Six-minute walk test as outcome measure of fatigability in adults with spinal muscular atrophy treated with nusinersen.
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Govoni Alessandra, Ricci Giulia, Bonanno Silvia, Bello Luca, Magri Francesca, Meneri Megi, Torri Francesca, Caponnetto Claudia, Passamano Luigia, Grandis Marina, Trojsi Francesca, Cerri Federica, Gadaleta Giulio, Capece Giuliana, Caumo Luca, Tanel Raffaella, Saccani Elena, Vacchiano Veria, Sorarù Gianni, D'Errico Eustachio, Tramacere Irene, Bortolani Sara, Rolle Enrica, Gellera Cinzia, Zanin Riccardo, Silvestrini Mauro, Politano Luisa, Schenone Angelo, Previtali Stefano Carlo, Berardinelli Angela, Turri Mara, Verriello Lorenzo, Coccia Michela, Mantegazza Renato, Liguori Rocco, Filosto Massimiliano, Maioli Maria Antonietta, Simone Isabella Laura, Mongini Tiziana, Corti Stefania, Manca Maria Laura, Pegoraro Elena, Siciliano Gabriele, Comi Giacomo Pietro, Maggi Lorenzo
Аннотация
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.