Vlazna Daniela, Krkoska Peter, Sladeckova Michaela, Parmova Olesja, Barusova Tamara, Hrabcova Karolina, Vohanka Stanislav, Matulova Katerina, Adamova Blanka
Department of Neurology, Center for Neuromuscular Diseases (Associated National Center in the European Reference Network ERN EURO-NMD), University Hospital Brno, Brno, Czechia.
Faculty of Medicine, Masaryk University, Brno, Czechia.
Front Neurol. 2023 Oct 26;14:1258342. doi: 10.3389/fneur.2023.1258342. eCollection 2023.
Myotonic dystrophy type 2 (MD2) presents with a varied manifestation. Even though the myopathy in these patients is more widespread, axial musculature involvement is one of the most prominent conditions. MD2 patients also often report chronic low back pain (CLBP). The purpose of this study was to evaluate trunk muscle function, including respiratory muscles, in patients with MD2 and to compare it with healthy controls, to determine the occurrence of CLBP in patients with MD2, and to assess whether trunk muscle dysfunction increases the risk of CLBP in these patients.
We enrolled 40 MD2 patients (age range 23 to 76 years, 26 women). A comprehensive battery of tests was used to evaluate trunk muscle function. The tests consisted of quantitative muscle strength testing of low back extensor muscles and respiratory muscles and the assessment of trunk muscle endurance. A neurological evaluation contained procedures assessing the distribution of muscle weakness, myotonia, and pain, and used questionnaires focused on these items and on disability, depression, and physical activity.
The results of this study suggest that patients with MD2 show significant dysfunction of the trunk muscles, including the respiratory muscles, expressed by decreased muscle strength and endurance. The prevalence of CLBP in patients with MD2 was 52.5%. Based on our analysis, the only independent significant risk factor for CLBP in these patients was maximal isometric lower back extensor strength in a prone position ≤ 15.8 kg (OR = 37.3). Other possible risk factors were severity of myotonia and reduced physical activity.
Outcomes of this study highlighted the presence of axial muscle dysfunction, respiratory muscle weakness, and frequent occurrence of CLBP together with its risk factors in patients with MD2. We believe that the findings of this study may help in management and prevention programs for patients with MD2.
2型强直性肌营养不良（MD2）表现多样。尽管这些患者的肌病更为广泛，但轴性肌肉受累是最突出的情况之一。MD2患者还常报告慢性下腰痛（CLBP）。本研究的目的是评估MD2患者的躯干肌肉功能，包括呼吸肌，并与健康对照进行比较，以确定MD2患者中CLBP的发生率，并评估躯干肌肉功能障碍是否会增加这些患者患CLBP的风险。
我们纳入了40例MD2患者（年龄范围23至76岁，26名女性）。使用一系列综合测试来评估躯干肌肉功能。测试包括下背部伸肌和呼吸肌的定量肌力测试以及躯干肌肉耐力评估。神经学评估包括评估肌肉无力、肌强直和疼痛分布的程序，并使用专注于这些项目以及残疾、抑郁和身体活动的问卷。
本研究结果表明，MD2患者表现出明显的躯干肌肉功能障碍，包括呼吸肌，表现为肌肉力量和耐力下降。MD2患者中CLBP的患病率为52.5%。根据我们的分析，这些患者中CLBP的唯一独立显著风险因素是俯卧位时最大等长下背部伸肌力量≤15.8千克（OR = 37.3）。其他可能的风险因素是肌强直的严重程度和身体活动减少。
本研究结果突出了MD2患者存在轴性肌肉功能障碍、呼吸肌无力以及CLBP的频繁发生及其风险因素。我们认为本研究结果可能有助于MD2患者的管理和预防方案。
