Assessing Function and Endurance in Adults with Spinal and Bulbar Muscular Atrophy: Validity of the Adult Myopathy Assessment Tool

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.

[1]  S. Dal Corso,et al.  Reference Equation for the 2-Minute Walk Test in Adults and the Elderly , 2014, Respiratory Care.

[2]  Todd E. Davenport,et al.  Are Repeated Single-Limb Heel Raises and Manual Muscle Testing Associated With Peak Plantar-Flexor Force in People With Inclusion Body Myositis? , 2013, Physical Therapy.

[3]  R. Kothary,et al.  More than a bystander: the contributions of intrinsic skeletal muscle defects in motor neuron diseases , 2013, Front. Physiol..

[4]  Douglas B. Smith,et al.  Assessment of the benefit of powered exercises for muscular endurance and functional capacity in elderly participants. , 2012, Journal of physical activity & health.

[5]  M. Schroll,et al.  Mobility-related fatigue, walking speed, and muscle strength in older people. , 2012, The journals of gerontology. Series A, Biological sciences and medical sciences.

[6]  B. Dijkmans,et al.  Moving instead of asking? Performance-based tests and BASFI-questionnaire measure different aspects of physical function in ankylosing spondylitis , 2012, Arthritis Research & Therapy.

[7]  Lindsay Alfano,et al.  Knee extensor strength exhibits potential to predict function in sporadic inclusion‐body myositis , 2012, Muscle & nerve.

[8]  K. Fischbeck,et al.  Efficacy and safety of dutasteride in patients with spinal and bulbar muscular atrophy: a randomised placebo-controlled trial , 2011, The Lancet Neurology.

[9]  J. DeLuca,et al.  Actual reality: a new approach to functional assessment in persons with multiple sclerosis. , 2010, Archives of physical medicine and rehabilitation.

[10]  K. Fischbeck,et al.  Clinical features of spinal and bulbar muscular atrophy , 2009, Brain : a journal of neurology.

[11]  N. Chahin,et al.  Serum creatine kinase levels in spinobulbar muscular atrophy and amyotrophic lateral sclerosis , 2009, Muscle & nerve.

[12]  G. Sobue,et al.  Phase 2 trial of leuprorelin in patients with spinal and bulbar muscular atrophy , 2009, Annals of neurology.

[13]  Marybeth Brown,et al.  Skeletal muscle and bone: effect of sex steroids and aging. , 2008, Advances in physiology education.

[14]  J. Mandrekar,et al.  Natural history of spinal-bulbar muscular atrophy , 2008, Neurology.

[15]  G. Lankhorst,et al.  SF36 physical functioning scale and 2-minute walk test advocated as core qualifiers to evaluate physical functioning in patients with late-onset sequelae of poliomyelitis. , 2008, Journal of rehabilitation medicine.

[16]  J M Farmer,et al.  Measuring Friedreich ataxia , 2006, Neurology.

[17]  G. Sobue,et al.  Natural history of spinal and bulbar muscular atrophy (SBMA): a study of 223 Japanese patients. , 2006, Brain : a journal of neurology.

[18]  P. Schmucker,et al.  Quality of life in elderly men with androgen deficiency , 2006, Andrologia.

[19]  I. Lundberg,et al.  Functional index-2: Validity and reliability of a disease-specific measure of impairment in patients with polymyositis and dermatomyositis. , 2006, Arthritis and rheumatism.

[20]  M. Hallett,et al.  Measuring Friedreich ataxia: Interrater reliability of a neurologic rating scale , 2005, Neurology.

[21]  D. Koziol,et al.  PERFORMANCE-BASED ASSESSMENT OF FUNCTIONAL LIMITATION AND MUSCLE ENDURANCE: RELIABILITY OF THE ADULT MYOSITIS ASSESSMENT TOOL. , 2004 .

[22]  P. Kitzman.  CHANGES IN EXCITATORY AND INHIBITORY SYNAPSES ON SACROCAUDAL MOTONEURONS IN THE SCI-INDUCED SPASTIC RAT. , 2004 .

[23]  E. Bertini,et al.  Motor function–muscle strength relationship in spinal muscular atrophy , 2004, Muscle & nerve.

[24]  M. Harris-Love.  Physical activity and disablement in the idiopathic inflammatory myopathies , 2003, Current opinion in rheumatology.

[25]  A J Thompson,et al.  Using the SF-36 measure to compare the health impact of multiple sclerosis and Parkinson’s disease with normal population health profiles , 2003, Journal of neurology, neurosurgery, and psychiatry.

[26]  R. Shephard.  Limits to the measurement of habitual physical activity by questionnaires , 2003, British journal of sports medicine.

