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. 2023 Dec 8;12(24):7582.
doi: 10.3390/jcm12247582.

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

Whitney J Tang  1 , Bo Gu  1 , Samuel Montalvo  2 , Sally Dunaway Young  1 , Dana M Parker  1 , Constance de Monts  1 , Paxton Ataide  1 , Noirin Ni Ghiollagain  1 , Matthew T Wheeler  2 , Carolina Tesi Rocha  1 , Jeffrey W Christle  2 , Zihuai He  1 , John W Day  1 , Tina Duong  1
Affiliations

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

Whitney J Tang et al. J Clin Med. .

Abstract

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.

Keywords: exercise; fatigability; function; nusinersen; outcome measure; risdiplam; upper extremity.

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Conflict of interest statement

S.D.Y.: Advisory board: Biogen, Roche/Genentech, Scholar Rock; Consultancy/speaker: Biogen, Cure SMA, Scholar Rock; Received research support from Cure SMA. T.D.: Advisory board: CuresSMA, DuchenneUK, Actigraph, Biogen, Scholar Rock, Roche, Novartis, Dyne; Consultancy/speaker: Sarepta, Pfizer, Roche, Genentech, Trinds, NMDPharma, Novartis; Received research support from Biogen, Ionis. J.W.D.: Advisory Board: Muscular Dystrophy Association, Myotonic Dystrophy Foundation, Foundation Building Strength for Nemaline Myopathy, Cure Congenital Muscular Dystrophy, Pepgen, Epirium Bio.; Consulting: Avidity, Biogen, Novartis Gene Therapies, Roche/Genentech, Sarepta Therapeutics, Scholar Rock, Vertex; Contracted Research: A.M.O. Pharmaceuticals, Astellas, Avidity, Biogen, Ionis Pharmaceuticals, Novartis Gene Therapies, Roche Pharmaceuticals, Sanofi-Genzyme, Sarepta Therapeutics, Scholar Rock, Vertex. C.T.R.: Advisory board: Biogen, Sarepta, NS Pharma, Novartis; Consultancy/speaker: Sarepta, Roche, Biogen. All other authors have no conflicts of interest to report.

Figures

Figure 1
Figure 1
A6MCT Equipment Setup.
Figure 2
Figure 2
Total Revolutions Difference among Functional Groups.
Figure 3
Figure 3
Percent Fatigue Difference among Functional Groups.
Figure 4
Figure 4
A6MCT Association with ATEND.
Figure 5
Figure 5
A6MCT Association with RULM.
Figure 6
Figure 6
A6MCT Association with Brooke.
Figure 7
Figure 7
A6MCT Association with EK2.
See this image and copyright information in PMC

References

    1. Darras B.T., De Vivo D.C. Precious SMA natural history data. Neurology. 2018;91:337–339. doi: 10.1212/WNL.0000000000006026. - DOI - PubMed
    1. Kolb S.J., Coffey C.S., Yankey J.W., Krosschell K., Arnold W.D., Rutkove S.B., Swoboda K.J., Reyna S.P., Sakonju A., Darras B.T., et al. Natural history of infantile-onset spinal muscular atrophy. Ann. Neurol. 2017;82:883–891. doi: 10.1002/ana.25101. - DOI - PMC - PubMed
    1. Hoy S.M. Nusinersen: First Global Approval. Drugs. 2017;77:473–479. doi: 10.1007/s40265-017-0711-7. - DOI - PubMed
    1. Ratni H., Ebeling M., Baird J., Bendels S., Bylund J., Chen K.S., Denk N., Feng Z., Green L., Guerard M., et al. Discovery of Risdiplam, a Selective Survival of Motor Neuron-2 (SMN2) Gene Splicing Modifier for the Treatment of Spinal Muscular Atrophy (SMA) J. Med. Chem. 2018;61:6501–6517. doi: 10.1021/acs.jmedchem.8b00741. - DOI - PubMed
    1. Mendell J.R., Al-Zaidy S., Shell R., Arnold W.D., Rodino-Klapac L.R., Prior T.W., Lowes L., Alfano L., Berry K., Church K., et al. Single-Dose Gene-Replacement Therapy for Spinal Muscular Atrophy. N. Engl. J. Med. 2017;377:1713–1722. doi: 10.1056/NEJMoa1706198. - DOI - PubMed

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