were consistent with alien hand sign (AHS). Two and a half months after its onset, with the development of rapidly progressive dementia and generalized myoclonus, AHS gradually disappeared. Three months after the AHS onset, MRI with DWI showed restricted diffusion within the cortex involving the cingulated gyrus and bilateral temporal lobes, which was more prominent on the right than on the left side. Four months after the AHS onset, 14-3-3 protein level of the cerebrospinal fluid was elevated, and EEG recordings showed diffuse slowing of basic activity with periodic complexes. The patient was clinically diagnosed as having CJD. The patient died of pneumonia four and a half months after the AHS onset. AHS has rarely been reported in patients with CJD. Our case illustrates the importance of considering CJD in the differential diagnosis, if the patient showed AHS, even with normal MRI findings.

The alien hand: cases, categorizations, and anatomical correlates.

The clinical characteristics and neuroanatomical damage reported in more than 50 published cases of observed "alien-hand" signs are reviewed. The terms alien-hand sign and alien-hand syndrome describe phenomena experienced by patients in which an upper limb performs complex motor activities outside of volitional control. The categories of frontal and callosal subtypes and their relation to behavior and neuropathology are evaluated with reference to the dual premotor system theory, which emphasizes the role of the supplementary motor areas in alien-hand phenomena. Detailed consideration is given to the more recently described posterior subtype, which is purported to result from damage to the parietal lobe or other posterior brain areas. The lack of uniformity in reported assessment methods (behavioral tests, neuroimaging) in published cases contributes to the difficulty in establishing clear subtypes of alien-hand phenomena. Suggestions are made regarding current categorizations and available assessment methods.

Textbook of Clinical Neuropsychiatry

Textbook of Clinical Neuropsychiatry

Examination of motor output pathways in patients with corticobasal ganglionic degeneration using transcranial magnetic stimulation.

The alien hand sign (AHS) is often encountered in patients with corticobasal ganglionic degeneration (CBGD), revealing a unilateral dysfunction of the motor system of unknown pathophysiology. We examined the possibility of an abnormal cortical representation of hand muscles in 10 patients with probable CBGD and a prominent AHS. Cortical maps were obtained from the responses to magnetic stimuli applied with a figure of eight coil at an intensity of 110% above motor threshold. For comparison, the same study was carried out in 10 normal volunteers, eight patients with Parkinson's disease and eight patients with Alzheimer's disease. AHS patients had a larger extension of the cortical map to stimulation of the hemisphere contralateral to the AHS in comparison with the ipsilateral hemisphere. Furthermore, in six patients, focal stimulation of the hemisphere ipsilateral to the AHS gave rise to ipsilateral responses, delayed by a mean of 7.7 +/- 2.2 ms with respect to those recorded in the same muscle to contralateral stimulation. None of the other patients or control subjects had ipsilateral responses. Our results indicate an enhanced excitability, or reduced inhibition, of the motor area of the hemisphere contralateral to the AHS. The delay of the ipsilateral responses is compatible with a disinhibited transcallosal input.

Utilization behavior: clinical manifestations and neurological mechanisms.

This paper describes a variety of motor release phenomena, including manual grasping and groping, imitation behavior, utilization behavior, and alien hand sign, their clinical manifestations. and proposed neural mechanisms. One of these specific neurobehavioral disorders, initially described by Lhermitte (Brain [1983] 106: 237-255), and termed utilization behavior, is addressed in more detail. Patients with this disorder are described as reaching out and using objects in the environment in an automatic manner. The current paper provides a comprehensive review of studies that have documented utilization behavior in individuals with a variety of pathologies, all having a specific predilection for the frontal lobes and frontal-striatal systems. Goldberg's (Behavioral and Brain Sciences [1985] 8: 567-616) theoretical framework for understanding motor release phenomena, which conceptualizes these behaviors as resulting from an imbalance between proposed medial (voluntary, goal directed, and future directed) and lateral (automatic, stimulus bound, and visually based) motor systems, is also discussed. Utilization behavior may prove to be a common underlying cause of high levels of excessive and intrusive motor behaviors within various clinical populations. A more comprehensive understanding of the neural systems underlying utilization behavior may prove highly useful for the differential diagnosis of conditions involving the mesial frontal cortex and fronto-striatal connections. Recent studies have started to investigate utilization behavior in clinical populations with known or suspected frontal system dysfunction, including adult patients with a variety of psychiatric conditions and children with ADHD.

Alien hand sign

Usage of the term "alien hand sign" is reviewed in 20 published cases. It refers to apparently purposeful, upper-extremity movements that the patient reports are beyond his/her control. Lesions were found in medial frontal cortex and/or the corpus callosum with presentation being unilateral and transient, unless bilateral cortical and subcortical structures were involved. There was a high co-occurrence of speech hesitation, limb dyspraxia, tactile dysnomia, muscle weakness, and grasp reflex on the alien side. Five behavioral manifestations of alien movement have been described, with grasping movements and intermanual conflict being the most common. Two manifestations, exaggerated ataxic automatisms and drifting movements, likely represent other types of movement disorders. Dysfunction of the supplementary motor area and related structures has been implicated, but the exact causal mechanism remains uncertain.

