多灶性运动神经病以及运动型和典型慢性炎症性脱髓鞘性神经病的神经超声检查

Nerve Ultrasound of Multifocal Motor Neuropathy and Motor and Typical Chronic Inflammatory Demyelinating Neuropathy.

作者信息

Niu Jingwen, Ding Qingyun, Hu Nan, Cui Liying, Liu Mingsheng

机构信息

Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Dongcheng-qu, Beijing, China.

出版信息

Can J Neurol Sci. 2025 Jun 9:1-9. doi: 10.1017/cjn.2025.10136.

DOI:10.1017/cjn.2025.10136

PMID:40485489

Abstract

OBJECTIVE

Both multifocal motor neuropathy (MMN) and chronic inflammatory demyelinating polyneuropathy (CIDP) are chronic progressive immune-mediated peripheral neuropathies without sensory loss. We aimed to explore the different features of ultrasonographic and electrophysiological changes among MMN, motor CIDP and typical CIDP patients.

METHODS

Nerve ultrasonographic studies were performed in 19 patients with MMN, 15 patients with motor CIDP and 117 patients with typical CIDP. Cross-sectional areas (CSAs) were measured on the bilateral median and ulnar nerves and brachial plexus. Nerve conduction studies (NCSs) were performed on the median and ulnar nerves.

RESULTS

In patients with MMN and typical CIDP, the percentage enlargement in the brachial plexus (MMN 45.7%, typical CIDP 78%) was similar to that in the arm (MMN 42.9%, typical CIDP 76.8%) and forearm (MMN 42.9%, typical CIDP 79.4%). However, in patients with motor CIDP, the percentage enlargement in the brachial plexus (74.1%) was more pronounced than in the arm (65.5%) and forearm (58.6%). The CMAPerb/CMAPaxilla in MMN was significantly higher than in motor CIDP (median nerve, 0.82 ± 0.28 for MMN and 0.60 ± 0.37 for motor CIDP, = 0.017). The CSA decreased in the order of typical CIDP, motor predominant CIDP (MPred-CIDP), pure motor CIDP (PM-CIDP) and MMN. The motor nerve conduction velocity increased in the order of typical CIDP, MPred-CIDP, PM-CIDP and MMN. A total of 3/6 PM-CIDP and 3/3 MPred-CIDP patients responded to steroid treatment.

CONCLUSION

Treatment response, nerve ultrasonography and NCS in MMN, PM-CIDP, MPred-CIDP and typical CIDP constitute a spectrum.

摘要

目的

多灶性运动神经病（MMN）和慢性炎症性脱髓鞘性多发性神经病（CIDP）均为慢性进行性免疫介导的周围神经病，无感觉丧失。我们旨在探讨MMN、运动性CIDP和典型CIDP患者超声及电生理变化的不同特征。

方法

对19例MMN患者、15例运动性CIDP患者和117例典型CIDP患者进行神经超声检查。测量双侧正中神经、尺神经及臂丛神经的横截面积（CSA）。对正中神经和尺神经进行神经传导研究（NCS）。

结果

在MMN和典型CIDP患者中，臂丛神经增粗百分比（MMN为45.7%，典型CIDP为78%）与上臂（MMN为42.9%，典型CIDP为76.8%）和前臂（MMN为42.9%，典型CIDP为79.4%）相似。然而，在运动性CIDP患者中，臂丛神经增粗百分比（74.1%）比上臂（65.5%）和前臂（58.6%）更明显。MMN患者的复合肌肉动作电位波幅比值（CMAPerb/CMAPaxilla）显著高于运动性CIDP患者（正中神经，MMN为0.82±0.28，运动性CIDP为0.60±0.37，P = 0.017）。CSA按典型CIDP、运动为主型CIDP（MPred-CIDP）、纯运动型CIDP（PM-CIDP）和MMN的顺序递减。运动神经传导速度按典型CIDP、MPred-CIDP、PM-CIDP和MMN的顺序递增。共有3/6例PM-CIDP患者和3/3例MPred-CIDP患者对类固醇治疗有反应。