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Reliability and Validity of the Korean Version of the Spinal and Bulbar Muscular Atrophy Functional Rating Scale

Journal of Clinical Neurology 2020년 16권 4호 p.586 ~ 591
오성일, 오주연, 박동휘, 손광주, 박진성,
소속 상세정보
오성일 ( Oh Seong-Il ) - Inje University College of Medicine Busan Paik Hospital Department of Neurology
오주연 ( Oh Ju-Yeon ) - Dankook University College of Nursing
박동휘 ( Park Dong-Hwi ) - University of Ulsan College of Medicine Ulsan University Hospital Department of Physical Medicine and Rehabilitation
손광주 ( Son Kwang-Joo ) - Kyungpook National University School of Medicine Kyungpook National University Chilgok Hospital Department of Neurology
박진성 ( Park Jin-Sung ) - Kyungpook National University School of Medicine Kyungpook National University Chilgok Hospital Department of Neurology

Abstract


Background and Purpose: 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.

Methods: 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.

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.

Conclusions: 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.

키워드

spinal and bulbar muscular atrophy; translations; validity and reliability

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