{"title":"肢腰肌营养不良症:对目前可用的康复策略进行范围界定和概述。","authors":"Giorgia D'Este, Mattia Spagna, Sara Federico, Luisa Cacciante, Błażej Cieślik, Pawel Kiper, Rita Barresi","doi":"10.1002/mus.28284","DOIUrl":null,"url":null,"abstract":"<p><p>Limb-girdle muscular dystrophies (LGMDs) constitute a diverse group of inherited disorders primarily affecting skeletal muscle. Despite the absence of cures, rehabilitative treatments offer potential for preventing and mitigating loss of muscle strength. However, the role of exercise training in LGMD patients remains contentious. This review aims to provide an overview of the currently available motor rehabilitation strategies for the most common subtypes of LGMD. To identify relevant articles, we performed a systematic search in PubMed, Embase, Cochrane Library, and Web of Science, focusing on muscular and respiratory interventions. The search resulted in 560 potentially relevant articles, of which 16 were included in the review. Eight studies concentrated on neuromuscular functional rehabilitation therapy programs, seven combined both neuromuscular rehabilitation and interventions to maintain or enhance respiratory functionality and one focused on respiratory intervention only. Altogether, the papers examined offered a comprehensive view on the rehabilitative strategies available and provided an indication of the most valuable practices to deal with patients' health and needs. Upon analysis, we conclude that, when tailored to individual needs, muscle training can enhance strength and functional abilities, positively impacting psychological well-being. However, generic protocols may lead to limited benefits, fatigue, pain, and compliance issues. Moreover, early management of respiratory symptoms and personalized respiratory physiotherapy can enhance patients' well-being and their capability to participate in muscle training exercises. Future studies should not only refine rehabilitation approaches but also assess their impact on patients' quality of life, including psychological factors like depression and self-esteem.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"138-146"},"PeriodicalIF":2.8000,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11708444/pdf/","citationCount":"0","resultStr":"{\"title\":\"Limb-girdle muscular dystrophies: A scoping review and overview of currently available rehabilitation strategies.\",\"authors\":\"Giorgia D'Este, Mattia Spagna, Sara Federico, Luisa Cacciante, Błażej Cieślik, Pawel Kiper, Rita Barresi\",\"doi\":\"10.1002/mus.28284\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Limb-girdle muscular dystrophies (LGMDs) constitute a diverse group of inherited disorders primarily affecting skeletal muscle. 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Altogether, the papers examined offered a comprehensive view on the rehabilitative strategies available and provided an indication of the most valuable practices to deal with patients' health and needs. Upon analysis, we conclude that, when tailored to individual needs, muscle training can enhance strength and functional abilities, positively impacting psychological well-being. However, generic protocols may lead to limited benefits, fatigue, pain, and compliance issues. Moreover, early management of respiratory symptoms and personalized respiratory physiotherapy can enhance patients' well-being and their capability to participate in muscle training exercises. 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引用次数: 0
摘要
肢腰肌营养不良症(LGMDs)是一组主要影响骨骼肌的遗传性疾病。尽管无法治愈,但康复治疗为预防和减轻肌肉力量的丧失提供了可能。然而,运动训练在 LGMD 患者中的作用仍存在争议。本综述旨在概述目前针对最常见亚型 LGMD 的运动康复策略。为了确定相关文章,我们在 PubMed、Embase、Cochrane Library 和 Web of Science 中进行了系统检索,重点关注肌肉和呼吸干预。搜索结果显示有 560 篇潜在相关文章,其中 16 篇被纳入综述。八项研究集中于神经肌肉功能康复治疗计划,七项研究结合了神经肌肉康复和干预措施,以维持或增强呼吸功能,一项研究仅关注呼吸干预。总之,所研究的论文对现有的康复策略提供了一个全面的视角,并提供了应对患者健康和需求的最有价值的做法。经过分析,我们得出结论:根据个人需求进行肌肉训练可以增强力量和功能,对心理健康产生积极影响。然而,一般的方案可能会导致有限的益处、疲劳、疼痛和依从性问题。此外,早期治疗呼吸系统症状和个性化呼吸理疗可提高患者的幸福感和参与肌肉训练的能力。未来的研究不仅应完善康复方法,还应评估其对患者生活质量的影响,包括抑郁和自尊等心理因素。
Limb-girdle muscular dystrophies: A scoping review and overview of currently available rehabilitation strategies.
Limb-girdle muscular dystrophies (LGMDs) constitute a diverse group of inherited disorders primarily affecting skeletal muscle. Despite the absence of cures, rehabilitative treatments offer potential for preventing and mitigating loss of muscle strength. However, the role of exercise training in LGMD patients remains contentious. This review aims to provide an overview of the currently available motor rehabilitation strategies for the most common subtypes of LGMD. To identify relevant articles, we performed a systematic search in PubMed, Embase, Cochrane Library, and Web of Science, focusing on muscular and respiratory interventions. The search resulted in 560 potentially relevant articles, of which 16 were included in the review. Eight studies concentrated on neuromuscular functional rehabilitation therapy programs, seven combined both neuromuscular rehabilitation and interventions to maintain or enhance respiratory functionality and one focused on respiratory intervention only. Altogether, the papers examined offered a comprehensive view on the rehabilitative strategies available and provided an indication of the most valuable practices to deal with patients' health and needs. Upon analysis, we conclude that, when tailored to individual needs, muscle training can enhance strength and functional abilities, positively impacting psychological well-being. However, generic protocols may lead to limited benefits, fatigue, pain, and compliance issues. Moreover, early management of respiratory symptoms and personalized respiratory physiotherapy can enhance patients' well-being and their capability to participate in muscle training exercises. Future studies should not only refine rehabilitation approaches but also assess their impact on patients' quality of life, including psychological factors like depression and self-esteem.
期刊介绍:
Muscle & Nerve is an international and interdisciplinary publication of original contributions, in both health and disease, concerning studies of the muscle, the neuromuscular junction, the peripheral motor, sensory and autonomic neurons, and the central nervous system where the behavior of the peripheral nervous system is clarified. Appearing monthly, Muscle & Nerve publishes clinical studies and clinically relevant research reports in the fields of anatomy, biochemistry, cell biology, electrophysiology and electrodiagnosis, epidemiology, genetics, immunology, pathology, pharmacology, physiology, toxicology, and virology. The Journal welcomes articles and reports on basic clinical electrophysiology and electrodiagnosis. We expedite some papers dealing with timely topics to keep up with the fast-moving pace of science, based on the referees'' recommendation.