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Split phenomena in amyotrophic lateral sclerosis: Current evidences, pathogenetic hypotheses and diagnostic implications

Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease and has emerged among the disorders with the largest increasing incidence in Western countries. Although the diagnosis is based on clinical grounds, electromyography (EMG), and nerve conduction studies (NCS) play a crucial r...

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Autores principales: Zoccolella, Stefano, Giugno, Alessia, Logroscino, Giancarlo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9868395/
https://www.ncbi.nlm.nih.gov/pubmed/36699516
http://dx.doi.org/10.3389/fnins.2022.1100040
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author Zoccolella, Stefano
Giugno, Alessia
Logroscino, Giancarlo
author_facet Zoccolella, Stefano
Giugno, Alessia
Logroscino, Giancarlo
author_sort Zoccolella, Stefano
collection PubMed
description Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease and has emerged among the disorders with the largest increasing incidence in Western countries. Although the diagnosis is based on clinical grounds, electromyography (EMG), and nerve conduction studies (NCS) play a crucial role to exclude other potential etiologies of lower motor neuron (LMN) dysfunction. Based on clinical grounds, a peculiar pattern of dissociated atrophy of the intrinsic hand and foot muscles, termed the “split-hand” (SH) and “split-leg” (SL) signs, has been described in a significant proportion of subjects with ALS, even at the early stages of the disease, when symptoms are focal. These signs are rare in neurological and non-neurological diseases other than ALS. In this review, we discussed current evidences concerning SH and SL signs, their pathogenetic hypotheses and neurophysiological findings. We also analyze whether SH and SL signs can be reliable markers in the differential diagnosis and in the prognosis of ALS.
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spelling pubmed-98683952023-01-24 Split phenomena in amyotrophic lateral sclerosis: Current evidences, pathogenetic hypotheses and diagnostic implications Zoccolella, Stefano Giugno, Alessia Logroscino, Giancarlo Front Neurosci Neuroscience Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease and has emerged among the disorders with the largest increasing incidence in Western countries. Although the diagnosis is based on clinical grounds, electromyography (EMG), and nerve conduction studies (NCS) play a crucial role to exclude other potential etiologies of lower motor neuron (LMN) dysfunction. Based on clinical grounds, a peculiar pattern of dissociated atrophy of the intrinsic hand and foot muscles, termed the “split-hand” (SH) and “split-leg” (SL) signs, has been described in a significant proportion of subjects with ALS, even at the early stages of the disease, when symptoms are focal. These signs are rare in neurological and non-neurological diseases other than ALS. In this review, we discussed current evidences concerning SH and SL signs, their pathogenetic hypotheses and neurophysiological findings. We also analyze whether SH and SL signs can be reliable markers in the differential diagnosis and in the prognosis of ALS. Frontiers Media S.A. 2023-01-09 /pmc/articles/PMC9868395/ /pubmed/36699516 http://dx.doi.org/10.3389/fnins.2022.1100040 Text en Copyright © 2023 Zoccolella, Giugno and Logroscino. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Neuroscience
Zoccolella, Stefano
Giugno, Alessia
Logroscino, Giancarlo
Split phenomena in amyotrophic lateral sclerosis: Current evidences, pathogenetic hypotheses and diagnostic implications
title Split phenomena in amyotrophic lateral sclerosis: Current evidences, pathogenetic hypotheses and diagnostic implications
title_full Split phenomena in amyotrophic lateral sclerosis: Current evidences, pathogenetic hypotheses and diagnostic implications
title_fullStr Split phenomena in amyotrophic lateral sclerosis: Current evidences, pathogenetic hypotheses and diagnostic implications
title_full_unstemmed Split phenomena in amyotrophic lateral sclerosis: Current evidences, pathogenetic hypotheses and diagnostic implications
title_short Split phenomena in amyotrophic lateral sclerosis: Current evidences, pathogenetic hypotheses and diagnostic implications
title_sort split phenomena in amyotrophic lateral sclerosis: current evidences, pathogenetic hypotheses and diagnostic implications
topic Neuroscience
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9868395/
https://www.ncbi.nlm.nih.gov/pubmed/36699516
http://dx.doi.org/10.3389/fnins.2022.1100040
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