Assessing the Assisted Six-Minute Cycling Test as a Measure of Endurance in Non-Ambulatory Patients with Spinal Muscular Atrophy (SMA)

• Published in: Journal of clinical medicine Vol. 12; no. 24; p. 7582
• Main Authors: Tang, Whitney J, Gu, Bo, Montalvo, Samuel, Dunaway Young, Sally, Parker, Dana M, de Monts, Constance, Ataide, Paxton, Ni Ghiollagain, Noirin, Wheeler, Matthew T, Tesi Rocha, Carolina, Christle, Jeffrey W, He, Zihuai, Day, John W, Duong, Tina
• Format: Journal Article
• Language: English
• Published: Switzerland MDPI AG 01.12.2023
• Subjects: Analysis; Atrophy; Care and treatment; Clinical medicine; Clinical outcomes; Cycling; Diagnosis; Disease; Exercise; Exercise tests; Fatigue; Health aspects; Longitudinal studies; Medical research; Medicine, Experimental; Muscular dystrophy; Neuromuscular diseases; Patients; Pharmaceutical industry; Population; Psychometrics; Spinal muscular atrophy; United States; Switzerland; fatigability; outcome measure; function; exercise; nusinersen; risdiplam; upper extremity
• ISSN: 2077-0383; 2077-0383
• DOI: 10.3390/jcm12247582

Assessing endurance in non-ambulatory individuals with Spinal Muscular Atrophy (SMA) has been challenging due to limited evaluation tools. The Assisted 6-Minute Cycling Test (A6MCT) is an upper limb ergometer assessment used in other neurologic disorders to measure endurance. To study the performance of the A6MCT in the non-ambulatory SMA population, prospective data was collected on 38 individuals with SMA (13 sitters; 25 non-sitters), aged 5 to 74 years (mean = 30.3; SD = 14.1). The clinical measures used were A6MCT, Revised Upper Limb Module (RULM), Adapted Test of Neuromuscular Disorders (ATEND), and Egen Klassifikation Scale 2 (EK2). Perceived fatigue was assessed using the Fatigue Severity Scale (FSS), and effort was assessed using the Rate of Perceived Exertion (RPE). Data were analyzed for: (1) Feasibility, (2) Clinical discrimination, and (3) Associations between A6MCT with clinical characteristics and outcomes. Results showed the A6MCT was feasible for 95% of the tested subjects, discriminated between functional groups ( = 0.0086), and was significantly associated with results obtained from RULM, ATEND, EK2, and Brooke ( < 0.0001; = 0.029; < 0.001; = 0.005). These findings indicate the A6MCT's potential to evaluate muscular endurance in non-ambulatory SMA individuals, complementing clinician-rated assessments. Nevertheless, further validation with a larger dataset is needed for broader application.