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

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

Krosschell K J; Maczulski J A; Crawford T O; Scott C; Swoboda K J

Neuromuscular Disorders

2006

Male; Severity of Illness Index; Videotape Recording; Humans; Child Preschool; Reproducibility of Results; Infant; Disability Evaluation; Observer Variation; Contracture/di [Diagnosis]; Contracture/pp [Physiopathology]; Spinal Muscular Atrophies of Childhood/di [Diagnosis]; Spinal Muscular Atrophies of Childhood/pp [Physiopathology]; tone and motor problems; SMA1; tool development; scale development; HFMS

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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