The alien hand and related signs.

Alien limb sign includes failure to recognise ownership of one's limb when visual cues are removed, a feeling that one body part is foreign, personification of the affected body part, and autonomous activity which is perceived as outside voluntary control. Although the hand is most frequently affected, any limb or combination of limbs may fulfil the alien limb criteria. Alien hand sign should be reserved for cases in which the hand feels foreign together with observable involuntary motor activity. To characterise this phenomenon, seven patients with alien hand sign and other motor or behavioural manifestations are described. Aetiologies included multiple infarcts and cortobasal ganglionic degeneration (CBD). In this study, all patients had apraxia in response to verbal commands and problems with bimanual coordination. Most displayed non-goal directed involuntary motor activities, and two had self destructive motor behaviours. Grasp reflex occurred with alien hand due to either aetiology. Cortical reflex myoclonus was frequently seen in CBD patients. The phenomenological spectrum is reviewed, a diagnostic protocol proposed, and possible anatomical bases of alien hand discussed.

Left alien hand sign and mirror writing after left anterior cerebral artery infarction

A 43-year-old, right-handed man was admitted complaining of peculiar movements of his left hand. Computed tomography scan and magnetic resonance imaging showed an infarct area in the territory of the left anterior cerebral artery. The abnormal movements of the left hand were diagnosed as so-called alien hand sign. This patient also exhibited mirror writing with his left hand. The clinical manifestations and neuroradiological findings of this case are described in detail, and the possible mechanisms of these extremely rare combinations of alien hand sign and mirror writing are discussed.

Alien Hand Sign and Other Cognitive Deficits following Ruptured Aneurysm of the Anterior Communicating Artery.

We describe a right-handed patient who suffered a ruptured aneurysm of the anterior communicating artery (ACoA) which was clipped successfully. Computerized tomography indicated a low density area in the genu of the corpus callosum and the infero-lateral aspect of the left frontal lobe. On recovery the patient's most notable deficit was the "alien hand sign" whereby the left hand would frequently interfere with the actions of the right hand. Problems in response initiation were also evident. There was significant memory loss and performance was impaired on some tests of frontal lobe function. Discussion centres on the functional locus of the alien hand sign but other aspects of the patient's deficits are also considered.

Alien Hand Sign and Other Cognitive Deficits following Ruptured Aneurysm of the Anterior Communicating Artery

We describe a right-handed patient who suffered a ruptured aneurysm of the anterior communicating artery (ACoA) which was clipped successfully. Computerized tomography indicated a low density area in the genu of the corpus callosum and the infero-lateral aspect of the left frontal lobe. On recovery the patient's most notable deficit was the “alien hand sign” whereby the left hand would frequently interfere with the actions of the right hand. Problems in response initiation were also evident. There was significant memory loss and performance was impaired on some tests of frontal lobe function. Discussion centres on the functional locus of the alien hand sign but other aspects of the patient's deficits are also considered.

The Alien Hand Sign

The alien hand sign was first described by Brion and Jedynak as a "feeling of estrangement between the patient and one of his hands." The affected hand frequently shows a grasp reflex and an instinctive grasp reaction as well as elements of what Denny-Brown referred to as a "magnetic apraxia" associated with frontal lobe damage. Most notably, however, the affected hand is observed to perform apparently purposive behaviors that are perceived as being outside the volitional control of the patient. The patients interpret the behavior of their own affected limb as being controlled by an external agent. They do not feel that they are initiating or controlling the behavior of the hand and often express dismay at the hand's "extravolitional" activity. The patients attempt to control behavior of the alien hand with the unimpaired hand by forcibly restraining the affected limb, an act that may be termed "self-restriction." In this paper, we report an additional four cases of alien hand sign in right-handed subjects: two involving the right hand and two involving the left hand. In each case, the clinical findings were associated with extensive unilateral damage of the medial frontal cortex of the hemisphere contralateral to the affected hand. Furthermore, the alien movement gradually disappears over the course of 6-12 months after the stroke. These clinical case studies are presented and discussed in the context of the "dual premotoer systems hypothesis," an anatomicophysiological model that proposes that action is organized by two separate but interactive premotor brain systems corresponding to evolutionarily defined medial and lateral cortical moieties. It is hypothesized that the alien mode behavior results from unconstrained activity of the lateral premotor system in the damaged hemisphere. The residual volitional control in the limb occurs through the activity of the intact medial premotor system of the ipsilateral hemisphere. Recovery may occur through extension of these ipsilateral control mechanisms by compensatory changes in subcortical systems controlling hemispheric activation associated with adaptive behavior. This observation may be important in understanding mechanisms involved in motor recovery after stroke.