[27]  T. Rantanen,et al.  Muscle strength, disability and mortality , 2003, Scandinavian journal of medicine & science in sports.

[28]  Slobodan Jaric,et al.  Role of Body Size in the Relation Between Muscle Strength and Movement Performance , 2003, Exercise and sport sciences reviews.

[29]  R. Lieber.  Skeletal Muscle Structure, Function, and Plasticity: The Physiological Basis of Rehabilitation , 2002 .

[30]  P. Lachenbruch,et al.  Validation of the Childhood Health Assessment Questionnaire in the juvenile idiopathic myopathies. Juvenile Dermatomyositis Disease Activity Collaborative Study Group. , 2001, The Journal of rheumatology.

[31]  G. Salem,et al.  Knee strength and lower- and higher-intensity functional performance in older adults. , 2000, Medicine and science in sports and exercise.

[32]  J D Williamson,et al.  Association of muscle strength with maximum walking speed in disabled older women. , 1998, American journal of physical medicine & rehabilitation.

[33]  G Grimby,et al.  Muscle fatigue in a standing heel-rise test. , 1998, Scandinavian journal of rehabilitation medicine.

[34]  Suzanne G. Leveille,et al.  Racial differences in muscle strength in disabled older women. , 1998, The journals of gerontology. Series A, Biological sciences and medical sciences.

[35]  A. Wilbourn,et al.  The characteristic electrodiagnostic features of Kennedy's disease , 1997, Muscle & nerve.

[36]  National-Isometric-Muscle-Strength-Database-Consor.  Muscular weakness assessment: use of normal isometric strength data. The National Isometric Muscle Strength (NIMS) Database Consortium. , 1996, Archives of physical medicine and rehabilitation.

[37]  E. Romanus,et al.  A functional index in myositis. , 1996, The Journal of rheumatology.

[38]  Marsha P. Johnson.  Statistical Methods for Health Care Research , 1996 .

[39]  R W Bohannon,et al.  Normative values for isometric muscle force measurements obtained with hand-held dynamometers. , 1996, Physical therapy.

[40]  R. Abresch,et al.  Profiles of neuromuscular diseases. Becker's muscular dystrophy. , 1995, American journal of physical medicine & rehabilitation.

[41]  J. Cone,et al.  Validity Issues in Clinical Assessment. , 1995 .

[42]  C. McHorney,et al.  Individual-patient monitoring in clinical practice: are available health status surveys adequate? , 1995, Quality of Life Research.

[43]  R. Rd.  Evaluation of neuromuscular function in inflammatory myopathy , 1994 .

[44]  C. L. Warner,et al.  Meiotic stability and genotype – phenotype correlation of the trinucleotide repeat in X–linked spinal and bulbar muscular atrophy , 1992, Nature Genetics.

[45]  T. Kachi,et al.  Severity of X‐linked recessive bulbospinal neuronopathy correlates with size of the tandem cag repeat in androgen receptor gene , 1992, Annals of neurology.

[46]  K. Fischbeck,et al.  Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy , 1991, Nature.

[47]  J. Miller,et al.  Clinical investigation in duchenne dystrophy: 2. Determination of the “power” of therapeutic trials based on the natural history , 1983, Muscle & nerve.

[48]  A. Woodcock,et al.  Two-, six-, and 12-minute walking tests in respiratory disease. , 1982, British medical journal.

[49]  Leslie G. Portney Dpt PhD Fapta,et al.  Foundations of Clinical Research: Applications to Practice , 2015 .

[50]  A. Williams,et al.  International classification of functioning, disability, and health: ICF-CY World Health Organization , 2013 .

[51]  Akihiko Saito.  Skeletal Muscle Structure. , 2003 .

[52]  D. Wade,et al.  Validity and reliability comparison of 4 mobility measures in patients presenting with neurologic impairment. , 2001, Archives of physical medicine and rehabilitation.

[53]  Andy P. Field,et al.  Discovering Statistics Using SPSS , 2000 .

[54]  E. Brandt,et al.  Enabling America: Assessing the Role of Rehabilitation Science and Engineering , 1997 .

[55]  W. Triggs,et al.  The 2-Minute Walk Test: A Tool for Evaluating Walking Endurance in Clients with Parkinsonʼs Disease , 1997 .

[56]  J. Guralnik.  Assessment of physical performance and disability in older persons , 1997, Muscle & nerve. Supplement.

[57]  R. Abresch,et al.  Profiles of neuromuscular diseases. Duchenne muscular dystrophy. , 1995, American journal of physical medicine & rehabilitation.

[58]  R. Moxley.  Evaluation of neuromuscular function in inflammatory myopathy. , 1994, Rheumatic diseases clinics of North America.

[59]  L. Portney,et al.  Foundations of Clinical Research , 1993 .