scholarly journals Alien Limb Syndrome Responsive to Amantadine in a Patient with Corticobasal Syndrome

2015 ◽  
Vol 5 (0) ◽  
pp. 309 ◽  
Author(s):  
Francisco de Assis Aquino Gondim ◽  
José Wagner Leonel Tavares Júnior ◽  
Arlindo A. Morais ◽  
Paulo Marcelo Gondim Sales ◽  
Horta Goes Wagner
Keyword(s):  
Corticobasal Syndrome ◽  
Alien Limb

scholarly journals Network Localization of Alien Limb in Patients with Corticobasal Syndrome

2020 ◽  
Vol 88 (6) ◽  
pp. 1118-1131
Author(s):  
Aaron M. Tetreault ◽  
Tony Phan ◽  
Kalen J. Petersen ◽  
Daniel O. Claassen ◽  
Byran J. Neth ◽  
...  
Keyword(s):  
Corticobasal Syndrome ◽  
Network Localization ◽  
Alien Limb

scholarly journals Corticobasal syndrome: a practical guide

2021 ◽  
Vol 21 (4) ◽  
pp. 276-283
Author(s):  
Duncan Wilson ◽  
Campbell Le Heron ◽  
Tim Anderson
Keyword(s):  
Prognostic Significance ◽  
Corticobasal Degeneration ◽  
Poor Response ◽  
Corticobasal Syndrome ◽  
Language Therapist ◽  
Alien Limb ◽  
Wide Range ◽  
Extrapyramidal Features ◽  
Underlying Pathology ◽  
Insight Into

Corticobasal syndrome is a disorder of movement, cognition and behaviour with several possible underlying pathologies, including corticobasal degeneration. It presents insidiously and is slowly progressive. Clinicians should consider the diagnosis in people presenting with any combination of extrapyramidal features (with poor response to levodopa), apraxia or other parietal signs, aphasia and alien-limb phenomena. Neuroimaging showing asymmetrical perirolandic cortical changes supports the diagnosis, while advanced neuroimaging may give insight into the underlying pathology. Identifying corticobasal syndrome carries some management implications (especially if protein-based treatments arise in the future) and prognostic significance. Its treatment is largely symptomatic and is best undertaken within a multidisciplinary setting, including a neurologist, physiotherapist, occupational therapist, speech language therapist, psychiatrist and, ultimately, a palliative care clinician. Corticobasal syndrome can be a confusing entity for neurologists, not least because it has over time evolved from being considered predominantly as a movement disorder to a condition spanning a wide range of cognitive and motor manifestations. In this practical review, we attempt to disentangle this syndrome and provide clarity around diagnosis, its underlying pathological substrates, key clinical features and potential treatments.

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

2020 ◽  
Vol 267 (4) ◽  
pp. 1147-1157 ◽  
Author(s):  
David J. Lewis-Smith ◽  
Noham Wolpe ◽  
Boyd C. P. Ghosh ◽  
James B. Rowe
Keyword(s):  
Positive Association ◽  
Theoretical Models ◽  
Corticobasal Syndrome ◽  
Voluntary Action ◽  
Control Group ◽  
Left Hand ◽  
Alien Limb ◽  
Limb Apraxia ◽  
Low Probability ◽  
And Control

AbstractAlien 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.

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

2019 ◽  
Author(s):  
David J. Lewis-Smith ◽  
Noham Wolpe ◽  
Boyd C.P. Ghosh ◽  
James B. Rowe
Keyword(s):  
Positive Association ◽  
Theoretical Models ◽  
Corticobasal Syndrome ◽  
Voluntary Action ◽  
Control Group ◽  
Left Hand ◽  
Alien Limb ◽  
Limb Apraxia ◽  
Low Probability ◽  
And Control

ABSTRACTAlien 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 77% of patients. Moreover, 83% 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 both the occurrence of an alien limb and 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 severe 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.

Reply: Sensory alien limb syndrome in stroke and corticobasal syndrome

2011 ◽  
Vol 26 (7) ◽  
pp. 1365-1366
Author(s):  
Julien Delrieu ◽  
Pierre Payoux ◽  
Thierry Voisin
Keyword(s):  
Corticobasal Syndrome ◽  
Alien Limb

scholarly journals Unraveling corticobasal syndrome and alien limb syndrome with structural brain imaging

Cortex ◽  
2019 ◽  
Vol 117 ◽  
pp. 33-40 ◽  
Author(s):  
Franziska Albrecht ◽  
Karsten Mueller ◽  
Tommaso Ballarini ◽  
Leonie Lampe ◽  
Janine Diehl-Schmid ◽  
...  
Keyword(s):  
Brain Imaging ◽  
Corticobasal Syndrome ◽  
Alien Limb ◽  
Structural Brain ◽  
Structural Brain Imaging

