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.     

Subjective experience, involuntary movement, and posterior alien hand syndrome

The alien hand syndrome, as originally defined, was used to describe cases involving anterior corpus callosal lesions producing involuntary movement and a concomitant inability to distinguish the affected hand from an examiner's hand when these were placed in the patient's unaffected hand. In recent years, acceptable usage of the term has broadened considerably, and has been defined as involuntary movement occurring in the context of feelings of estrangement from or personification of the affected limb or its movements. 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/subcortical areas. A patient with posterior alien hand syndrome of vascular aetiology is reported and the findings are discussed in the light of a conceptualization of posterior alien hand syndrome as a disorder which may be less associated with specific focal neuropathology than are its callosal and callosal-frontal counterparts.     

Involuntary hand levitation associated with parietal damage: another alien hand syndrome

The alien hand syndrome (AHS) usually consists of an autonomous motor activity perceived as an involuntary and purposeful movement, with a feeling of foreignness of the involved limb, commonly associated with a failure to recognise ownership of the limb in the absence of visual clues. It has been described in association to lesions of the frontal lobes and corpus callosum. However, parietal damage can promote an involuntary, but purposeless, hand levitation, which, sometimes, resembles AHS. In the present study, four patients (cortico-basal ganglionic degeneration - n=2; Alzheimer's disease - n=1 and parietal stroke - n=1) who developed alien hand motor behaviour and whose CT, MRI and/or SPECT have disclosed a major contralateral parietal damage or dysfunction are described. These results reinforce the idea that parietal lobe lesions may also play a role in some patients with purposeless involuntary limb levitation, which is different from the classic forms of AHS.     

异己手综合征7例临床及影像学分析

目的:探讨异己手综合征(AHS)的病因、临床表现、影像学特点以及预后转归.方法:回顾性分析7例AHS患者的临床及影像学资料.结果:以胼胝体型(6/7例)最为多见,主要表现为非优势手的对抗失用或手间冲突(4/6例),71.4%患者由脑梗死所致,6例患者存在胼胝体病变;5例患者在对因治疗1个月时有不同程度好转.结论:AHS临床表现复杂多样,影像学检查对AHS的病因诊断、指导治疗具有重要意义.     

Waking up the alien hand: rubber hand illusion interacts with alien hand syndrome

It has been shown that combinations of visual, tactile, and proprioceptive manipulations in healthy subjects may elicit illusory feelings of embodiment (the rubber hand illusion and the somatic rubber hand illusion). We report a case of alien hand syndrome in which the alien hand interacted with the somatic rubber hand illusion to provoke a very strong movement of the alien hand. This effect could be reliably replicated at every application of the experimental procedure. Thus, the illusion seemed to wake up the alien hand. The results demonstrate that the alien hand syndrome can be affected by experimentally induced bodily illusions, which are based on the manipulation of touch and proprioceptive information.     

Paroxysmal alien hand syndrome.

Four patients are described who presented with a paroxysmal form of the alien hand syndrome. Two patients with damage to one frontomedial cortex had brief episodes of abnormal motor behaviour of the contralateral arm that featured groping, grasping, and apparently purposeful but perseverative movements, which both patients interpreted as alien or foreign. The other two patients, with posterior parietal damage, reported a paroxysmal feeling of unawareness of the location of the contralateral arm, lack of recognition of the arm as their own, purposeless movements, and personification of the arm. These cases represent a new form of the alien hand syndrome manifested by brief, paroxysmal episodes, which may be due to ictal mechanisms.     

Alien hand syndrome: contradictive movement and disorder of color discrimination

A senile Chinese female patient with alien hand syndrome of vascular etiology is reported. This case exhibited contradictive movement, left-lateral paresis and disorder of color discrimination, which might be a new subtype of the alien limb 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.     

