alien limb syndrome

BACKGROUND AND OBJECTIVES: Nonfluent variant primary progressive aphasia (nfvPPA) and primary progressive apraxia of speech (PPAOS) can be precursors to corticobasal syndrome (CBS). Details on their progression remain unclear. We aimed to examine the clinical and neuroimaging evolution of nfvPPA and PPAOS into CBS. METHODS: We conducted a retrospective longitudinal study in 140 nfvPPA or PPAOS patients and applied the consensus criteria for possible and probable CBS for every visit, evaluating limb rigidity, akinesia, limb dystonia, myoclonus, ideomotor apraxia, alien limb phenomenon, and nonverbal oral apraxia (NVOA)...

Alien hand syndrome (AHS) is an uncommon neurological condition characterized by involuntary, yet seemingly purposeful, movements of a limb, typically an upper extremity, with variable awareness and control by the affected individual. It is associated with a range of peculiar sensations, such as the feeling of limb estrangement, alien control and involuntary mirroring or restraining of movements. AHS indicates a profound disruption in volitional motor control and personal agency. The aetiology of AHS is the dysfunction of critical brain regions secondary to diverse neurological insults, such as tumours, vascular disorders, infarction or neurodegenerative diseases...

Alien hand syndrome (AHS) is a rare but significant disorder, characterized by involuntary and often complex movements of one's hand. These movements seem to occur independently and unconsciously, separate from a person's intended actions.  We report a case of a 70-year-old male who presented to the emergency department with left sided hemiplegia following a stroke in the right middle cerebral artery (MCA) that affected the right temporal, parietal and internal capsule region. Upon additional inquiry, the patient reported experiencing involuntary, abrupt movements in his left upper and lower limbs particularly while attempting voluntary movements...

The motor phenomena accompanying frontal lobe disease are diverse, reflecting the various roles the frontal lobes play in the organization of motor control. The principal frontal motor areas, the primary motor cortex, the premotor cortex, and the supplementary motor area, have different but interrelated functions in motor control. The principal efferent pathway of the primary motor cortex is the corticospinal tract which conducts fine motor control. Damage to the primary motor cortex and the corticospinal tract results in paralysis and loss of skilled, particularly distal, motor function...

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...

INTRODUCTION: One of the strangest kinds of misperceptions of the body is "Body Integrity Dysphoria" (BID), formerly named as "Body Identity Integrity Disorder" (BIID). The affected people have the feeling that a limb is not part of their body. They can feel it, they can use it, they can move it, but they cannot get along with the fact that it is a part of their own body. Most feel the need for an amputation of a leg, others of both legs, some want a palsy and use a wheelchair. Still discussed is whether other disablements such as blindness, dumbness, deafness or a desire to have an incontinency can be included in this diagnosis...

In Germany, around 1.8 million people currently suffer from dementia and the numbers are increasing. The main cause of dementia is Alzheimer's disease. This is classically manifested in the form of an amnestic syndrome but also encompasses various atypical variants, especially in younger patients and in the clinical routine are not always easy to recognize. These are described in this narrative review with case studies. Posterior cortical atrophy (PCA) presents with visual disorders, in the logopenic variants of primary progressive aphasia (lvPPA) impaired word retrieval is the main symptom, in the frontal variant of Alzheimer's disease behavioral disorders are prominent and in corticobasal syndrome (CBS) an akinetic rigid Parkinson's syndrome with alien limb phenomenon...

Our aim was to analyze the phenotypic-genetic correlations in a patient diagnosed with early onset corticobasal syndrome with progressive non-fluent aphasia (CBS-PNFA), characterized by predominant apraxia of speech, accompanied by prominent right-sided upper-limb limb-kinetic apraxia, alien limb phenomenon, synkinesis, myoclonus, mild cortical sensory loss, and right-sided hemispatial neglect. Whole-exome sequencing (WES) identified rare single heterozygous variants in ATP7B (c.3207C>A), SORL1 (c.352G>A), SETX (c...

Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive neurodegenerative disorder that has an invariably fatal outcome. Aside from rapidly progressive dementia, this condition manifests as myoclonus, cerebellar, pyramidal, extrapyramidal, visual, and psychiatric symptoms. On the other hand, nonspecific symptoms might be difficult to diagnose, leading to a late or incorrect diagnosis. Given its high fatality, most patients die within 12 months of the disease's onset. This case report describes a healthy man who presented with cerebellar and pyramidal signs along with memory loss worsening over six weeks...

Corticobasal syndrome (CBS) is a clinical syndrome characterised by progressive asymmetric limb rigidity and apraxia with dystonia, myoclonus, cortical sensory loss and alien limb phenomenon. Corticobasal degeneration (CBD) is one of the most common underlying pathologies of CBS, but other disorders, such as progressive supranuclear palsy (PSP), Alzheimer's disease (AD) and frontotemporal lobar degeneration with TDP-43 inclusions, are also associated with this syndrome.In this review, we describe common and rare neuropathological findings in CBS, including tauopathies, synucleinopathies, TDP-43 proteinopathies, fused in sarcoma proteinopathy, prion disease (Creutzfeldt-Jakob disease) and cerebrovascular disease, based on a narrative review of the literature and clinicopathological studies from two brain banks...

