School of Life and Health Sciences, Aston Brain Centre, Aston University, Birmingham, UK; Department of Clinical Neurophysiology, The Birmingham Women's and Children's Hospital NHS Foundation Trust, UK.
School of Life and Health Sciences, Aston Brain Centre, Aston University, Birmingham, UK; Regional Neuromuscular Service, University Hospitals Birmingham, Birmingham, UK.
Clin Neurophysiol. 2019 Oct;130(10):1743-1749. doi: 10.1016/j.clinph.2019.06.231. Epub 2019 Jul 19.
To compare motor unit number index (MUNIX) values in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and healthy controls, to assess correlations between MUNIX and clinical assessments used in CIDP and to assess short-term changes in MUNIX in CIDP following intravenous immunoglobulins (IVIg).
MUNIX sum scores were calculated from three muscles in patients and healthy controls. CIDP patients also underwent a series of clinical assessments and completed the Overall Neuropathy Limitations Scale (ONLS) and the Rasch-built Overall-Disability Scale (R-ODS). Repeat assessments were performed in CIDP patients receiving IVIg and CIDP patients not on active treatment.
MUNIX sum scores were significantly lower in CIDP patients than healthy controls (mean values 214.0 vs 516.9, respectively; p < 0.001). MUNIX sum scores correlated with clinical assessment of motor and sensory function and ONLS and R-ODS scores in CIDP patients. Significant short-term improvements were seen in MUNIX values on repeat testing following IVIg (188.3-226.4, p = 0.001), but not in CIDP patients not receiving IVIg.
MUNIX values show stronger correlation with commonly-used clinical assessments and disability scores than other routinely used electrophysiological parameters. Rapid improvements in MUNIX sum scores are seen following IVIg.
MUNIX sum score may provide an objective marker of response to IVIg.
比较慢性炎症性脱髓鞘性多发性神经病(CIDP)患者和健康对照者的运动单位数目指数(MUNIX)值,评估 MUNIX 与 CIDP 中使用的临床评估之间的相关性,并评估 CIDP 患者在接受静脉注射免疫球蛋白(IVIg)后 MUNIX 的短期变化。
从患者和健康对照者的 3 块肌肉中计算 MUNIX 总和评分。CIDP 患者还接受了一系列临床评估,并完成了总体神经功能受限量表(ONLS)和 Rasch 构建的总体残疾量表(R-ODS)。对接受 IVIg 的 CIDP 患者和未进行积极治疗的 CIDP 患者进行重复评估。
CIDP 患者的 MUNIX 总和评分明显低于健康对照组(平均值分别为 214.0 和 516.9,p<0.001)。MUNIX 总和评分与 CIDP 患者的运动和感觉功能以及 ONLS 和 R-ODS 评分的临床评估相关。在接受 IVIg 后重复测试时,MUNIX 值出现显著短期改善(188.3-226.4,p=0.001),但在未接受 IVIg 的 CIDP 患者中则没有。
MUNIX 值与常用的临床评估和残疾评分的相关性强于其他常规使用的电生理参数。在接受 IVIg 后,MUNIX 总和评分迅速改善。
MUNIX 总和评分可能为 IVIg 反应提供客观标志物。