Assessing the Assisted Six-Minute Cycling Test as a Measure of Endurance in Non-Ambulatory Patients with Spinal Muscular Atrophy (SMA)
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 performanc...
| Main Authors: | , , , , , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2023-12-01
|
| Series: | Journal of Clinical Medicine |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2077-0383/12/24/7582 |
| _version_ | 1797380573326475264 |
|---|---|
| author | Whitney J. Tang Bo Gu Samuel Montalvo Sally Dunaway Young Dana M. Parker Constance de Monts Paxton Ataide Noirin Ni Ghiollagain Matthew T. Wheeler Carolina Tesi Rocha Jeffrey W. Christle Zihuai He John W. Day Tina Duong |
| author_facet | Whitney J. Tang Bo Gu Samuel Montalvo Sally Dunaway Young Dana M. Parker Constance de Monts Paxton Ataide Noirin Ni Ghiollagain Matthew T. Wheeler Carolina Tesi Rocha Jeffrey W. Christle Zihuai He John W. Day Tina Duong |
| author_sort | Whitney J. Tang |
| collection | DOAJ |
| description | 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 (<i>p</i> = 0.0086), and was significantly associated with results obtained from RULM, ATEND, EK2, and Brooke (<i>p</i> < 0.0001; <i>p</i> = 0.029; <i>p</i> < 0.001; <i>p</i> = 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. |
| first_indexed | 2024-03-08T20:39:16Z |
| format | Article |
| id | doaj.art-d7a56aefb4154cbcbb6ed794fe26bf48 |
| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-08T20:39:16Z |
| publishDate | 2023-12-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-d7a56aefb4154cbcbb6ed794fe26bf482023-12-22T14:17:04ZengMDPI AGJournal of Clinical Medicine2077-03832023-12-011224758210.3390/jcm12247582Assessing the Assisted Six-Minute Cycling Test as a Measure of Endurance in Non-Ambulatory Patients with Spinal Muscular Atrophy (SMA)Whitney J. Tang0Bo Gu1Samuel Montalvo2Sally Dunaway Young3Dana M. Parker4Constance de Monts5Paxton Ataide6Noirin Ni Ghiollagain7Matthew T. Wheeler8Carolina Tesi Rocha9Jeffrey W. Christle10Zihuai He11John W. Day12Tina Duong13Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USADepartment of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USADepartment of Medicine, Division of Cardiovascular Medicine, Stanford University, Palo Alto, CA 94305, USADepartment of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USADepartment of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USADepartment of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USADepartment of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USADepartment of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USADepartment of Medicine, Division of Cardiovascular Medicine, Stanford University, Palo Alto, CA 94305, USADepartment of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USADepartment of Medicine, Division of Cardiovascular Medicine, Stanford University, Palo Alto, CA 94305, USADepartment of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USADepartment of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USADepartment of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USAAssessing 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 (<i>p</i> = 0.0086), and was significantly associated with results obtained from RULM, ATEND, EK2, and Brooke (<i>p</i> < 0.0001; <i>p</i> = 0.029; <i>p</i> < 0.001; <i>p</i> = 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.https://www.mdpi.com/2077-0383/12/24/7582exerciseoutcome measurefunctionupper extremityfatigabilitynusinersen |
| spellingShingle | Whitney J. Tang Bo Gu Samuel Montalvo Sally Dunaway Young Dana M. Parker Constance de Monts Paxton Ataide Noirin Ni Ghiollagain Matthew T. Wheeler Carolina Tesi Rocha Jeffrey W. Christle Zihuai He John W. Day Tina Duong Assessing the Assisted Six-Minute Cycling Test as a Measure of Endurance in Non-Ambulatory Patients with Spinal Muscular Atrophy (SMA) Journal of Clinical Medicine exercise outcome measure function upper extremity fatigability nusinersen |
| title | Assessing the Assisted Six-Minute Cycling Test as a Measure of Endurance in Non-Ambulatory Patients with Spinal Muscular Atrophy (SMA) |
| title_full | Assessing the Assisted Six-Minute Cycling Test as a Measure of Endurance in Non-Ambulatory Patients with Spinal Muscular Atrophy (SMA) |
| title_fullStr | Assessing the Assisted Six-Minute Cycling Test as a Measure of Endurance in Non-Ambulatory Patients with Spinal Muscular Atrophy (SMA) |
| title_full_unstemmed | Assessing the Assisted Six-Minute Cycling Test as a Measure of Endurance in Non-Ambulatory Patients with Spinal Muscular Atrophy (SMA) |
| title_short | Assessing the Assisted Six-Minute Cycling Test as a Measure of Endurance in Non-Ambulatory Patients with Spinal Muscular Atrophy (SMA) |
| title_sort | assessing the assisted six minute cycling test as a measure of endurance in non ambulatory patients with spinal muscular atrophy sma |
| topic | exercise outcome measure function upper extremity fatigability nusinersen |
| url | https://www.mdpi.com/2077-0383/12/24/7582 |
| work_keys_str_mv | AT whitneyjtang assessingtheassistedsixminutecyclingtestasameasureofenduranceinnonambulatorypatientswithspinalmuscularatrophysma AT bogu assessingtheassistedsixminutecyclingtestasameasureofenduranceinnonambulatorypatientswithspinalmuscularatrophysma AT samuelmontalvo assessingtheassistedsixminutecyclingtestasameasureofenduranceinnonambulatorypatientswithspinalmuscularatrophysma AT sallydunawayyoung assessingtheassistedsixminutecyclingtestasameasureofenduranceinnonambulatorypatientswithspinalmuscularatrophysma AT danamparker assessingtheassistedsixminutecyclingtestasameasureofenduranceinnonambulatorypatientswithspinalmuscularatrophysma AT constancedemonts assessingtheassistedsixminutecyclingtestasameasureofenduranceinnonambulatorypatientswithspinalmuscularatrophysma AT paxtonataide assessingtheassistedsixminutecyclingtestasameasureofenduranceinnonambulatorypatientswithspinalmuscularatrophysma AT noirinnighiollagain assessingtheassistedsixminutecyclingtestasameasureofenduranceinnonambulatorypatientswithspinalmuscularatrophysma AT matthewtwheeler assessingtheassistedsixminutecyclingtestasameasureofenduranceinnonambulatorypatientswithspinalmuscularatrophysma AT carolinatesirocha assessingtheassistedsixminutecyclingtestasameasureofenduranceinnonambulatorypatientswithspinalmuscularatrophysma AT jeffreywchristle assessingtheassistedsixminutecyclingtestasameasureofenduranceinnonambulatorypatientswithspinalmuscularatrophysma AT zihuaihe assessingtheassistedsixminutecyclingtestasameasureofenduranceinnonambulatorypatientswithspinalmuscularatrophysma AT johnwday assessingtheassistedsixminutecyclingtestasameasureofenduranceinnonambulatorypatientswithspinalmuscularatrophysma AT tinaduong assessingtheassistedsixminutecyclingtestasameasureofenduranceinnonambulatorypatientswithspinalmuscularatrophysma |