The alien limb is a phenomenon characterized by a cluster of clinical features wherein the limb behaves autonomously and as separated from a person's identity. We herein report a 36-year-old Indian female with multiple comorbidities who presented with recurrent episodes of limb-shaking transient ischemic attacks for 1 year, followed by left-sided hemiplegia. During recovering, the patient noticed a feeling that as if her left hand did not belong to herself and acted autonomously (alien limb phenomenon) along with visuospatial deficits...

Hereditary diffuse leukoencephalopathy with spheroids is a rare genetic disorder caused by mutations of the colony-stimulating factor 1 receptor gene. It is an adult-onset leukodystrophy, with a wide spectrum of neurological and psychiatric manifestations that includes Parkinsonism, dementia, seizures, limb weakness, spasticity and abnormal motor behaviour. Alien-hand syndrome and mirror movements are rare manifestations of this and other neurodegenerative disorders. We describe a woman with progressive limb and trunk rigidity, Parkinsonism and dementia, who also had involuntary left arm levitation (part of the posterior variant of alien-hand syndrome) and left-hand mirror movements...

Several variants of the TANK-Binding Kinase 1 ( TBK1 ) gene have been associated with frontotemporal dementia - amyotrophic lateral sclerosis (FTD-ALS) spectrum diseases. Corticobasal syndrome (CBS) is characterized by asymmetric limb rigidity, dystonia or myoclonus, in association with speech or limb apraxia, cortical sensory deficit, and/or alien limb. It can result from a variety of underlying pathologies and although typically sporadic, it has been occasionally associated with MAPT and GRN variants. We describe here the proband of a family with multiple occurrences of FTD-ALS spectrum disease who developed an isolated right-sided primary asymmetric akinetic-rigid syndrome and subsequent speech and cognitive dysfunction associated with contralateral anterior temporal lobe atrophy on MRI and corresponding hypometabolism by FDG-PET...

BACKGROUND: Variants of the TANK-Binding Kinase 1 (TBK1) gene have been associated with frontotemporal dementia - amyotrophic lateral sclerosis (FTD-ALS) spectrum diseases. Corticobasal syndrome (CBS), characterized by asymmetric limb rigidity, dystonia or myoclonus, in association with speech or limb apraxia, cortical sensory deficit, and/or alien limb can result from a variety of underlying pathologies. It is most commonly sporadic, although it has been rarely described in association with genetic defects, especially MAPT and GRN genes...

BACKGROUND AND PURPOSE: Patients with a corticobasal syndrome (CBS) present a rare form of atypical parkinsonism characterized by asymmetric clinical symptoms and progressive motor and nonmotor impairment, such as apraxia, alien limb phenomenon, aphasia, myoclonus, dystonia, and cognitive impairment. At early stages, clinical differentiation between CBS and idiopathic Parkinson's disease (IPD) can be challenging. METHODS: Using high-resolution T1-weighted images and voxel-based morphometry (VBM), we sought to identify disease-specific patterns of brain atrophy in a small sample of CBS and IPD patients at early stages of disease...

While corticobasal syndrome (CBS) has long been associated with corticobasal degeneration (CBD), only 24-57% of CBS patients will have the classic histopathologic findings of CBD postmortem. Here, we present a 28-year-old male who had a 3-year history of progressive right sided predominant, atypical parkinsonism, limb dystonia, stimulus sensitive myoclonus, apraxia, aphasia, alien limb phenomenon, and cognitive impairment, typical of CBS, who, based on Armstrong criteria will qualify as possible CBD. In conclusion, among young patients presenting with CBS, tauopathies are still the most common causes, but inherited metabolic and white matter diseases as well as other non-tau associated neurodegenerative conditions should also be ruled out...

Consensus criteria on corticobasal degeneration (CBD) include alien limb (AL) phenomena. However, the gist of the behavioral features of AL is still "a matter of debate." CBD-related AL has so far included the description of involuntary movements, frontal release phenomena (frontal AL), or asomatognosia (posterior or "real" AL). In this context, the most frequent symptoms are language and praxis deficits and cortical sensory misperception. However, asomatognosia requires, by definition, intact perception and cognition...

BACKGROUND: Over 80% of the global population consider themselves religious, with even more identifying as spiritual, but the neural substrates of spirituality and religiosity remain unresolved. METHODS: In two independent brain lesion datasets (N1  = 88; N2  = 105), we applied lesion network mapping to test whether lesion locations associated with spiritual and religious belief map to a specific human brain circuit. RESULTS: We found that brain lesions associated with self-reported spirituality map to a brain circuit centered on the periaqueductal gray...

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...

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...