Invited Review: Limb-girdle muscular dystrophies: A scoping review and overview of currently available rehabilitation strategies
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Abstract
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.
Objectives:(1) Appreciate the heterogeneity of the limb-girdle muscular dystrophies and be able design individualized rehabilitative approaches to patients; (2) Understand and be able to implement appropriate aerobic and strength training as part of a comprehensive rehabilitative strategy for patients with limb-girdle muscle dystrophies; (3) Understand the symptoms and signs of respiratory involvement in the limb-girdle muscular dystrophies and implement appropriate respiratory interventions as part of comprehensive rehabilitative strategies.
ACCREDITATION STATEMENT The AANEM is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians.
CREDIT DESIGNATION The AANEM designates this enduring material for a maximum of 1
AMA PRA Category 1 Credits TM. Physicians should claim only the credit commensurate with the extent of their participation in the activity.
DISCLOSURE INFORMATIONThe authors have no conflicts to disclose.
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Giorgia D'Este PhD; Mattia Spagna MSc; Sara Federico MSc; Luisa Cacciante MSc; Błażej Cieslik PhD; Pawel Kiper PhD; Rita Barresi PhD