Assessing function and endurance in adults with spinal and bulbar muscular atrophy: validity of the adult myopathy assessment tool

Michael O Harris-Love, Lindsay Fernandez-Rhodes, Galen Joe, Joseph A Shrader, Angela Kokkinis, Alison La Pean Kirschner, Sungyoung Auh, Cheunju Chen, Li Li, Ellen Levy, Todd E Davenport, Nicholas A Di Prospero, Kenneth H Fischbeck, Michael O Harris-Love, Lindsay Fernandez-Rhodes, Galen Joe, Joseph A Shrader, Angela Kokkinis, Alison La Pean Kirschner, Sungyoung Auh, Cheunju Chen, Li Li, Ellen Levy, Todd E Davenport, Nicholas A Di Prospero, Kenneth H Fischbeck

Abstract

Purpose. The adult myopathy assessment tool (AMAT) is a performance-based battery comprised of functional and endurance subscales that can be completed in approximately 30 minutes without the use of specialized equipment. The purpose of this study was to determine the construct validity and internal consistency of the AMAT with a sample of adults with spinal and bulbar muscular atrophy (SBMA). Methods. AMAT validity was assessed in 56-male participants with genetically confirmed SBMA (mean age, 53 ± 10 years). The participants completed the AMAT and assessments for disease status, strength, and functional status. Results. Lower AMAT scores were associated with longer disease duration (r = -0.29; P < 0.03) and lower serum androgen levels (r = 0.49-0.59; P < 0.001). The AMAT was significantly correlated with strength and functional status (r = 0.82-0.88; P < 0.001). The domains of the AMAT exhibited good internal consistency (Cronbach's α = 0.77-0.89; P < 0.001). Conclusions. The AMAT is a standardized, performance-based tool that may be used to assess functional limitations and muscle endurance. The AMAT has good internal consistency, and the construct validity of the AMAT is supported by its significant associations with hormonal, strength, and functional characteristics of adults with SBMA. This trial is registered with Clinicaltrials.gov identifier NCT00303446.

Figures

Figure 1
Figure 1
Isometric maximal voluntary contraction testing via the quantitative muscle assessment device. (Participant positioning shown for the: (a) hip extensors and (b) elbow flexors.)

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