Jacqueline Montes, Cara H Kanner, Ton Duong, Rafael Rodriguez-Torres, David Uher, Sally Dunaway Young, Rabia Farooquee, Abby Druffner, Amy Pasternak, Maria Fragala-Pinkham, Damiano Zanotto
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引用次数: 0
Abstract
Introduction/aims: Despite disease-modifying therapies, fatigability persists in spinal muscular atrophy (SMA). Performance fatigability (PF) during the six-minute walk test (6MWT) is mostly unchanged in treated SMA. This cross-sectional study characterized PF using instrumented insoles.
Methods: Ambulatory individuals with SMA (n = 14) and controls (HC) (n = 10) were included. Spatiotemporal and kinetic parameters were collected with custom-engineered instrumented insoles during the 6MWT. Linear mixed models analyzed parameter trends, with trend slope representing PF. Changes in mean velocity (Vavg) and stride-by-stride parameters were compared between minute 1 and 6 and between groups.
Results: Decreases in Vavg were greatest for severe SMA (p < 0.001). Changes were found in stride length (SL) (p = 0.048) and stride velocity (SV) (p = 0.030) for severe SMA, and in stance phase (%St) (p = 0.012) and percent terminal double support (%DS) (p = 0.02) for mild SMA. SMA subgroups showed downward trends from minute 1 to 6 in SL, SV, and anterior-posterior center of pressure (AP-COP) (p < 0.001), and increases in Absolute COP-Cyclogram Asymmetry Index (|ASI|) (p < 0.05). Trends differed between severe SMA and other groups for SL, SV, %St, and %DS (p < 0.001), and for AP-COP and |ASI| (p < 0.05). Trends for SL (p < 0.001), SV and AP-COP (p < 0.01) differed between HC and mild SMA.
Discussion: PF in SMA manifests as changes in gait parameters. Instrumented insoles revealed fatigue-related changes not captured with the conventional method of comparing the first and last minutes of the 6MWT. Spatiotemporal and kinetic parameters contribute to understanding of impairments and inform therapeutic development.
期刊介绍:
Muscle & Nerve is an international and interdisciplinary publication of original contributions, in both health and disease, concerning studies of the muscle, the neuromuscular junction, the peripheral motor, sensory and autonomic neurons, and the central nervous system where the behavior of the peripheral nervous system is clarified. Appearing monthly, Muscle & Nerve publishes clinical studies and clinically relevant research reports in the fields of anatomy, biochemistry, cell biology, electrophysiology and electrodiagnosis, epidemiology, genetics, immunology, pathology, pharmacology, physiology, toxicology, and virology. The Journal welcomes articles and reports on basic clinical electrophysiology and electrodiagnosis. We expedite some papers dealing with timely topics to keep up with the fast-moving pace of science, based on the referees'' recommendation.