| Autor: |
Tang WJ; Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA., Gu B; Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA., Montalvo S; Department of Medicine, Division of Cardiovascular Medicine, Stanford University, Palo Alto, CA 94305, USA., Dunaway Young S; Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA., Parker DM; Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA., de Monts C; Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA., Ataide P; Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA., Ni Ghiollagain N; Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA., Wheeler MT; Department of Medicine, Division of Cardiovascular Medicine, Stanford University, Palo Alto, CA 94305, USA., Tesi Rocha C; Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA., Christle JW; Department of Medicine, Division of Cardiovascular Medicine, Stanford University, Palo Alto, CA 94305, USA., He Z; Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA., Day JW; Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA., Duong T; Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA. |
| Abstrakt: |
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 ( p = 0.0086), and was significantly associated with results obtained from RULM, ATEND, EK2, and Brooke ( p < 0.0001; p = 0.029; p < 0.001; p = 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. |
