title: Assessing function and endurance in adults with spinal and bulbar muscular atrophy: validity of the adult myopathy assessment tool.
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.
authors.affiliation: Research Service/Geriatrics and Extended Care, Washington, DC Veterans Affairs Medical Center, 50 Irving Street, NW, Room 11G, Washington, DC 20422, USA ; School of Public Health and Health Services, George Washington University, 2033 K Street, NW, Suite 210, Washington, DC 20006, USA ; Rehabilitation Medicine Department, Clinical Center, Department of Health and Human Services (DHHS), National Institutes of Health (NIH), 10 Center Drive, Bethesda, MD 20892, USA.
authors.forename: Michael O
authors.initials: MO
authors.lastname: Harris-Love
authors.affiliation: National Institute of Neurological Disorders and Stroke (NINDS), Neurogenetics Branch, Department of Health and Human Services (DHHS), National Institutes of Health (NIH), Building 35, Room 2A-1000, 35 Convent Drive, MSC 3705, Bethesda, MD 20892, USA ; Department of Epidemiology, University of North Carolina at Chapel Hill Gillings, School of Global Public Health, 170 Rosenau Hall, Campus Box 7400, 135 Dauer Drive, Chapel Hill, NC 27599, USA.
authors.forename: Lindsay
authors.initials: L
authors.lastname: Fernandez-Rhodes
authors.affiliation: Rehabilitation Medicine Department, Clinical Center, Department of Health and Human Services (DHHS), National Institutes of Health (NIH), 10 Center Drive, Bethesda, MD 20892, USA.
authors.forename: Galen
authors.initials: G
authors.lastname: Joe
authors.affiliation: Rehabilitation Medicine Department, Clinical Center, Department of Health and Human Services (DHHS), National Institutes of Health (NIH), 10 Center Drive, Bethesda, MD 20892, USA.
authors.forename: Joseph A
authors.initials: JA
authors.lastname: Shrader
authors.affiliation: National Institute of Neurological Disorders and Stroke (NINDS), Neurogenetics Branch, Department of Health and Human Services (DHHS), National Institutes of Health (NIH), Building 35, Room 2A-1000, 35 Convent Drive, MSC 3705, Bethesda, MD 20892, USA.
authors.forename: Angela
authors.initials: A
authors.lastname: Kokkinis
authors.affiliation: National Institute of Neurological Disorders and Stroke (NINDS), Neurogenetics Branch, Department of Health and Human Services (DHHS), National Institutes of Health (NIH), Building 35, Room 2A-1000, 35 Convent Drive, MSC 3705, Bethesda, MD 20892, USA ; Center for Patient Care and Outcomes Research, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI 53226, USA.
authors.forename: Alison
authors.initials: A
authors.lastname: La Pean Kirschner
authors.affiliation: Clinical Neurosciences Program, NINDS, NIH, 10 Center Drive, Room 5N230, Bethesda, MD 20814, USA.
authors.forename: Sungyoung
authors.initials: S
authors.lastname: Auh
authors.affiliation: National Institute of Neurological Disorders and Stroke (NINDS), Neurogenetics Branch, Department of Health and Human Services (DHHS), National Institutes of Health (NIH), Building 35, Room 2A-1000, 35 Convent Drive, MSC 3705, Bethesda, MD 20892, USA ; Neurology Department, University of Maryland, 110 South Paca Street, Baltimore, MD 21201, USA.
authors.forename: Cheunju
authors.initials: C
authors.lastname: Chen
authors.affiliation: Rehabilitation Medicine Department, Clinical Center, Department of Health and Human Services (DHHS), National Institutes of Health (NIH), 10 Center Drive, Bethesda, MD 20892, USA ; Physical Medicine and Rehabilitation Service, Veterans Affairs Medical Center, 650 East Indian School Road, Phoenix AZ 85012, USA.
authors.forename: Li
authors.initials: L
authors.lastname: Li
authors.affiliation: Rehabilitation Medicine Department, Clinical Center, Department of Health and Human Services (DHHS), National Institutes of Health (NIH), 10 Center Drive, Bethesda, MD 20892, USA.
authors.forename: Ellen
authors.initials: E
authors.lastname: Levy
authors.affiliation: Department of Physical Therapy, Thomas J. Long School of Pharmacy & Health Sciences, University of the Pacific, 3601 Pacific Avenue, Stockton, CA 95211, USA.
authors.forename: Todd E
authors.initials: TE
authors.lastname: Davenport
authors.affiliation: National Institute of Neurological Disorders and Stroke (NINDS), Neurogenetics Branch, Department of Health and Human Services (DHHS), National Institutes of Health (NIH), Building 35, Room 2A-1000, 35 Convent Drive, MSC 3705, Bethesda, MD 20892, USA.
authors.forename: Nicholas A
authors.initials: NA
authors.lastname: Di Prospero
authors.affiliation: National Institute of Neurological Disorders and Stroke (NINDS), Neurogenetics Branch, Department of Health and Human Services (DHHS), National Institutes of Health (NIH), Building 35, Room 2A-1000, 35 Convent Drive, MSC 3705, Bethesda, MD 20892, USA.
authors.forename: Kenneth H
authors.initials: KH
authors.lastname: Fischbeck