The alien hand syndrome: classification of forms reported and discussion of a new condition

Abstract. The term alien hand refers to a variety of clinical conditions whose common characteristic is the uncontrolled behavior or the feeling of strangeness of one extremity, most commonly the left hand. Acommon classification distinguishes between the posterior or sensory form of the alien hand, and the anterior or motor form of this condition. However, there are inconsistencies, such as the phenomenon of diagonistic dyspraxia, which is largely a motor syndrome despite being more frequently associated with posterior callosal lesions. We discuss critically the existing nomenclature and we also describe a case recently reported by us which does not fit any previously reported condition, termed agonistic dyspraxia. We propose that the cases of alien hand described in the literature can be classified into at least five broad categories: (i) diagonistic dyspraxia and related syndromes, (ii) alien hand, (iii) way-ward hand and related syndromes, (iv) supernumerary hands and (v) agonistic dyspraxia.     

Alien hand syndrome without a corpus callosum lesion.

A 58 year old right handed woman developed a left alien hand syndrome after a right capsulothalamic haemorrhage with mesencephalic extension. No lesion was detected in the corpus callosum. Positron emission tomography suggested that right cortical dysfunction might account for the absence of recognition of the left hand whereas involuntary motor activity was attributed to the lesion itself. This case shows that the alien hand syndrome is not always the expression of a disconnection syndrome and may occur after a large, strategically located, posterior lesion.     

"Ictal" Alien Hand Syndrome

The alien hand syndrome is rare, occurring with lesions in the mesial frontal lobe area and corpus callosum. We report an unusual presentation of seizures in a 65-year-old woman with a presumed low-grade glioma involving the mesial frontal lobe region, with the "alien hand" seen only during a seizure. Neuropsychological evaluation revealed lateralized cognitive dysfunction consistent with the location of the lesion. There were no interictal indications of disconnection syndrome. The mechanism postulated is disruption of transcallosal motor connections due to the propagating seizure across nondominant motor regions.     

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.     

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.     

Four "alien" hands for two hands after a lesion in corpus callosum

Callosal lesions, associated or not to internal frontal lesions, may produce different types of complex gestural behaviors. Four signs can be identified, each of which has been generally reported separately: the "alien hand" sign, the "diagnostic apraxia", the "wayward hand" and the "callosal apraxia". Some authors justify considering these signs as different entities, while others propose regrouping them either in an unique syndrome--the "alien hand"--or as two syndromes--the "frontal alien hand" and the "callosal alien hand". We present the observation of a patient who presented with the four mentioned syndromes in association. In this context, we review the clinical features of each of the four signs and the arguments supporting their individualization.     

Exaggerated object affordance and absent automatic inhibition in alien hand syndrome

Patients with alien hand syndrome (AHS) experience making apparently deliberate and purposeful movements with their hand against their will. However, the mechanisms contributing to these involuntary actions remain poorly understood. Here, we describe two experimental investigations in a patient with corticobasal syndrome (CBS) with alien hand behaviour in her right hand. First, we show that responses with the alien hand are made significantly more quickly to images of objects which afford an action with that hand compared to objects which afford an action with the unaffected hand. This finding suggests that involuntary grasping behaviours in AHS might be due to exaggerated, automatic motor activation evoked by objects which afford actions with that limb. Second, using a backwards masked priming task, we found normal automatic inhibition of primed responses in the patient's unaffected hand, but importantly there was no evidence of such suppression in the alien limb. Taken together, these findings suggest that grasping behaviours in AHS may result from exaggerated object affordance effects, which might potentially arise from disrupted inhibition of automatically evoked responses.     

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.     

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.     

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.     

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.     

感觉症状与Tourette综合征预后的关系

目的：分析Tourette综合征感觉症状对预后的影响.方法：随机选取Tourette综合征男性患儿120例,使用感觉性症状问卷进行评定,其中伴有感觉症状的患儿66例,不伴感觉症状的患儿54例,采用耶鲁抽动障碍严重程度量表（YGTSS）、抑郁自评量表（SDS）以及焦虑自评量表（SAS）评定,使用氟哌啶醇治疗4周后再次评定.结果：不伴有感觉症状的Tourette综合征患儿较伴有感觉症状的患儿抽动症状好转明显（P＜0.01）;抑郁自评、焦虑自评严重程度轻.结论：Tourette综合征常伴有各种感觉症状,重视感觉症状将对疗效可产生有利的影响.