Galassi Giuliana, Tondelli Manuela, Ariatti Alessandra, Benuzzi Francesca, Nichelli Paolo, Valzania Franco
a Department of Biomedical, Metabolic and Neural Science , University of Modena & Reggio Emilia , Modena , Italy.
Int J Neurosci. 2017 May;127(5):439-447. doi: 10.1080/00207454.2016.1191013. Epub 2016 Jun 7.
Neuropathy associated with IgM monoclonal gammopathy (MGUS) represents distinctive clinical syndrome, characterized by male predominance, late age of onset, slow progression, predominantly sensory symptoms, deep sensory loss, ataxia, minor motor impairment. More than 50% of patients with neuropathy-associated MGUS possess antibodies against myelin-associated glycoprotein (MAG). Purpose of our study was to assess effects on disease progression of demographic, clinical and neurophysiological variables in our large cohort of patients.
Forty-three Caucasians patients were followed every eight months for median duration time of 93 months. Extremity strength was assessed with Medical Research Council (MRC) Scale, disability with overall disability status scale (ODSS), modified Rankin Scale and sensory function with Inflammatory Neuropathy Cause and Treatment (INCAT) sensory scale (ISS). Statistical analyses were conducted with parametric or non-parametric measures as appropriate. Survival analysis was used to test predictive value of clinical, demographical and neurophysiological variables. Variance analysis was conducted to explain difference on MRC between patients and groups at different time from onset.
Results showed that demyelinating pattern, older age and absence of treatment were significant risk factors for disability worsening. No other factors emerged as predictors including gender, ataxia and tremor at baseline, level of anti-MAG and IgM protein concentration in serum. Despite worsening of all outcome measures between first and last visit, quality of life (HRQol) judged by patients did not vary significantly.
Our study provides evidence that electrophysiologic pattern, age of onset and absence of treatment are strong predictor of prognosis in anti-MAG polyneuropathy.
与IgM单克隆丙种球蛋白病（MGUS）相关的神经病变代表一种独特的临床综合征，其特征为男性居多、发病年龄较晚、进展缓慢、主要为感觉症状、深部感觉丧失、共济失调、轻度运动障碍。超过50%的与神经病变相关的MGUS患者拥有抗髓鞘相关糖蛋白（MAG）抗体。我们研究的目的是评估在我们的大型患者队列中，人口统计学、临床和神经生理学变量对疾病进展的影响。
43名白种人患者每8个月随访一次，中位随访时间为93个月。采用医学研究委员会（MRC）量表评估肢体力量，采用总体残疾状态量表（ODSS）、改良Rankin量表评估残疾情况，采用炎症性神经病变病因与治疗（INCAT）感觉量表（ISS）评估感觉功能。根据情况采用参数或非参数方法进行统计分析。生存分析用于检验临床、人口统计学和神经生理学变量的预测价值。进行方差分析以解释患者和不同发病时间组之间MRC的差异。
结果显示，脱髓鞘模式、年龄较大和未接受治疗是残疾恶化的重要危险因素。没有其他因素成为预测指标，包括性别、基线时的共济失调和震颤、血清中抗MAG水平和IgM蛋白浓度。尽管首次和末次就诊之间所有结局指标都有所恶化，但患者判断的生活质量（HRQol）没有显著变化。
我们的研究提供了证据，表明电生理模式、发病年龄和未接受治疗是抗MAG多神经病预后的有力预测指标。
