Performance of the timed “up &amp; go” test in spinal muscular atrophy

Dunaway, Sally; Montes, Jacqueline; Garber, Carol Ewing; Carr, Brendan M.; Kramer, Samantha; Kamil-Rosenberg, Shirit; Strauss, Nancy E.; Sproule, Douglas M.; Vivo, Darryl C. De

doi:10.1002/mus.24153

Cited by 24 publications

(21 citation statements)

References 46 publications

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“…35 These gains are consistent with a previous study of older individuals with SMA in which 8 of 12 individuals had similar improvements in upper limb and head movement. 34 Although limited natural history data in SMA are available for the TUG 29 , SMAFRS 26 , and the respiratory measures including MIP and MEP 36 , our data in adults treated with nusinersen diverge from the expected progressive course of the disease. 16,36 Further data collection and validation will be helpful to determine the utility of these measures in monitoring response to treatment.…”

Section: Discussionmentioning

confidence: 63%

“…Validated motor outcomes in SMA were selected for each participant based on the participant's baseline functional status: Walker (able to take 5 steps independently), sitter (able to sit independently for 3 seconds without support), and non-sitter (unable to sit independently) (Figure 1). Revised Upper Limb Module (RULM), 28 six-minute walk test (6MWT), and Timed Up and Go (TUG) 29 were performed using standardized reliable methods established by the PNCR Network. 17,30 Statistical Analysis Five participants did not have clinical evaluations coincident with nusinersen initiation; therefore, the most recent evaluations before starting nusinersen, all within 5 months of the first dose, were used as the pre-treatment value in the analyses.…”

Section: Clinical Evaluationsmentioning

confidence: 99%

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Nusinersen Treatment in Adults With Spinal Muscular Atrophy

et al. 2021

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Abstract:Purpose To determine change in motor function after treatment with nusinersen in adults with spinal muscular atrophy (SMA) during the first two years of commercial availability.Recent Findings All forty-two adult SMA patients (mean age: 34 years, range 17-66) receiving nusinersen at PNCR sites were assessed for a mean of 12.5 months (range 3-24 months). Data collected prospectively generated a mean annual rate of change over time determined using linear mixed effects models. Motor and ventilatory measures showed positive changes distinct from the progressive decline expected in untreated adults. All participants tolerated nusinersen with normal surveillance labs and no significant adverse events.Summary Trends of improvement in functional motor, patient-reported, and ventilatory measures, suggested nusinersen benefit in adults with SMA, including the chronic, weaker phenotype. Longer duration and larger studies are needed to more firmly establish nusinersen efficacy in adults with SMA.

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Section: Discussionmentioning

confidence: 63%

Section: Clinical Evaluationsmentioning

confidence: 99%

Nusinersen Treatment in Adults With Spinal Muscular Atrophy

et al. 2021

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“…The TUG test, which is a quick method to assess balance and mobility, measures the time it takes an individual to stand up from a chair, walk 3 meters, turn around, and sit down in the same chair . In SMA, it demonstrates good test–retest reliability and is associated with standard clinical assessments . TUG scores are associated with standard functional and strength assessments, decline linearly over time, and predict falls in adult motor neuron disease …”

Section: Methodsmentioning

confidence: 99%

Six-minute walk test is reliable and valid in spinal muscular atrophy

et al. 2016

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“…[20] Pulmonary function was assessed by measuring forced expiratory vital capacity (FVC) as percent predicted for age and height. [21]…”

Section: Methodsmentioning

confidence: 99%

Single-Blind, Randomized, Controlled Clinical Trial of Exercise in Ambulatory Spinal Muscular Atrophy: Why are the Results Negative?

Montes

Garber

Kramer

et al. 2015

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Background:The benefits of exercise on long-term health and well-being are well established. The possible benefits of exercise in Spinal Muscular Atrophy (SMA) have not been explored in a controlled clinical trial format.Objective:To assess the effects of exercise on measures of function, strength, and exercise capacity in ambulatory SMA patients.Methods:Fourteen participants, ages 10–48 years, were randomized to control and exercise cohorts after a 1 month lead-in period. The exercise group received 6 months of intervention. Thereafter, both groups received the intervention for the remaining 12 months. Participants were monitored for a total of 19 months. Exercise included individualized home-based cycling and strengthening. The primary outcome measure was distance walked during the six-minute walk test (6MWT). Secondary outcomes included strength, function, exercise capacity, quality of life and fatigue.Results:Twelve participants completed the first 7 months of the study, and 9 completed all 19 months. At baseline, the groups were similar on all clinical variables. There were no group changes at any time point in the 6MWT, fatigue, or function. Percent-predicted VO2 max improved 4.9% in all participants in 6 months (p = 0.036) (n = 10).Conclusion:Daily exercise is safe in ambulatory SMA and should be encouraged. We did not uncover any deleterious effects on strength, function, or fatigue. Our study documented a reduction in oxidative capacity and a blunted conditioning response to exercise possibly representing an important insight into underlying pathophysiological mechanisms. These findings also may be linked causally to mitochondrial depletion in SMA and warrant further study.

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Performance of the timed “up & go” test in spinal muscular atrophy

Abstract: In SMA, the TUG test is easily administered, reliable, and correlates with established outcome measures. TUG testing is a potentially useful outcome measure for clinical trials and a measure of disability in ambulatory patients with SMA.

Cited by 24 publications

References 46 publications

Nusinersen Treatment in Adults With Spinal Muscular Atrophy

Nusinersen Treatment in Adults With Spinal Muscular Atrophy

Six-minute walk test is reliable and valid in spinal muscular atrophy

Single-Blind, Randomized, Controlled Clinical Trial of Exercise in Ambulatory Spinal Muscular Atrophy: Why are the Results Negative?

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