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Multicenter Study
. 2024 Oct;70(4):816-823.
doi: 10.1002/mus.28225. Epub 2024 Aug 2.

Six-minute walk test as outcome measure of fatigability in adults with spinal muscular atrophy treated with nusinersen

Alessandra Govoni  1   2 , Giulia Ricci  2 , Silvia Bonanno  3 , Luca Bello  4 , Francesca Magri  5 , Megi Meneri  1   6 , Francesca Torri  2 , Claudia Caponnetto  7 , Luigia Passamano  8 , Marina Grandis  7   9 , Francesca Trojsi  10 , Federica Cerri  11 , Giulio Gadaleta  12 , Giuliana Capece  4 , Luca Caumo  4 , Raffaella Tanel  13 , Elena Saccani  14 , Veria Vacchiano  15   16 , Gianni Sorarù  4 , Eustachio D'Errico  17   18 , Irene Tramacere  19 , Sara Bortolani  12 , Enrica Rolle  12 , Cinzia Gellera  20 , Riccardo Zanin  21 , Mauro Silvestrini  22   23 , Luisa Politano  8 , Angelo Schenone  7   9 , Stefano Carlo Previtali  11 , Angela Berardinelli  24 , Mara Turri  25 , Lorenzo Verriello  26 , Michela Coccia  23 , Renato Mantegazza  3 , Rocco Liguori  15   16 , Massimiliano Filosto  27   28 , Maria Antonietta Maioli  29 , Isabella Laura Simone  17   18 , Tiziana Mongini  12 , Stefania Corti  1   6 , Maria Laura Manca  2   30 , Elena Pegoraro  4 , Gabriele Siciliano  2 , Giacomo Pietro Comi  1   6 , Lorenzo Maggi  3
Affiliations
Free article
Multicenter Study

Six-minute walk test as outcome measure of fatigability in adults with spinal muscular atrophy treated with nusinersen

Alessandra Govoni et al. Muscle Nerve. 2024 Oct.
Free article

Abstract

Introduction/aims: Fatigue (subjective perception) and fatigability (objective motor performance worsening) are relevant aspects of disability in individuals with spinal muscular atrophy (SMA). The effect of nusinersen on fatigability in SMA patients has been investigated with conflicting results. We aimed to evaluate this in adult with SMA3.

Methods: We conducted a multicenter retrospective cohort study, including adult ambulant patients with SMA3, data available on 6-minute walk test (6MWT) and Hammersmith Functional Motor Scale-Expanded (HFMSE) at baseline and at least at 6 months of treatment with nusinersen. We investigated fatigability, estimated as 10% or higher decrease in walked distance between the first and sixth minute of the 6MWT, at baseline and over the 14-month follow-up.

Results: Forty-eight patients (56% females) were included. The 6MWT improved after 6, 10, and 14 months of treatment (p < 0.05). Of the 27 patients who completed the entire follow-up, 37% improved (6MWT distance increase ≥30 m), 48.2% remained stable, and 14.8% worsened (6MWT distance decline ≥30 m). Fatigability was found at baseline in 26/38 (68%) patients and confirmed at subsequent time points (p < 0.05) without any significant change over the treatment period. There was no correlation between fatigability and SMN2 copy number, sex, age at disease onset, age at baseline, nor with 6MWT total distance and baseline HFMSE score.

Discussion: Fatigability was detected at baseline in approximately 2/3 of SMA3 walker patients, without any correlation with clinical features, included motor performance. No effect on fatigability was observed during the 14-month treatment period with nusinersen.

Keywords: 6MWT; SMA; fatigability; fatigue; nusinersen.

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References

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