Safety and Tolerability of Strength Training in Spinal and Bulbar Muscular Atrophy: A Case Report

Vincent Shieh; Cris Zampieri; Paul Stout; Galen O. Joe; Angela Kokkinis; Kenneth H.  Fischbeck; Christopher Grunseich; Joseph A. Shrader

doi:10.2340/jrmcc.v5.2513

Authors

Vincent Shieh Rehabilitation Medicine Department, Clinical Center, National Institutes of Health
Cris Zampieri Rehabilitation Medicine Department, Clinical Center, National Institutes of Health
Paul Stout Rehabilitation Medicine Department, Clinical Center, National Institutes of Health
Galen O. Joe Rehabilitation Medicine Department, Clinical Center, National Institutes of Health
Angela Kokkinis Neurogenetics Branch, National Institute of Neurological Disorders and Stroke and National Institutes of Health, Bethesda, MD, USA
Kenneth H. Fischbeck Neurogenetics Branch, National Institute of Neurological Disorders and Stroke and National Institutes of Health, Bethesda, MD, USA
Christopher Grunseich Neurogenetics Branch, National Institute of Neurological Disorders and Stroke and National Institutes of Health, Bethesda, MD, USA
Joseph A. Shrader Rehabilitation Medicine Department, Clinical Center, National Institutes of Health

DOI:

https://doi.org/10.2340/jrmcc.v5.2513

Keywords:

motor neurone disease, spinal and bulbar muscular atrophy, weight lifting, neuromuscular disease

Abstract

Objective: Spinal and bulbar muscular atrophy is characterized by slow-progressive muscle weakness, decreased functional performance and falls. Research into the use of exercise in spinal and bulbar muscular atrophy has shown equivocal to negative results, although authors suggest that patients with spinal and bulbar muscular atrophy may benefit from both increased exercise intensity and shorter bout duration. The aim of this case report is to explore the safety of a moderate-intensity strength training programme coupled with dynamic balance and function-specific training in a patient with spinal and bulbar muscular atrophy.
Case report: A 56-year-old man with spinal and bulbar muscular atrophy presented with multiple falls and declining performance in physical, vocational, and recreational activities. Examination revealed several musculoskeletal impairments that were sub-clinical to mild compared with an SBMA natural history cohort.
Intervention and outcome: A 15-week moderate-intensity exercise programme combining weightlifting and functional exercises was performed under clinical supervision. Exercise volume, frequency and intensity were adjusted based on patient-reported outcomes and muscle damage blood markers. Performance-based and selfreported functional improvements occurred that exceeded the minimal clinically important difference. The intervention was well tolerated and the patient nearly doubled his baseline 10-repetition maximums for weight-lifting exercises.
Conclusion: Exercise therapy combining weightlifting and upright functional training led to meaningful performance improvements in this case of a patient with spinal and bulbar muscular atrophy and relatively low disease burden.

LAY ABSTRACT
Spinal and bulbar muscular atrophy (SBMA) is a rare neuromuscular disease characterized by slow-progressive muscle weakness, decreased functional performance, and falling. With limited research for guidance, medical practitioners often advise patients with SBMA to avoid weight lifting or intensive exercise. The patient was a high-functioning 56-year-old man with SBMA who struggled with performing daily activities and intensive physical work demands. He participated in a closely monitored 15-week exercise program that combined weight lifting and functional exercises. The patient safely tolerated the program, self-reported physical improvements, and nearly doubled the weight for lifting exercises. This case report highlights one individual with SBMA who benefitted from moderate-intensity exercise, including weight lifting, under careful clinical supervision. More research is needed before this intervention can be recommended for people with SBMA.