In:
Journal of Child Neurology, SAGE Publications, Vol. 26, No. 12 ( 2011-12), p. 1499-1507
Abstract:
The relationships between the Expanded Hammersmith Functional Motor Scale (HFMSE) and genotype and motor and respiratory outcomes were examined in patients with spinal muscular atrophy types II and III ( n = 70). The correlation between the HFMSE and Gross Motor Function Measure was r = 0.98. Correlations between HFMSE and forced vital capacity (percentage of predicted normal) ( n = 56) and a functional rating ( n = 57) were r = 0.87 and r = 0.92, respectively. Correlations with strength were as follows: knee extension, r = 0.74 ( n = 60); elbow flexion, r = 0.77 ( n = 61); and knee flexion, r = 0.74 ( n = 58). The HFMSE differentiated patients by SMN2 copy number ( P = .0007); bi-level positive airway pressure use, 〈 8 versus ≥8 hours/day ( P 〈 .0001); ambulatory status ( P 〈 .0001); and spinal muscular atrophy type ( P 〈 .0001). The HFMSE demonstrates significant associations with established measures of function, strength, and genotype, and discriminates patients based on function, diagnostic category, and bi-level positive airway pressure need. Time of administration averaged 12 minutes. The HFMSE is a valid, time-efficient outcome measure for clinical trials in spinal muscular atrophy types II and III.
Type of Medium:
Online Resource
ISSN:
0883-0738
,
1708-8283
DOI:
10.1177/0883073811420294
Language:
English
Publisher:
SAGE Publications
Publication Date:
2011
detail.hit.zdb_id:
2068710-2
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