Alkhawajah Nuha M, Dunnigan Samantha K, Bril Vera
Department of Medicine (Neurology), King Saud University, Riyadh, Saudi Arabia.
J Neurol. 2014 Aug;261(8):1485-91. doi: 10.1007/s00415-014-7357-0. Epub 2014 May 7.
There are varying reports on whether monoclonal gammopathy of undetermined significance-associated neuropathy (MGUSN) patients are distinguishable from those with chronic inflammatory demyelinating polyneuropathy (CIDP) and whether specific MGUSN subclasses are associated with specific clinical phenotypes.
We performed a retrospective chart review of MGUSN (n = 56) and CIDP (n = 67) patients. Data extracted included: demographics, neurological examination, and nerve conduction studies (NCS) at baseline and last visit. Clinical status was rated as 0 = worse, 1 = unchanged, 2 = stabilized after a declining course, or 3 = improved. The electrophysiology data were rated as 0 = worse, 1 = stable, or 2 = improved. Statistical analyses were performed using JMP (version 9.0.2 for Macintosh, from SAS).
Seventy percent were males, aged 68.1 ± 12.6 years with neuropathy for 9.8 ± 6.8 years and follow-up of 4.0 ± 3.2 years. CIDP patients had more severe neuropathy, and were more likely to receive treatment and to respond. The clinical neuropathy status remained unchanged in 52.8 % of the MGUSN and 24.2 % of the CIDP patients, and stabilized in 7.6 % of MGUSN and 30.3 % of CIDP patients. IgM-MGUSN patients did not differ from other immunoglobulin subclasses in response to treatment. The clinical severity and the number of abnormal NCS parameters were greater in the demyelinating MGUSN in comparison to the axonal group.
MGUSN patients have less severe neuropathy than CIDP patients, but among the MGUSN patients the severity is greater in the demyelinating and the IgM groups. MGUSN patients may do well without treatment and exposure to potential adverse effects.
关于意义未明的单克隆丙种球蛋白病相关神经病变(MGUSN)患者是否可与慢性炎症性脱髓鞘性多发性神经病(CIDP)患者相区分,以及特定的MGUSN亚类是否与特定的临床表型相关,存在不同的报道。
我们对MGUSN患者(n = 56)和CIDP患者(n = 67)进行了回顾性病历审查。提取的数据包括:人口统计学资料、神经学检查以及基线和末次随访时的神经传导研究(NCS)。临床状态评定为:0 = 恶化,1 = 未改变,2 = 在病程下降后稳定,或3 = 改善。电生理数据评定为:0 = 恶化,1 = 稳定,或2 = 改善。使用JMP(适用于Macintosh的9.0.2版本,来自SAS)进行统计分析。
70%为男性,年龄68.1±12.6岁,患有神经病变9.8±6.8年,随访4.0±3.2年。CIDP患者的神经病变更严重,更有可能接受治疗并产生反应。52.8%的MGUSN患者和24.2%的CIDP患者临床神经病变状态未改变,7.6%的MGUSN患者和30.3%的CIDP患者病情稳定。IgM - MGUSN患者在治疗反应方面与其他免疫球蛋白亚类无差异。与轴索性MGUSN相比,脱髓鞘性MGUSN的临床严重程度和异常NCS参数数量更多。
MGUSN患者的神经病变严重程度低于CIDP患者,但在MGUSN患者中,脱髓鞘性和IgM组的严重程度更高。MGUSN患者未经治疗且未接触潜在不良反应时可能情况良好。