Alien hand syndrome, a rare presentation of corpus callosum and cingulate infarction.

Alien hand syndrome (AHS) is a rare apraxia syndrome, characterized by involuntary and uncontrollable movements of one upper limb, often accompanied by intermanual conflict. Damage to the corpus callosum, acute infarction and neurodegenerative disease may result in AHS. Based on the presentation and impairment region, AHS has three variants: frontal, callosal and posterior. Each type may have a different clinical presentation. A total of 157 patients admitted to hospital with corpus callosum infarction between 2012 and 2022 were included for this study, of whom a number of 5 presented with AHS. 4 of them had significant symptoms of intermanual conflict and 1 had strong grip symptoms in the affected upper limb. Moreover, new infarcts involving the corpus callosum and cingulate gyrus were found on MRI in all five patients. We simultaneously performed a retrospective study on all reported AHS cases caused by infarction of the corpus callosum. Case reports and literature reviews were conducted in order to provide clinicians with a better understanding of AHS, its etiology, clinical presentation, diagnosis, and treatment.

Alien hand syndrome in ruptured aneurysms: case report and review of the literature.

Alien hand syndrome is a rare condition associated with lesions of the corpus callosum and of the supplementary motor area, which can be caused by ruptured cerebral aneurysms. We present a novel case of a patient with a subarachnoid haemorrhage from an aneurysm of the anterior communicating artery who developed alien hand syndrome and intermanual conflict. In addition, we performed a systematic literature search and evaluated data on clinical presentation, treatment and radiological findings from relevant papers. To date, 17 cases of alien hand syndrome in aneurysmatic subarachnoid haemorrhage have been reported. Aneurysms of the anterior communicating artery were the most common origin of subarachnoid haemorrhage (10/17), followed by pericallosal artery aneurysms (7/10). The prognosis appears to be favourable; however, damage to the supplementary motor area during treatment should be avoided to keep symptoms minimal.

Dr. Strangelove demystified: Disconnection of hand and language dominance explains alien-hand syndrome after corpus callosotomy.

BACKGROUND: Alien hand syndrome (AHS) is a disabling condition in which one hand behaves in a way that the person finds "alien". This feeling of alienation is related to the occurrence of movements of the respective hand performed without or against conscious intention. Most information on AHS stems from single case observations in patients with frontal, callosal, or parietal brain damage. METHODS: Retrospective analysis of distinctive clinical features of three out of 18 epilepsy patients who developed AHS with antagonistic movements of the left hand after corpus callosotomy (CC) (one anterior, two complete) for the control of epileptic seizures, particularly epileptic drop attacks (EDA). RESULTS: Remarkably, these three patients, two men and one woman, displayed atypical language dominance with a bilateral, left more than right hemisphere language representation in intracarotidal amobarbital testing before surgery. The overall additional distinctive feature of the target patients was genuine left-handedness, with writing retrained to right-handedness in two patients. After surgery the left hands became alien. The problem was permanent, despite strategies for compensation. CONCLUSION: From this observation we suggest that under the conditions of dissociation of language and motor dominance, loss of both intentional control of contralateral action and physiological inhibition of antagonistic movements lead to post-callosotomy alien-hand-like motor phenomena. The dissociation pattern posing this risk seems rare but needs to be considered when evaluating candidates for callosotomy.

[Alien hand syndrome is a rare symptom of stroke].

Alien hand syndrome (AHS) is a rare condition, which is defined as involuntary meaningful movements of a limb associated with loss of ownership over the limb. It affects mostly the left hand. Corticobasal degeneration, stroke and Creutzfeldt-Jakob disease are the three leading causes of AHS. AHS is classically divided into three subcategories: frontal, callosal and posterior. Loss of inhibitory tone and loss of bilateral hemisphere activation are the hypothesised pathophysiologic causes of AHS. This is a case report of a posterior AHS in a 47-year-old woman after stroke in the right parietal lobe.

Network Localization of Alien Limb in Patients with Corticobasal Syndrome.

OBJECTIVE: Perirolandic atrophy occurs in corticobasal syndrome (CBS) but is not specific versus progressive supranuclear palsy (PSP). There is heterogeneity in the locations of atrophy outside the perirolandic cortex and it remains unknown why atrophy in different locations would cause the same CBS-specific symptoms. In prior work, we used a wiring diagram of the brain called the human connectome to localize lesion-induced disorders to symptom-specific brain networks. Here, we use a similar technique termed "atrophy network mapping" to localize single-subject atrophy maps to symptom-specific brain networks. METHODS: Single-subject atrophy maps were generated by comparing cortical thickness in patients with CBS versus controls. Next, we performed seed-based functional connectivity using a large normative connectome to determine brain regions functionally connected to each patient's atrophied locations. RESULTS: Patients with CBS had perirolandic atrophy versus controls at the group level, but locations of atrophy in CBS were heterogeneous outside of the perirolandic cortex at the single-subject level (mean spatial correlation = 0.04). In contrast, atrophy occurred in locations functionally connected to the perirolandic cortex in all patients with CBS (spatial correlation = 0.66). Compared with PSP, patients with CBS had atrophy connected to a network of higher-order sensorimotor regions beyond perirolandic cortex, matching a CBS atrophy network from a recent meta-analysis. Finally, atrophy network mapping identified a symptom-specific network for alien limb, matching a lesion-induced alien limb network and a network associated with agency in healthy subjects. INTERPRETATION: We identified a syndrome-specific network for CBS and symptom-specific network for alien limb using single-subject atrophy maps and the human connectome. ANN NEUROL 2020;88:1118-1131.

