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Charcot-Marie-Tooth neuropathy score and ambulation index are both predictors of orthotic need for patients with CMT

Charcot-Marie-Tooth (CMT) disease is the most common hereditary neuropathy with an estimated prevalence of 1 person affected on 2500. Frequent symptoms include distal weakness and muscle wasting, sensory loss, reduced deep tendon reflexes, and skeletal deformities, such as hammer toes and pes cavus....

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Autores principales: Prada, Valeria, Zuccarino, Riccardo, Schenone, Cristina, Mennella, Giulia, Grandis, Marina, Shy, Michael E., Schenone, Angelo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer International Publishing 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8918134/
https://www.ncbi.nlm.nih.gov/pubmed/34613504
http://dx.doi.org/10.1007/s10072-021-05646-9
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author Prada, Valeria
Zuccarino, Riccardo
Schenone, Cristina
Mennella, Giulia
Grandis, Marina
Shy, Michael E.
Schenone, Angelo
author_facet Prada, Valeria
Zuccarino, Riccardo
Schenone, Cristina
Mennella, Giulia
Grandis, Marina
Shy, Michael E.
Schenone, Angelo
author_sort Prada, Valeria
collection PubMed
description Charcot-Marie-Tooth (CMT) disease is the most common hereditary neuropathy with an estimated prevalence of 1 person affected on 2500. Frequent symptoms include distal weakness and muscle wasting, sensory loss, reduced deep tendon reflexes, and skeletal deformities, such as hammer toes and pes cavus. CMT is a progressive disease and patients’ needs change over their lifetime. In particular, ambulation aids are increasingly needed to maintain ambulation and reduce the risk of falls. We performed a retrospective analysis of medical records from 149 patients with confirmed CMT to evaluate patients ambulation needs related to the severity of their CMT as measured by the CMT Neuropathy Score (CMTNS) and Ambulation Index (AI). Most patients required some form of orthotics (86.6%). The CMTNS and AI scores both differed significantly between patients with no orthotics compared to those who wore insoles/inserts. The CMTNS and AI also differed significantly between patients wearing insoles and those with ankle foot orthotics (AFOs). CMTNS and the AI were valid predictors of the type and choice of the orthotics. Both the CMTNS and AI can be effective tools to aid in the correct choice of orthotics in patients affected by CMT. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s10072-021-05646-9.
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spelling pubmed-89181342022-03-17 Charcot-Marie-Tooth neuropathy score and ambulation index are both predictors of orthotic need for patients with CMT Prada, Valeria Zuccarino, Riccardo Schenone, Cristina Mennella, Giulia Grandis, Marina Shy, Michael E. Schenone, Angelo Neurol Sci Original Article Charcot-Marie-Tooth (CMT) disease is the most common hereditary neuropathy with an estimated prevalence of 1 person affected on 2500. Frequent symptoms include distal weakness and muscle wasting, sensory loss, reduced deep tendon reflexes, and skeletal deformities, such as hammer toes and pes cavus. CMT is a progressive disease and patients’ needs change over their lifetime. In particular, ambulation aids are increasingly needed to maintain ambulation and reduce the risk of falls. We performed a retrospective analysis of medical records from 149 patients with confirmed CMT to evaluate patients ambulation needs related to the severity of their CMT as measured by the CMT Neuropathy Score (CMTNS) and Ambulation Index (AI). Most patients required some form of orthotics (86.6%). The CMTNS and AI scores both differed significantly between patients with no orthotics compared to those who wore insoles/inserts. The CMTNS and AI also differed significantly between patients wearing insoles and those with ankle foot orthotics (AFOs). CMTNS and the AI were valid predictors of the type and choice of the orthotics. Both the CMTNS and AI can be effective tools to aid in the correct choice of orthotics in patients affected by CMT. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s10072-021-05646-9. Springer International Publishing 2021-10-06 2022 /pmc/articles/PMC8918134/ /pubmed/34613504 http://dx.doi.org/10.1007/s10072-021-05646-9 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Original Article
Prada, Valeria
Zuccarino, Riccardo
Schenone, Cristina
Mennella, Giulia
Grandis, Marina
Shy, Michael E.
Schenone, Angelo
Charcot-Marie-Tooth neuropathy score and ambulation index are both predictors of orthotic need for patients with CMT
title Charcot-Marie-Tooth neuropathy score and ambulation index are both predictors of orthotic need for patients with CMT
title_full Charcot-Marie-Tooth neuropathy score and ambulation index are both predictors of orthotic need for patients with CMT
title_fullStr Charcot-Marie-Tooth neuropathy score and ambulation index are both predictors of orthotic need for patients with CMT
title_full_unstemmed Charcot-Marie-Tooth neuropathy score and ambulation index are both predictors of orthotic need for patients with CMT
title_short Charcot-Marie-Tooth neuropathy score and ambulation index are both predictors of orthotic need for patients with CMT
title_sort charcot-marie-tooth neuropathy score and ambulation index are both predictors of orthotic need for patients with cmt
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8918134/
https://www.ncbi.nlm.nih.gov/pubmed/34613504
http://dx.doi.org/10.1007/s10072-021-05646-9
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