Oh Seong Il, Oh Juyeon, Park Donghwi, Son Kwangjoo, Park Jin Sung
Department of Neurology, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea.
College of Nursing, Dankook University, Cheonan, Korea.
J Clin Neurol. 2020 Oct;16(4):586-591. doi: 10.3988/jcn.2020.16.4.586.
The Spinal and Bulbar Muscular Atrophy Functional Rating Scale (SBMAFRS) is a reliable and valid instrument for evaluating the functional status of patients with spinal and bulbar muscular atrophy (SBMA). This study aimed to validate a Korean version of the SBMAFRS in an SBMA population.
We applied the SBMAFRS to 64 SBMA patients at their regular follow-up clinical visits. The patients underwent clinical evaluations that included the 6-minute walking test (6MWT), forced vital capacity (FVC), manual muscle test, and the Penetration-Aspiration Scale (PAS). To estimate the stability of the SBMAFRS, the scale was reapplied to a subset of 31 randomly selected patients within 4 weeks of the initial test. The convergent validity was evaluated, and correlations were examined between SBMAFRS items and the muscular force, the total and subscores on the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R), FVC, PAS score, age at onset, disease duration, and 6MWT results.
The internal consistency of the scale was confirmed by a high Cronbach's alpha (total raw alpha=0.867, total standardized alpha=0.863). The test-retest reliability as assessed by Spearman's rho was also high. The total score and subscores of the SBMAFRS were strongly correlated with the respective items and subscores of the ALSFRS-R, respiratory function, and the 6MWT.
We have performed a validation study of the Korean version of a disease-specific functional rating scale for SBMA patients. The SBMAFRS is a useful tool for clinical practice and as a potential outcome measure for Korean SBMA patients.
脊髓延髓肌肉萎缩功能评定量表（SBMAFRS）是评估脊髓延髓肌肉萎缩（SBMA）患者功能状态的可靠且有效的工具。本研究旨在验证SBMAFRS韩语版在SBMA患者群体中的有效性。
我们在64例SBMA患者的定期随访临床就诊时应用了SBMAFRS。患者接受了临床评估，包括6分钟步行试验（6MWT）、用力肺活量（FVC）、徒手肌力测试和吞咽障碍评估量表（PAS）。为评估SBMAFRS的稳定性，在初次测试后4周内，对随机选取的31例患者再次应用该量表。评估了其收敛效度，并检查了SBMAFRS各项与肌力、修订的肌萎缩侧索硬化功能评定量表（ALSFRS-R）的总分及分项分数、FVC、PAS评分、发病年龄、病程以及6MWT结果之间的相关性。
通过高Cronbach's α系数（总原始α = 0.867，总标准化α = 0.863）证实了该量表的内部一致性。通过Spearman's rho评估的重测信度也很高。SBMAFRS的总分及分项分数与ALSFRS-R的相应项目及分项分数、呼吸功能和6MWT均密切相关。
我们对SBMA患者疾病特异性功能评定量表的韩语版进行了验证研究。SBMAFRS是临床实践中的有用工具，也是韩国SBMA患者潜在的结局指标。
