Leg muscle function and fatigue during walking in spinal muscular atrophy type 3

Montes, Jacqueline; Dunaway, Sally; Garber, Carol Ewing; Chiriboga, Claudia A.; Vivo, Darryl C. De; Rao, Ashwini K.

doi:10.1002/mus.24081

Cited by 37 publications

(31 citation statements)

References 23 publications

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“…Consistent with previous reports, we found decreasing stride length to contribute most to fatigue‐related changes in speed. Moreover, we found that SMA patients walk predominantly on their forefoot with a reduced initial heel strike, and the foot‐flat phase occurs early in the gait cycle.…”

Section: Discussionsupporting

confidence: 92%

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Gait assessment with solesound instrumented footwear in spinal muscular atrophy

et al. 2017

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Section: Discussionsupporting

confidence: 92%

“…The most common cause of the SMA phenotype results from a homozygous deletion of the survival motor neuron 1 ( SMN1 ) gene located on chromosome 5q13 . Individuals with the mildest phenotype, SMA type 3, are able to walk independently, but their proximal weakness results in gait and gross motor function impairments …”

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confidence: 99%

Gait assessment with solesound instrumented footwear in spinal muscular atrophy

et al. 2017

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“…Tools assessing walking ability and fatigue are clinically relevant in individuals with milder forms of SMA . The 6‐minute walk test (6MWT) is a valid and clinically meaningful measure of ambulatory function and has demonstrated sensitivity to fatigue‐related changes in individuals with SMA . Fatigue measured during the 6MWT may represent underlying disease‐specific mechanisms, and improvements may represent treatment benefit.…”

Section: Introductionmentioning

confidence: 99%

Nusinersen improves walking distance and reduces fatigue in later‐onset spinal muscular atrophy

et al. 2019

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Introduction Ambulatory individuals with spinal muscular atrophy (SMA) experience muscle weakness, gait impairments, and fatigue that affect their walking ability. Improvements have been observed in motor function in children treated with nusinersen, but its impact on fatigue has not been studied. Methods Post hoc analyses were used to examine changes in 6‐minute walk test (6MWT) distance and fatigue in children and adolescents with SMA type II and III who received their first dose of nusinersen in the phase Ib/IIa, open‐label CS2 study and were ambulatory during CS2 or the extension study, CS12. Results Fourteen children performed the 6MWT. Median (25th, 75th percentile) distance walked increased over time by 98.0 (62.0, 135.0) meters at day 1050, whereas median fatigue changed by −3.8% (−19.7%, 1.4%). Discussion These results support previous studies demonstrating clinically meaningful effects of nusinersen on motor function in children and adolescents with later‐onset SMA.

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“…A number of studies have reported an association between strength and motor function in SMA . At least 70% of patients with SMA type II and 40% of patients with type III require assistance with self‐care, and 90% with type II and 60% with type III require assistance with mobility .…”

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Resistance strength training exercise in children with spinal muscular atrophy

et al. 2015

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Introduction Preliminary evidence in adults with spinal muscular atrophy (SMA) and in SMA animal models suggests exercise has potential benefits in improving or stabilizing muscle strength and motor function. Methods We evaluated feasibility, safety, and effects on strength and motor function of a home-based, supervised progressive resistance strength training exercise program in children with SMA types II and III. Up to 14 bilateral proximal muscles were exercised 3 times weekly for 12 weeks. Results Nine children with SMA, aged 10.4±3.8 years, completed the resistance training exercise program. Ninety percent of visits occurred per protocol. Training sessions were pain-free (99.8%), and no study-related adverse events occurred. Trends in improved strength and motor function were observed. Conclusions A 12-week supervised, home-based, 3 days/week progressive resistance training exercise program is feasible, safe, and well tolerated in children with SMA. These findings can inform future studies of exercise in SMA.

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Leg muscle function and fatigue during walking in spinal muscular atrophy type 3

Abstract: Clinical measures are valid in assessing fatigue and function in SMA, and these assessments can be enhanced by use of gait analysis and EMG. Disease duration and strength measures may represent further stratification refinements when enrolling patients in clinical trials.

Cited by 37 publications

References 23 publications

Gait assessment with solesound instrumented footwear in spinal muscular atrophy

Gait assessment with solesound instrumented footwear in spinal muscular atrophy

Nusinersen improves walking distance and reduces fatigue in later‐onset spinal muscular atrophy

Resistance strength training exercise in children with spinal muscular atrophy

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