Division of Neuroscience, Department of Neurology, Institute of Experimental Neurology (INSPE), IRCCS San Raffaele Scientific Institute, Milan, Italy.
Department of Neurosciences, Sciences University of Padova, Padova, Italy.
J Neurol. 2018 Dec;265(12):2927-2933. doi: 10.1007/s00415-018-9081-7. Epub 2018 Oct 10.
Although anti-myelin-associated glycoprotein (MAG) antibody neuropathy is reported as a slowly progressive disease, it can lead to significant disability and impairment of health-related quality of life (HR-QoL) and social participation. The aim of this cross-sectional study was to evaluate the functioning and HR-QoL determinants in 67 patients with anti-MAG neuropathy in terms of the International Classification of Functioning, Disability, and Health (ICF). Evaluations included: Medical Research Council (MRC) sum score, Sensory Modality Sum score (SMS), Berg balance scale (BBS), Fatigue Severity Scale (FSS), Visual Analogue Scale (VAS) for pain, 9-Hole Peg Test (9-HPT), 6-min Walk Distance (6MWD), Impact on Participation and Autonomy (IPA) and the physical component score (PCS) and mental component score (MCS) of the short-form-36 health status scale (SF-36) HR-QoL measure. In the regression models, 6MWD was the most reliable predictor of PCS, explaining the 52% of its variance, while the strongest determinants of 6MWD were BBS and FSS, explaining the 41% of its variance. Consistently, VAS and BBS were good predictor of PCS, explaining together 54% of its variance. FSS was the most reliable determinant of MCS, explaining 25% of its variance. SMS and MRC were not QoL determinants. The results of our study suggest that 6MWD and FSS might be considered as potential meaningful outcome measures in future clinical trials. Furthermore, neurorehabilitation interventions aimed at improving balance and walking performance, fatigue management, and specific pain relief therapy should be considered to ameliorate participation in social life and HR-QoL in anti-MAG neuropathy patients.
尽管抗髓鞘相关糖蛋白（MAG）抗体神经病被报道为一种进展缓慢的疾病，但它可导致显著的残疾和健康相关生活质量（HR-QoL）以及社会参与受损。本横断面研究的目的是根据国际功能、残疾和健康分类（ICF）评估 67 例抗 MAG 神经病患者的功能和 HR-QoL 决定因素。评估包括：医学研究委员会（MRC）总和评分、感觉模态总和评分（SMS）、伯格平衡量表（BBS）、疲劳严重程度量表（FSS）、疼痛视觉模拟量表（VAS）、9 孔钉测试（9-HPT）、6 分钟步行距离（6MWD）、参与和自主影响（IPA）以及简短形式 36 健康状况量表（SF-36）HR-QoL 测量的身体成分评分（PCS）和心理成分评分（MCS）。在回归模型中，6MWD 是 PCS 的最可靠预测因子，解释了其 52%的变异，而 6MWD 的最强决定因素是 BBS 和 FSS，解释了其 41%的变异。一致地，VAS 和 BBS 是 PCS 的良好预测因子，共同解释了其 54%的变异。FSS 是 MCS 的最可靠决定因素，解释了其 25%的变异。SMS 和 MRC 不是 QoL 的决定因素。我们的研究结果表明，6MWD 和 FSS 可能被认为是未来临床试验中潜在的有意义的结局指标。此外，应考虑神经康复干预措施，旨在改善平衡和步行表现、疲劳管理和特定的疼痛缓解治疗，以改善抗 MAG 神经病患者的社会生活参与度和 HR-QoL。
