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Control of arm movements in Friedreich’s ataxia patients: role of sensory feedback
Friedreich’s ataxia (FA) is a hereditary system degeneration, which progressively affects sensory functions such as proprioceptive feedback, which causes progressive ataxia in FA patients. While major clinical features of movement disorders in FA patients have been identified, the underlying impaire...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer Berlin Heidelberg
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9038882/ https://www.ncbi.nlm.nih.gov/pubmed/35286422 http://dx.doi.org/10.1007/s00221-022-06343-5 |
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author | Zhang, Lei Straube, Andreas Eggert, Thomas |
author_facet | Zhang, Lei Straube, Andreas Eggert, Thomas |
author_sort | Zhang, Lei |
collection | PubMed |
description | Friedreich’s ataxia (FA) is a hereditary system degeneration, which progressively affects sensory functions such as proprioceptive feedback, which causes progressive ataxia in FA patients. While major clinical features of movement disorders in FA patients have been identified, the underlying impaired neural control is not sufficiently understood. To elucidate the underlying control mechanism, we investigated single-joint movements of the upper limb in FA patients. Small, tolerable force perturbations were induced during voluntary single-joint arm movements to examine the compensatory reaction of the FA patient’s motor system. Movement kinematics were measured, and muscle torques were quantified. We first found that as in healthy subjects, unperturbed single-joint movements in FA patients preserved similar temporal profiles of hand velocity and muscle torques, however, scaled in duration and amplitude. In addition, the small perturbations were compensated for efficiently in both groups, with the endpoint error < 0.5° (maximum displacement of 5–15°). We further quantified the differences in movement time, torque response, and displacement between patients and controls. To distinguish whether these differences were caused by a malfunction of top-down control or a malfunction of feedback control, the responses were fitted with a detailed model of the stretch reflex. The model simulations revealed that the feedback delay, but not the feedback gain was affected in FA patients. They also showed that the descending control signal was scaled in time and amplitude and co-contraction was smaller in FA patients. Thus, our study explains how the motor deficits of FA patients result from pathological alterations of both top-down and feedback control. |
format | Online Article Text |
id | pubmed-9038882 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Springer Berlin Heidelberg |
record_format | MEDLINE/PubMed |
spelling | pubmed-90388822022-05-07 Control of arm movements in Friedreich’s ataxia patients: role of sensory feedback Zhang, Lei Straube, Andreas Eggert, Thomas Exp Brain Res Research Article Friedreich’s ataxia (FA) is a hereditary system degeneration, which progressively affects sensory functions such as proprioceptive feedback, which causes progressive ataxia in FA patients. While major clinical features of movement disorders in FA patients have been identified, the underlying impaired neural control is not sufficiently understood. To elucidate the underlying control mechanism, we investigated single-joint movements of the upper limb in FA patients. Small, tolerable force perturbations were induced during voluntary single-joint arm movements to examine the compensatory reaction of the FA patient’s motor system. Movement kinematics were measured, and muscle torques were quantified. We first found that as in healthy subjects, unperturbed single-joint movements in FA patients preserved similar temporal profiles of hand velocity and muscle torques, however, scaled in duration and amplitude. In addition, the small perturbations were compensated for efficiently in both groups, with the endpoint error < 0.5° (maximum displacement of 5–15°). We further quantified the differences in movement time, torque response, and displacement between patients and controls. To distinguish whether these differences were caused by a malfunction of top-down control or a malfunction of feedback control, the responses were fitted with a detailed model of the stretch reflex. The model simulations revealed that the feedback delay, but not the feedback gain was affected in FA patients. They also showed that the descending control signal was scaled in time and amplitude and co-contraction was smaller in FA patients. Thus, our study explains how the motor deficits of FA patients result from pathological alterations of both top-down and feedback control. Springer Berlin Heidelberg 2022-03-14 2022 /pmc/articles/PMC9038882/ /pubmed/35286422 http://dx.doi.org/10.1007/s00221-022-06343-5 Text en © The Author(s) 2022 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 | Research Article Zhang, Lei Straube, Andreas Eggert, Thomas Control of arm movements in Friedreich’s ataxia patients: role of sensory feedback |
title | Control of arm movements in Friedreich’s ataxia patients: role of sensory feedback |
title_full | Control of arm movements in Friedreich’s ataxia patients: role of sensory feedback |
title_fullStr | Control of arm movements in Friedreich’s ataxia patients: role of sensory feedback |
title_full_unstemmed | Control of arm movements in Friedreich’s ataxia patients: role of sensory feedback |
title_short | Control of arm movements in Friedreich’s ataxia patients: role of sensory feedback |
title_sort | control of arm movements in friedreich’s ataxia patients: role of sensory feedback |
topic | Research Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9038882/ https://www.ncbi.nlm.nih.gov/pubmed/35286422 http://dx.doi.org/10.1007/s00221-022-06343-5 |
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