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Clinical Trial
. 2006 Jul;16(7):417-26.
doi: 10.1016/j.nmd.2006.03.015. Epub 2006 Jun 5.

A modified Hammersmith functional motor scale for use in multi-center research on spinal muscular atrophy

Kristin J Krosschell  1 , Jo Anne Maczulski, Thomas O Crawford, Charles Scott, Kathryn J Swoboda
Affiliations
Clinical Trial

A modified Hammersmith functional motor scale for use in multi-center research on spinal muscular atrophy

Kristin J Krosschell et al. Neuromuscul Disord. 2006 Jul.

Abstract

The Hammersmith functional motor scale for children with spinal muscular atrophy was modified to establish a standard measure of functional ability in children with non-ambulant spinal muscular atrophy types 2 and 3 in a longitudinal multi-center clinical trial. This study assessed the intra- and interrater reliability and the test-retest stability of a modified version of the scale. Both intra- and interrater reliability were established. Results indicate that the scale is reliable and stable over a 6 month period. Reliability was maintained when patient sample criteria were expanded to include children younger than 30 months and children with popliteal angles greater than 20 degrees . These data establish the modified Hammersmith functional motor scale for children with spinal muscular atrophy as a reliable instrument for use in multi-center treatment trials in non-ambulant spinal muscular atrophy children. Our data provides additional support for the use of original scale items in terms of ease of administration, usefulness and reliability, while incorporating modifications to optimize its use in a multi-center clinical research setting.

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Figures

Fig. 1
Fig. 1
Sample of test item criterion with operational definitions, scoring criteria and scoring examples from the modified-Hammersmith functional motor scale for children with SMA test manual for test item ‘rolling prone to supine’.
Fig. 2
Fig. 2
Sample of test item criterion with operational definitions, scoring criteria and scoring examples from the modified-Hammersmith functional motor scale for children with SMA test manual for test item ‘floor/chair sitting’.
Fig. 3
Fig. 3
Test–retest stability of the modified-Hammersmith functional motor scale for children with SMA. Mean scores for each group at T0 and T1.
Fig. 4
Fig. 4
Summary of modified-Hammersmith functional motor scale for children with SMA scores for patients in each identified subgroup. Y-Score- all patients between 2 and 12 years with popliteal angles <20°, G-Score- all patients under 2 years of age, R-score- all patients between 2 and 12 years with popliteal angles >20°.
Fig. 5
Fig. 5
Distribution of scores for types 2 and 3 patients used in discriminative validity analysis. Of note: both type 3 children with lower scores demonstrated significant issues with behavior and cooperation at time of testing.
See this image and copyright information in PMC

References

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