Shieh Vincent, Zampieri Cris, Stout Paul, Joe Galen O, Kokkinis Angela, Fischbeck Kenneth H, Grunseich Christopher, Shrader Joseph A
Rehabilitation Medicine Department, Clinical Center, National Institutes of Health.
Neurogenetics Branch, National Institute of Neurological Disorders and Stroke and National Institutes of Health, Bethesda, MD, USA.
J Rehabil Med Clin Commun. 2022 Aug 18;5:2513. doi: 10.2340/jrmcc.v5.2513. eCollection 2022.
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.
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.
A 15-week moderate intensity exercise programme combining weight-lifting 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 self-reported 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.
Exercise therapy combining weight-lifting 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.
脊髓延髓肌肉萎缩症的特征为肌肉无力缓慢进展、功能表现下降及跌倒。对脊髓延髓肌肉萎缩症患者运动疗法的研究结果不一，甚至呈阴性，不过有作者指出，脊髓延髓肌肉萎缩症患者可能会从增加运动强度及缩短运动时长中获益。本病例报告旨在探讨在一名脊髓延髓肌肉萎缩症患者中，进行中等强度力量训练计划并结合动态平衡和功能特定训练的安全性。
一名56岁脊髓延髓肌肉萎缩症男性患者，出现多次跌倒，身体、职业及娱乐活动表现下降。检查发现与脊髓延髓肌肉萎缩症自然病史队列相比，存在一些亚临床至轻度的肌肉骨骼损伤。
在临床监督下进行了为期15周的中等强度运动计划，该计划结合了举重和功能锻炼。根据患者报告的结果及肌肉损伤血液指标，调整了运动量、频率和强度。基于表现和自我报告的功能均有改善，且超过了最小临床重要差异。该干预措施耐受性良好，患者举重练习的基线10次重复最大值几乎翻倍。
在该例疾病负担相对较低的脊髓延髓肌肉萎缩症患者中，结合举重和直立功能训练的运动疗法带来了有意义的功能改善。
