Frezza Erica, Merra Giuseppe, Greco Giulia, Goglia Mariangela, Seraceno Silvia, Boffa Laura, Mercuri Nicola B, De Lorenzo Antonino, Massa Roberto
Neuromuscular Diseases Unit, Policlinico Tor Vergata, Rome, Italy.
Department of Systems Medicine, Tor Vergata University of Rome, Rome, Italy.
Front Neurol. 2024 Dec 4;15:1451537. doi: 10.3389/fneur.2024.1451537. eCollection 2024.
In myotonic dystrophy type 2 (DM2), metabolic dysfunctions are frequent. Therefore, measurement of muscle mass and body composition by non-invasive methods could help in evaluating disease severity and progression. The aim of our study was to investigate, by means of bioelectrical impedance analysis (BIA), whether DM2 patients have an alteration in their body composition and if this finding correlates with strength and motor performances.
We obtained anthropometric measures, nutritional data, BIA and blood tests in 18 DM2 patients and correlated them with motor function tests.
The 33% of male and 22% of female patients had BMI values compatible with severe obesity, whereas 44% of males and 33% of females had a metabolic syndrome. Considering BIA parameters, phase angle was under normal values in 56% of males and 89% of females. This may be regarded as a marker of deranged cell membrane function. Motor tests showed a fair to strong direct correlation with several BIA parameters.
These data suggest that BIA findings may be faithful markers of the degree of muscle wasting and impairment in DM2. The different degree of BIA alterations between genders indicate that body composition undergoes sex-related modifications in this disease. The potential of this technique to capture changes in a slowly progressive disorder such as DM2 should be tested in longitudinal studies.
在2型强直性肌营养不良症（DM2）中，代谢功能障碍很常见。因此，通过非侵入性方法测量肌肉质量和身体成分有助于评估疾病的严重程度和进展。我们研究的目的是通过生物电阻抗分析（BIA）来调查DM2患者的身体成分是否发生改变，以及这一发现是否与力量和运动表现相关。
我们获取了18例DM2患者的人体测量数据、营养数据、BIA和血液检查结果，并将它们与运动功能测试结果进行关联。
33%的男性患者和22%的女性患者的BMI值符合重度肥胖，而44%的男性患者和33%的女性患者患有代谢综合征。考虑BIA参数，56%的男性患者和89%的女性患者的相位角低于正常值。这可能被视为细胞膜功能紊乱的一个标志。运动测试显示与几个BIA参数存在中等至强的直接相关性。
这些数据表明，BIA结果可能是DM2中肌肉萎缩程度和损伤的可靠标志。性别之间BIA改变程度的差异表明，在这种疾病中身体成分会发生与性别相关的变化。这种技术在捕捉诸如DM2等缓慢进展性疾病变化方面的潜力应在纵向研究中进行测试。
