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A functional scale for spinal and bulbar muscular atrophy: Cross-sectional and longitudinal study.
- Source :
-
Neuromuscular Disorders . Jul2015, Vol. 25 Issue 7, p554-562. 9p. - Publication Year :
- 2015
-
Abstract
- We aimed to develop, validate, and evaluate a disease-specific outcome measure for SBMA: the Spinal and Bulbar Muscular Atrophy Functional Rating Scale (SBMAFRS). We examined the Japanese version (SBMAFRS-J) in 80 Japanese SBMA subjects to evaluate its validity and reliability. We then assessed this scale longitudinally in 41 additional SBMA subjects. The English version (SBMAFRS-E) was also tested in 15 US subjects. The total score of the SBMAFRS-J was distributed normally without an extreme ceiling or floor effect. For SBMAFRS-J, the high intra- and inter-rater agreement was confirmed (intra-class correlation coefficients [ICCs] 0.910 and 0.797, respectively), and internal consistency was satisfactory (Cronbach's alpha 0.700–0.822). In addition, SBMAFRS-J demonstrated concurrent, convergent, and discriminant validity, except for the respiratory subscale. The inter-rater reliability and internal consistency of SBMAFRS-E were also satisfactory. Longitudinally, SBMAFRS-J showed a higher sensitivity to disease progression than the existing clinical measures. In conclusion, we developed and validated a disease-specific functional rating scale for SBMA in both Japanese and English versions, although it needs to be re-assessed in interventional studies with a larger sample size including English speaking subjects. [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 09608966
- Volume :
- 25
- Issue :
- 7
- Database :
- Academic Search Index
- Journal :
- Neuromuscular Disorders
- Publication Type :
- Academic Journal
- Accession number :
- 103179973
- Full Text :
- https://doi.org/10.1016/j.nmd.2015.03.008