抗MAG神经病变患者的运动单位数量指数（MUNIX）
Motor unit number index (MUNIX) in patients with anti-MAG neuropathy.
作者信息
Fatehi Farzad, Delmont Emilien, Grapperon Aude-Marie, Salort-Campana Emmanuelle, Sévy Amandine, Verschueren Annie, Boucraut José, Attarian Shahram
机构信息
Referral Centre for ALS and Neuromuscular Diseases, La Timone University Hospital, Aix-Marseille University, France; Iranian Centre of Neurological Research, Shariati Hospital, Tehran University of Medical Sciences, Iran.
Referral Centre for ALS and Neuromuscular Diseases, La Timone University Hospital, Aix-Marseille University, France; Aix-Marseille University, CNR2M, CNRS UMR 7286, Medicine Faculty, Marseille, France.
出版信息
Clin Neurophysiol. 2017 Jul;128(7):1264-1269. doi: 10.1016/j.clinph.2017.04.022. Epub 2017 May 9.
OBJECTIVE
To investigate the relationship between Motor Unit Number Index (MUNIX) and functional scales in patients with anti-Myelin Associated Glycoprotein (MAG) neuropathy and to know if MUNIX is modify after rituximab (RTX) therapy.
METHODS
17 patients were enrolled, of whom 6 were prospectively evaluated during one year after RTX treatment. MUNIX technique was assessed in abductor digiti mini (ADM), abductor pollicis brevi (APB) and tibialis anterior (TA) muscles. MUNIX sum score was calculated by adding the results of ADM, APB and TA muscles.
RESULTS
MUNIX sum score was correlated with overall neuropathy limitation scale (ONLS) (r=-0.55, p=0.02), grip strength in dominant hand (r=0.63, p=0.01) MRC testing (r=0.71, p<0.001) and CMAP sum score (r=0.71, p=0.001). Twelve months after RTX, four patients improved their disability measured on the ONLS score, five patients had improved MUNIX sum score with a median increase of 37% compared to initial evaluation.
CONCLUSIONS
MUNIX is related to motor impairment and disability in anti-MAG neuropathy and MUNIX is modified after immunosuppressive treatment.
SIGNIFICANCE
Considering its advantages, MUNIX may be a suitable test to evaluate anti-MAG neuropathy in clinical trials.
目的
研究抗髓鞘相关糖蛋白（MAG）神经病患者的运动单位数量指数（MUNIX）与功能量表之间的关系，并了解利妥昔单抗（RTX）治疗后MUNIX是否会发生改变。
方法
纳入17例患者，其中6例在RTX治疗后1年内进行前瞻性评估。在小指展肌（ADM）、拇短展肌（APB）和胫骨前肌（TA）中评估MUNIX技术。通过将ADM、APB和TA肌肉的结果相加来计算MUNIX总分。
结果
MUNIX总分与总体神经病变限制量表（ONLS）相关（r = -0.55，p = 0.02）、优势手握力（r = 0.63，p = 0.01）、医学研究委员会（MRC）测试（r = 0.71，p < 0.001）和复合肌肉动作电位（CMAP）总分（r = 0.71，p = 0.001）。RTX治疗12个月后，4例患者的ONLS评分所测量的残疾情况有所改善，5例患者的MUNIX总分有所改善，与初始评估相比，中位数增加了37%。
结论
MUNIX与抗MAG神经病中的运动障碍和残疾相关，且免疫抑制治疗后MUNIX会发生改变。
意义
考虑到其优势，MUNIX可能是临床试验中评估抗MAG神经病的合适检测方法。
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