Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2022 Feb;32(2):125-134.
doi: 10.1016/j.nmd.2021.08.010. Epub 2021 Aug 24.

Natural history of 10-meter walk/run test performance in spinal muscular atrophy: A longitudinal analysis

Kristin J Krosschell  1 , Elise L Townsend  2 , Michael Kiefer  3 , Sarah D Simeone  4 , Katelyn Zumpf  5 , Leah Welty  5 , Kathryn J Swoboda  4
Affiliations

Natural history of 10-meter walk/run test performance in spinal muscular atrophy: A longitudinal analysis

Kristin J Krosschell et al. Neuromuscul Disord. 2022 Feb.

Abstract

As trials and treatments for spinal muscular atrophy (SMA) rapidly evolve, understanding the natural history and potential utility of the 10-meter walk/run test (10MWRT) in ambulant individuals is critical. Study aims were to: 1) establish change over time and across age for 10MWRT time in an untreated natural history cohort of young, ambulatory participants with SMA and 2) identify relations between 10MWRT time and age, SMA type, SMN2 copy number and anthropometrics. Untreated individuals (n = 56) age 2 to 21 years who were enrolled in a long-term natural history study between 2005 and 2014 and met inclusion criteria were included. Linear mixed effects models were used to assess changes in 10MWRT time with age and associations with SMA type, SMN2 copy number, and body mass. SMA type 3b (versus 3a), SMN2 copy number 4 (versus 3) and lower body mass were associated with faster 10MWRT. 10MWRT performance improved between 3 and 8 years of age, was stable between 9 and 10, and gradually declined from 11 to 18. Findings provide the first longitudinal natural history report of 10MWRT time in young individuals with SMA and offer a critical foundation for interpreting childhood change in short distance walking speed with pharmacologic treatment.

Keywords: 10-meter walk/run test; 10MWRT; Natural history; Outcome measures; Spinal muscular atrophy; Timed function tests.

PubMed Disclaimer

Conflict of interest statement

Declaration of Competing Interests The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Figures

Figure 1
Figure 1
Consort Diagram
Figure 2:
Figure 2:
Predicted Values of 10-meter Walk/Run Test (10MWRT) Time by Age. Predictions for 3 SMN2 copies, SMA type 3a, mean BMI=16.80, and no VPA event.
Figure 3:
Figure 3:
Predicted Values of 10-meter Walk/Run Test (10MWRT) Time by Age and Copy Number. Predictions for SMA type 3a, mean BMI=16.80 and no VPA event.
Figure 4:
Figure 4:
Predicted Values of 10-meter Walk/Run Test (10MWRT) Time by Age and BMI. Predictions for 3 SMN2 copies, SMA type 3a, and no VPA event
See this image and copyright information in PMC

References

    1. Lefebvre S, Bürglen L, Reboullet S, Clermont O, Burlet P, Viollet L, et al. Identification and characterization of a spinal muscular atrophy-determining gene. Cell 1995;80:155–65. 10.1016/0092-8674(95)90460-3. - DOI - PubMed
    1. Wang CH, Finkel RS, Bertini ES, Schroth M, Simonds A, Wong B, et al. Consensus statement for standard of care in spinal muscular atrophy. J Child Neurol 2007;22:1027–49. 10.1177/0883073807305788 - DOI - PubMed
    1. Mercuri E, Finkel RS, Muntoni F, Wirth B, Montes J, Main M, et al. Diagnosis and management of spinal muscular atrophy: Part 1: Recommendations for diagnosis, rehabilitation, orthopedic and nutritional care. Neuromuscul Disord NMD 2018;28:103–15. doi: 10.1016/j.nmd.2017.11.005 - DOI - PubMed
    1. Finkel RS, Mercuri E, Meyer OH, Simonds AK, Schroth MK, Graham RJ, et al. Diagnosis and management of spinal muscular atrophy: Part 2: Pulmonary and acute care; medications, supplements and immunizations; other organ systems; and ethics. Neuromuscul Disord NMD 2018;28:197–207. doi:10.1016/j.nmd.2017.11.004 - DOI - PubMed
    1. Montes J, McDermott MP, Mirek E, Mazzone ES, Main M, Glanzman AM, et al. Ambulatory function in spinal muscular atrophy: Age-related patterns of progression. PLOS ONE 2018;13:e0199657. doi: 10.1371/journal.pone.0199657 - DOI - PMC - PubMed

Publication types

LinkOut - more resources