Swallowing Rehabilitation in People With Spinal Muscular Atrophy: A Scoping Review

Vollständiger Abstract

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Spinal muscular atrophy (SMA) is a neuromuscular disorder associated with motor, respiratory, and swallowing impairment. This scoping review aimed to explore how swallowing rehabilitation programmes and outcomes have been reported and measured in people with SMA. The Preferred Reporting Items for Systematic reviews and Meta-Analysis extension for Scoping Reviews guidelines and the Joanna Briggs Institute three-step search strategy were followed. Five electronic databases (Embase, CINAHL Complete, PubMed, Web of Science, and PsycInfo) and three grey literature databases (Wonder, Scopus, and MedNar) were searched without date, language, or study design restrictions. Two independent reviewers screened studies involving swallowing rehabilitation programmes in children and adults with SMA. Data extraction focused on rehabilitation characteristics, swallowing-related outcomes, outcome definitions, measurement methods, and assessment timepoints and frequency. A total of 8984 studies were identified. After duplicates were removed, 4032 studies underwent title and abstract screening, with 20 full texts assessed for eligibility. Four case reports/series involving four individuals with SMA met the inclusion criteria. Reported rehabilitation programmes included compensatory strategies, positioning, oral sensorimotor approaches, and oropharyngeal exercises. Six swallowing-related outcomes were identified, predominantly targeting physiological aspects of swallowing. Only one standardised outcome definition and two measurement tools were reported. Reporting of assessment procedures and timepoints was inconsistent. Evidence describing swallowing rehabilitation programmes in SMA is extremely limited and largely based on isolated case reports. Standardised and clinically meaningful swallowing outcome measures are lacking, limiting comparability across studies. Future research should prioritise the development of structured dysphagia rehabilitation protocols and standardised, clinically meaningful outcome measures.