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
. 2011 Aug;44(2):246-51.
doi: 10.1002/mus.22040. Epub 2011 Jun 22.

Reliability of the Modified Hammersmith Functional Motor Scale in young children with spinal muscular atrophy

Affiliations

Reliability of the Modified Hammersmith Functional Motor Scale in young children with spinal muscular atrophy

Kristin J Krosschell et al. Muscle Nerve. 2011 Aug.

Abstract

Introduction: The test-retest reliability of the Modified Hammersmith Functional Motor Scale (MHFMS) in children with spinal muscular atrophy (SMA) ≤30 months of age was assessed. The age at which typically developing children (TD) achieve maximum MHFMS scores was also studied.

Methods: Twenty-two children with SMA type II [mean age (SD) = 20 (5) months, range 9-30 months) were tested twice using the MHFMS. Twenty-five TD children [mean age (SD) = 18 (7) months, range 9-30 months) were tested once.

Results: The average difference between MHFMS scores for SMA children was 0.18 [first assessment: mean (SD) = 12.8 (9.8); second assessment: mean (SD) = 13.0 (8.8)]. Reliability was excellent (ICC(1,3) = 0.96, SEM 1.86). TD participants had MHFMS scores ranging from 36 to 40 [mean (SD) = 39.2 (1.2)] and achieved maximum test scores at 12 months of age.

Discussion: MHFMS scores in young children with SMA type II showed excellent test-retest stability. This suggests that the MHFMS can be used reliably in this younger population for clinical trials and follow-up.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Bivariate Fit of Score of first visit by score from second visit in SMA Type II patients ≤ 30 months.
Figure 2
Figure 2
Modified Hammersmith Functional Motor Scale scores in typically developing children. Scores ≥ 39 indicate that subjects were able to perform all test items.

References

    1. Wirth B. An update of the mutation spectrum of the survival motor neuron gene (SMN1) in autosomal recessive spinal muscular atrophy (SMA) Hum Mutat. 2000;15(3):228–37. - PubMed
    1. Crawford TO, Pardo CA. The neurobiology of childhood spinal muscular atrophy. Neurobiol Dis. 1996;3(2):97–110. - PubMed
    1. Swoboda KJ, Kissel JT, Crawford TO, et al. Perspectives on clinical trials in spinal muscular atrophy. J Child Neurol. 2007;22(8):957–66. - PMC - PubMed
    1. Moxley RT., 3rd Functional testing. Muscle Nerve. 1990;13 (Suppl):S26–9. - PubMed
    1. Main M, Kairon H, Mercuri E, Muntoni F. The Hammersmith functional motor scale for children with spinal muscular atrophy: a scale to test ability and monitor progress in children with limited ambulation. Eur J Paediatr Neurol. 2003;7(4):155–9. - PubMed

Publication types