Sensory alien limb syndrome in stroke and corticobasal syndrome

2011 ◽  
Vol 26 (7) ◽  
pp. 1365-1365
Author(s):  
Francisco de Assis Aquino Gondim
Keyword(s):  
Corticobasal Syndrome ◽  
Alien Limb

Mills’ syndrome (ascending spastic hemiparesis) progressing to corticobasal syndrome

2021 ◽  
pp. 1-6
Author(s):  
Ario Mirian ◽  
Jonathan Romsa ◽  
Mary E. Jenkins ◽  
Adrian Budhram
Keyword(s):  
Corticobasal Syndrome ◽  
Spastic Hemiparesis ◽  
Mills Syndrome

scholarly journals Unravelling Genetic Factors Underlying Corticobasal Syndrome: A Systematic Review

Cells ◽  
2021 ◽  
Vol 10 (1) ◽  
pp. 171
Author(s):  
Federica Arienti ◽  
Giulia Lazzeri ◽  
Maria Vizziello ◽  
Edoardo Monfrini ◽  
Nereo Bresolin ◽  
...  
Keyword(s):  
Risk Factors ◽  
Systematic Review ◽  
Genetic Risk ◽  
Genetic Factors ◽  
Diagnostic Algorithm ◽  
Behavioral Changes ◽  
Corticobasal Syndrome ◽  
Cochrane Library ◽  
Radiological Features ◽  
Genetically Determined

Corticobasal syndrome (CBS) is an atypical parkinsonian presentation characterized by heterogeneous clinical features and different underlying neuropathology. Most CBS cases are sporadic; nevertheless, reports of families and isolated individuals with genetically determined CBS have been reported. In this systematic review, we analyze the demographical, clinical, radiological, and anatomopathological features of genetically confirmed cases of CBS. A systematic search was performed using the PubMed, EMBASE, and Cochrane Library databases, included all publications in English from 1 January 1999 through 1 August 2020. We found forty publications with fifty-eight eligible cases. A second search for publications dealing with genetic risk factors for CBS led to the review of eight additional articles. GRN was the most common gene involved in CBS, representing 28 out of 58 cases, followed by MAPT, C9ORF72, and PRNP. A set of symptoms was shown to be significantly more common in GRN-CBS patients, including visuospatial impairment, behavioral changes, aphasia, and language alterations. In addition, specific demographical, clinical, biochemical, and radiological features may suggest mutations in other genes. We suggest a diagnostic algorithm to help in identifying potential genetic cases of CBS in order to improve the diagnostic accuracy and to better understand the still poorly defined underlying pathogenetic process.

Ogilvie's syndrome presented with delirium in an older lady with corticobasal syndrome

2021 ◽  
pp. 1-3
Author(s):  
Feyza Mutlay ◽  
Derya Kaya ◽  
Ahmet Turan Isik
Keyword(s):  
Palliative Care ◽  
Health Care Professionals ◽  
Colonic Obstruction ◽  
Holistic Approach ◽  
Abdominal Distension ◽  
Corticobasal Syndrome ◽  
Ogilvie's Syndrome ◽  
Geriatric Population ◽  
Ogilvie’S Syndrome ◽  
Neurological Illnesses

Abstract Objective Corticobasal syndrome (CBS) is one of an atypical parkinsonian syndromes characterized by extrapyramidal features as well as cortical involvement signs. A variety of factors may lead to delirium in older adults with chronic progressive life-limiting neurological illnesses like CBS. Ogilvie's syndrome (OS) is an acute colonic pseudo-obstruction in which abdominal distension, nausea, vomiting, and constipation can be seen. We report a case of OS identified as the underlying possible cause of delirium in an 80-year-old woman with CBS. We also discuss the importance of holistic approach which is essential to manage the underlying cause and to preserve the quality of life in particular for the frail geriatric population who potentially needs palliative care or already benefits from palliative care. Method An older patient with CBS presented with symptoms similar to that of acute colonic obstruction and subsequently developed delirium. The patient was found to have colonic pseudo-obstruction (OS). Result Neostigmin infusion was therefore given to treat it and delirium was resolved. Significance of results To the best of our knowledge, clinical manifestation of delirium as OS in a patient with CBS has not been previously reported. OS may be superimposed to CBS in older patients, and OS in such patients may play a role as a precipitating factor for the development of delirium. Given the fact that CBS is progressive and rare neurodegenerative disease and almost all of these patients need palliative care, eventually, health-care professionals, especially in palliative care, should be aware of distinctive challenges of life-limiting chronic neurological illnesses, such as conditions that may lead to the development of acute colonic pseudo-obstruction because the rapid treatment of them prevents the use of potentially harmful drugs, surgical procedures, or inappropriate interventions.

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