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      An 8-month adapted motor activity program in a young CMT1A male patient

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          Abstract

          Background:

          It is unclear whether prolonged periods of training can be well tolerated. In Charcot-Marie Tooth disease (CMT). We report the effects of an 8-month, adapted motor activity (AMA) program in a 16-years-old CMT1A male patient. The program included strength, mobility, and balance training (two sessions per week, 1 h per session).

          Measures:

          Walking ability and walking velocity (Six-Minute Walking Test—6MWT, Ten Meters Walking Test—10 mW T), balance (Y-Balance Test—YBT, Berg Balance Scale—BBS), functional mobility (Short Physical Performance Battery—Short physical performance battery), fatigue (Checklist Individual strength questionnaire - CIS20R), health and quality of life (Short Form Health Survey 36 questionnaire—SF-36) were evaluated in three moments: before (T0), after 5 (T1) and 8 (T2) months of adapted motor activity. Dorsal and plantar foot flexion strength (Maximal Voluntary Contraction—maximum voluntary contraction) and neuromuscular functions (Electromyography—sEMG, interpolated twitch technique—ITT) were measured at T1 and T2.

          Results:

          Relative to T0, an amelioration of walking ability (6MWT, +9,3%) and balance (with improvements on Y-balance composite normalized mean reach of the right and left limb of 15,3% and 8,5%, respectively) was appreciable. Relative to T1, an increase in foot strength in three out of four movements (right plantar flexion, +39,3%, left plantar flexion, +22,7%, left dorsal flexion, 11,5%) was observed. Concerning voluntary muscle activation, a greater recruitment in the left, unlike right, medial gastrocnemius was observed.

          Conclusion:

          Results suggest the safety of an 8-month AMA program in a young patient affected by CMT1A.

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          Most cited references37

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          ATS statement: guidelines for the six-minute walk test.

          (2002)
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            A short physical performance battery assessing lower extremity function: association with self-reported disability and prediction of mortality and nursing home admission.

            A short battery of physical performance tests was used to assess lower extremity function in more than 5,000 persons age 71 years and older in three communities. Balance, gait, strength, and endurance were evaluated by examining ability to stand with the feet together in the side-by-side, semi-tandem, and tandem positions, time to walk 8 feet, and time to rise from a chair and return to the seated position 5 times. A wide distribution of performance was observed for each test. Each test and a summary performance scale, created by summing categorical rankings of performance on each test, were strongly associated with self-report of disability. Both self-report items and performance tests were independent predictors of short-term mortality and nursing home admission in multivariate analyses. However, evidence is presented that the performance tests provide information not available from self-report items. Of particular importance is the finding that in those at the high end of the functional spectrum, who reported almost no disability, the performance test scores distinguished a gradient of risk for mortality and nursing home admission. Additionally, within subgroups with identical self-report profiles, there were systematic differences in physical performance related to age and sex. This study provides evidence that performance measures can validly characterize older persons across a broad spectrum of lower extremity function. Performance and self-report measures may complement each other in providing useful information about functional status.
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              Development of recommendations for SEMG sensors and sensor placement procedures.

              The knowledge of surface electromyography (SEMG) and the number of applications have increased considerably during the past ten years. However, most methodological developments have taken place locally, resulting in different methodologies among the different groups of users.A specific objective of the European concerted action SENIAM (surface EMG for a non-invasive assessment of muscles) was, besides creating more collaboration among the various European groups, to develop recommendations on sensors, sensor placement, signal processing and modeling. This paper will present the process and the results of the development of the recommendations for the SEMG sensors and sensor placement procedures. Execution of the SENIAM sensor tasks, in the period 1996-1999, has been handled in a number of partly parallel and partly sequential activities. A literature scan was carried out on the use of sensors and sensor placement procedures in European laboratories. In total, 144 peer-reviewed papers were scanned on the applied SEMG sensor properties and sensor placement procedures. This showed a large variability of methodology as well as a rather insufficient description. A special workshop provided an overview on the scientific and clinical knowledge of the effects of sensor properties and sensor placement procedures on the SEMG characteristics. Based on the inventory, the results of the topical workshop and generally accepted state-of-the-art knowledge, a first proposal for sensors and sensor placement procedures was defined. Besides containing a general procedure and recommendations for sensor placement, this was worked out in detail for 27 different muscles. This proposal was evaluated in several European laboratories with respect to technical and practical aspects and also sent to all members of the SENIAM club (>100 members) together with a questionnaire to obtain their comments. Based on this evaluation the final recommendations of SENIAM were made and published (SENIAM 8: European recommendations for surface electromyography, 1999), both as a booklet and as a CD-ROM. In this way a common body of knowledge has been created on SEMG sensors and sensor placement properties as well as practical guidelines for the proper use of SEMG.
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                Author and article information

                Contributors
                Role: Role: Role: Role:
                URI : https://loop.frontiersin.org/people/1989009/overviewRole: Role: Role:
                Role: Role: Role:
                URI : https://loop.frontiersin.org/people/2543505/overviewRole: Role:
                Role: Role:
                URI : https://loop.frontiersin.org/people/1408948/overviewRole: Role: Role:
                Role: Role: Role:
                URI : https://loop.frontiersin.org/people/402620/overviewRole: Role: Role: Role: Role: Role:
                Journal
                Front Physiol
                Front Physiol
                Front. Physiol.
                Frontiers in Physiology
                Frontiers Media S.A.
                1664-042X
                05 April 2024
                2024
                : 15
                : 1347319
                Affiliations
                [1] 1 CRIAMS Sport Medicine Centre Voghera , University of Pavia , Voghera, Italy
                [2] 2 Neurology Operative Unit , Civilian Hospital of Voghera , Voghera, Italy
                [3] 3 Department of Internal Medicine , University of Pavia , Pavia, Italy
                [4] 4 Department of Public Health Experimental and Forensic Medicine , University of Pavia , Voghera, Italy
                Author notes

                Edited by: Qichang Mei, Ningbo University, China

                Reviewed by: Alessandro Marco De Nunzio, Lunex University, Luxembourg

                Francesca Lanfranconi, Centro Maria Letizia Verga—Fondazione Monza e Brianza per il Bambino e la sua Mamma, Italy

                *Correspondence: Giuseppe D’Antona, gdantona@ 123456unipv.it
                Article
                1347319
                10.3389/fphys.2024.1347319
                11026674
                38645694
                1a56385d-c55a-4599-8957-54a59fbe6b6e
                Copyright © 2024 Bottoni, Crisafulli, Pisegna, Serra, Brambilla, Feletti, Cremonte and D’Antona.

                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.

                History
                : 30 November 2023
                : 12 March 2024
                Funding
                The author(s) declare that no financial support was received for the research, authorship, and/or publication of this article.
                Categories
                Physiology
                Brief Research Report
                Custom metadata
                Exercise Physiology

                Anatomy & Physiology
                charcot-marie-tooth,neuropathy,exercise,balance,gait
                Anatomy & Physiology
                charcot-marie-tooth, neuropathy, exercise, balance, gait

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