Rehabilitation of Alien Hand Syndrome Complicated by Contralateral Limb Apraxia.

A previously independent 66-yr-old right-handed man presented with right-sided weakness, preferring the lower limbs with additional impaired gait and dysarthria for 1-day duration. Imaging found a large left hemispheric anterior cerebral artery ischemic infarction with multiple lacunar infarcts. He exhibited frontal, callosal, and posterior variants of alien hand syndrome, which impeded activities of daily living. Though limited in evidence, a trial of clonazepam was initiated based on previous case reports describing suspected efficacy. Botulinum toxin A was not used given the patient's immediate need and limited hospital length of stay. Right upper limb constricting therapies improved intermanual conflict and spontaneous grasping and levitation (arm elevation in retroflexion) activity; however, concomitant left upper limb motor apraxia complicated task-oriented activities. The combination of pharmaceutical and therapeutic interventions improved the patient's quality of life as assessed by clinical observation, functional independence measures from 41 to 57, and patient reporting. This case report aims to increase awareness of a potential barrier to rehabilitation of a debilitating and rare condition and to discuss current assessment tools and treatment options supported by available evidence.

Alien limb syndrome: A Bayesian account of unwanted actions.

An alien limb is a debilitating disorder of volitional control. The core feature of alien limb is the performance of simple or complex semi-purposeful movements which the patient reports to be unintentional or unwanted, or occasionally in opposition to their intentions. Theories of the mechanism of alien limb phenomena have emphasised the role of disinhibition in the brain, and exaggerated action 'affordances'. However, despite advances in cognitive neuroscience research and a large public and media interest, there has been no unifying computational and anatomical account of the cause of alien limb movements. Here, we extend Bayesian brain principles to propose that alien limb is a disorder of 'predictive processing' in hierarchical sensorimotor brain networks. Specifically, we suggest that alien limb results from predictions about action outcomes that are afforded unduly high precision. The principal mechanism for this abnormally high precision is an impairment in the relay of input from medial regions, predominantly the supplementary motor area (SMA), which modulate the precision of lateral brain regions encoding the predicted action outcomes. We discuss potential implications of this model for future research and treatment of alien limb.

Alien limb in the corticobasal syndrome: phenomenological characteristics and relationship to apraxia.

Alien limb refers to movements that seem purposeful but are independent of patients' reported intentions. Alien limb often co-occurs with apraxia in the corticobasal syndrome, and anatomical and phenomenological comparisons have led to the suggestion that alien limb and apraxia may be causally related as failures of goal-directed movements. Here, we characterised the nature of alien limb symptoms in patients with the corticobasal syndrome (n = 30) and their relationship to limb apraxia. Twenty-five patients with progressive supranuclear palsy Richardson syndrome served as a disease control group. Structured examinations of praxis, motor function, cognition and alien limb were undertaken in patients attending a regional specialist clinic. Twenty-eight patients with corticobasal syndrome (93%) demonstrated significant apraxia and this was often asymmetrical, with the left hand preferentially affected in 23/30 (77%) patients. Moreover, 25/30 (83%) patients reported one or more symptoms consistent with alien limb. The range of these phenomena was broad, including changes in the sense of ownership and control as well as unwanted movements. Regression analyses showed no significant association between the severity of limb apraxia and either the occurrence of an alien limb or the number of alien limb phenomena reported. Bayesian estimation showed a low probability for a positive association between alien limb and apraxia, suggesting that alien limb phenomena are not likely to be related to severity apraxia. Our results shed light on the phenomenology of these disabling and as yet untreatable clinical features, with relevance to theoretical models of voluntary action.

Intermittent alien hand syndrome caused by Marchiafava-Bignami disease: A case report.

RATIONALE: Alien Hand syndrome (AHS) is characterized in most patients by seemingly purposeful, involuntary movements of the extremities. It is not well known among physicians on account of its diverse clinical manifestations. PATIENT CONCERNS: We present a 57-year-old Chinese man who could not stop or turn himself around as he involuntarily and uncontrollably walked forward, which had happened frequently in the month prior to treatment. He had been a heavy drinker for thirty years before the onset of the disease, with an alcohol intake of 600 to 800 ml/day. DIAGNOSES: History of alcohol intake and the brain magnetic resonance imaging findings indicated a diagnosis of Marchiafava-Bignami disease. The patient was additionally diagnosed with Alien Hand Syndrome according to his clinical symptoms. INTERVENTIONS: The patient was treated with high doses of vitamin B for 1 month. OUTCOMES: The patient's abnormal behaviors never appeared during the treatment, and no instance of recurrence was observed during the 6 months of follow-up. LESSONS: The clinical manifestation of AHS is non-specific. Only by considering its diverse manifestation can doctors better understand the disease and achieve early intervention.

