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Pathophysiology and Treatment of Alien Hand Syndrome

BACKGROUND: Alien hand syndrome (AHS) is a disorder of involuntary, yet purposeful, hand movements that may be accompanied by agnosia, aphasia, weakness, or sensory loss. We herein review the most reported cases, current understanding of the pathophysiology, and treatments. METHODS: We performed a P...

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Autores principales: Sarva, Harini, Deik, Andres, Severt, William Lawrence
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Columbia University Libraries/Information Services 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4261226/
https://www.ncbi.nlm.nih.gov/pubmed/25506043
http://dx.doi.org/10.7916/D8VX0F48
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author Sarva, Harini
Deik, Andres
Severt, William Lawrence
author_facet Sarva, Harini
Deik, Andres
Severt, William Lawrence
author_sort Sarva, Harini
collection PubMed
description BACKGROUND: Alien hand syndrome (AHS) is a disorder of involuntary, yet purposeful, hand movements that may be accompanied by agnosia, aphasia, weakness, or sensory loss. We herein review the most reported cases, current understanding of the pathophysiology, and treatments. METHODS: We performed a PubMed search in July of 2014 using the phrases “alien hand syndrome,” “alien hand syndrome pathophysiology,” “alien hand syndrome treatment,” and “anarchic hand syndrome.” The search yielded 141 papers (reviews, case reports, case series, and clinical studies), of which we reviewed 109. Non-English reports without English abstracts were excluded. RESULTS: Accumulating evidence indicates that there are three AHS variants: frontal, callosal, and posterior. Patients may demonstrate symptoms of multiple types; there is a lack of correlation between phenomenology and neuroimaging findings. Most pathologic and functional imaging studies suggest network disruption causing loss of inhibition as the likely cause. Successful interventions include botulinum toxin injections, clonazepam, visuospatial coaching techniques, distracting the affected hand, and cognitive behavioral therapy. DISCUSSION: The available literature suggests that overlap between AHS subtypes is common. The evidence for effective treatments remains anecdotal, and, given the rarity of AHS, the possibility of performing randomized, placebo-controlled trials seems unlikely. As with many other interventions for movement disorders, identifying the specific functional impairments caused by AHS may provide the best guidance towards individualized supportive care.
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spelling pubmed-42612262014-12-11 Pathophysiology and Treatment of Alien Hand Syndrome Sarva, Harini Deik, Andres Severt, William Lawrence Tremor Other Hyperkinet Mov (N Y) Reviews BACKGROUND: Alien hand syndrome (AHS) is a disorder of involuntary, yet purposeful, hand movements that may be accompanied by agnosia, aphasia, weakness, or sensory loss. We herein review the most reported cases, current understanding of the pathophysiology, and treatments. METHODS: We performed a PubMed search in July of 2014 using the phrases “alien hand syndrome,” “alien hand syndrome pathophysiology,” “alien hand syndrome treatment,” and “anarchic hand syndrome.” The search yielded 141 papers (reviews, case reports, case series, and clinical studies), of which we reviewed 109. Non-English reports without English abstracts were excluded. RESULTS: Accumulating evidence indicates that there are three AHS variants: frontal, callosal, and posterior. Patients may demonstrate symptoms of multiple types; there is a lack of correlation between phenomenology and neuroimaging findings. Most pathologic and functional imaging studies suggest network disruption causing loss of inhibition as the likely cause. Successful interventions include botulinum toxin injections, clonazepam, visuospatial coaching techniques, distracting the affected hand, and cognitive behavioral therapy. DISCUSSION: The available literature suggests that overlap between AHS subtypes is common. The evidence for effective treatments remains anecdotal, and, given the rarity of AHS, the possibility of performing randomized, placebo-controlled trials seems unlikely. As with many other interventions for movement disorders, identifying the specific functional impairments caused by AHS may provide the best guidance towards individualized supportive care. Columbia University Libraries/Information Services 2014-12-05 /pmc/articles/PMC4261226/ /pubmed/25506043 http://dx.doi.org/10.7916/D8VX0F48 Text en http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution–Noncommerical–No Derivatives License, which permits the user to copy, distribute, and transmit the work provided that the original author and source are credited; that no commercial use is made of the work; and that the work is not altered or transformed.
spellingShingle Reviews
Sarva, Harini
Deik, Andres
Severt, William Lawrence
Pathophysiology and Treatment of Alien Hand Syndrome
title Pathophysiology and Treatment of Alien Hand Syndrome
title_full Pathophysiology and Treatment of Alien Hand Syndrome
title_fullStr Pathophysiology and Treatment of Alien Hand Syndrome
title_full_unstemmed Pathophysiology and Treatment of Alien Hand Syndrome
title_short Pathophysiology and Treatment of Alien Hand Syndrome
title_sort pathophysiology and treatment of alien hand syndrome
topic Reviews
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4261226/
https://www.ncbi.nlm.nih.gov/pubmed/25506043
http://dx.doi.org/10.7916/D8VX0F48
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