Unraveling corticobasal syndrome and alien limb syndrome with structural brain imaging.

Alien limb phenomenon is a rare syndrome associated with a feeling of non-belonging and disowning toward one's limb. In contrast, anarchic limb phenomenon leads to involuntary but goal-directed movements. Alien/anarchic limb phenomena are frequent in corticobasal syndrome (CBS), an atypical parkinsonian syndrome characterized by rigidity, akinesia, dystonia, cortical sensory deficit, and apraxia. The structure-function relationship of alien/anarchic limb was investigated in multi-centric structural magnetic resonance imaging (MRI) data. Whole-group and single-subject comparisons were made in 25 CBS and eight CBS-alien/anarchic limb patients versus controls. Support vector machine was used to see if CBS with and without alien/anarchic limb could be distinguished by structural MRI patterns. Whole-group comparison of CBS versus controls revealed asymmetric frontotemporal atrophy. CBS with alien/anarchic limb syndrome versus controls showed frontoparietal atrophy including the supplementary motor area contralateral to the side of the affected limb. Exploratory analysis identified frontotemporal regions encompassing the pre-/and postcentral gyrus as compromised in CBS with alien limb syndrome. Classification of CBS patients yielded accuracies of 79%. CBS-alien/anarchic limb syndrome was differentiated from CBS patients with an accuracy of 81%. Predictive differences were found in the cingulate gyrus spreading to frontomedian cortex, postcentral gyrus, and temporoparietoocipital regions. We present the first MRI-based group analysis on CBS-alien/anarchic limb. Results pave the way for individual clinical syndrome prediction and allow understanding the underlying neurocognitive architecture.

[Right parietal cerebral infarction with symptoms challenging to differentiate between alien hand sign and sensory ataxia: a case report].

We report the case of a 73-year-old right-handed female with a right parietal cerebral infarction and presented symptoms that were challenging to differentiate between alien hand sign (AHS) and sensory ataxia. She presented to our emergency department with chief complaints of abnormal involuntary movements and a feeling of foreignness on her left upper limb. The first neurological examination revealed left spatial neglect, left-side sensory impairment that included superficial and deep sensations, left limb-kinetic apraxia, and left limb ataxia. Furthermore, her symptoms and complaints had characteristics of AHS that includes a sensation that her left upper limb dose not belong to herself and an abnormal behavior of left hand that is contrary to her own intent. Brain MRI revealed an acute cerebral infarction confined to the right postcentral gyrus. This case highlights that sensory ataxia due to the disturbance of deep sensation might present symptoms similar to AHS. Previous studies suggested the involvement of the disturbance of somatosensory pathway in posterior-variant AHS. Therefore, a precise distinction between AHS and sensory ataxia, especially in posterior-variant AHS, is imperative to avoid confusion regarding the term "alien hand sign."

Alien hand syndrome and migraine with aura: A case report.

BACKGROUND: Alien Hand Syndrome (AHS) is an uncontrollable, involuntary, but in appearance, purposeful motor control disorder of the upper extremity. CASE REPORT: A 42-year-old male patient was admitted to our clinic complaining of involuntary motor activity in his right hand. He had a previous history of migraine with visual aura. The uncontrollable motor control disorder was compatible with Alien Hand Syndrome, which was appearing immediately after the visual aura and before the beginning of headache. CONCLUSION: Alien Hand Syndrome is usually observed with anterior cerebral artery infarction, midline tumors, trauma and several neurodegenerative diseases, but is rarely seen in paroxysmal conditions such as migraine with aura.

Progressive multifocal leukoencephalopathy in the absence of immunosuppression.

A 69-year-old woman presented with a cortical hand syndrome progressing over several weeks. MRI brain showed characteristic appearances of progressive multifocal leukoencephalopathy (PML), confirmed by detection of the JC virus in CSF, despite the absence of any evidence of immunosuppression. Treatment with mirtazapine, mefloquine and cidofovir did not affect the progression of the disease, which was fatal within 7 months of presentation. This report adds to the small case literature that suggests that PML can occur in immunocompetent people, albeit extremely rarely.

Arm Levitation as Initial Manifestation of Creutzfeldt-Jakob Disease: Case Report and Review of the Literature.

Background: Arm levitation is an involuntary elevation of the upper limb, a manifestation of the alien-limb phenomenon. It has rarely been reported in Creutzfeldt-Jakob disease (CJD), less so as an initial manifestation. Case Report: We report a 56-year-old right-handed man with rapidly progressive gait ataxia and involuntary elevation of the left upper limb. During the next few weeks, the patient developed cognitive impairment, apraxia, visual hallucinations, and myoclonus. He met diagnostic criteria for CJD. We evaluated additional published cases of early-appearance of alien-limb phenomenon in the context of CJD; there were 22 such cases and alien-limb phenomenon was the first and exclusive manifestation in only five of them. Discussion: Arm levitation may be a distinct presentation of CJD, appearing earlier than other clinical features.