Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.
Muscle Nerve. 2024 Nov;70(5):972-979. doi: 10.1002/mus.28239. Epub 2024 Aug 28.
INTRODUCTION/AIMS: Nerve enlargement has been described in autoimmune nodopathy and chronic inflammatory demyelinating polyneuropathy (CIDP). However, comparisons of the distribution of enlargement between autoimmune nodopathy and CIDP have not been well characterized. To fill this gap, we explored differences in the ultrasonographic and electrophysiological features between autoimmune nodopathy and CIDP.
Between March 2015 and June 2023, patients fulfilling diagnostic criteria for CIDP were enrolled; among them, those with positive antibodies against nodal-paranodal cell-adhesion molecules were distinguished as autoimmune nodopathy. Nerve ultrasound and nerve conduction studies (NCS) were performed.
Overall, 114 CIDP patients and 13 patients with autoimmune nodopathy were recruited. Cross-sectional areas (CSA) at all sites were larger in patients with CIDP and autoimmune nodopathy than in healthy controls. CSAs at the roots and trunks of the brachial plexus were significantly larger in patients with anti-neurofascin-155 (NF155), anti-contactin-1 (CNTN1), and anti-contactin-associated protein 1 (CASPR1) antibodies than in CIDP patients. The patients with anti-NF186 antibody did not have enlargement in the brachial plexus. NCS showed more frequent probable conduction block at Erb's point in autoimmune nodopathy than in CIDP (61.9% vs. 36.6% for median nerve, 52.4% vs. 39.5% for ulnar nerve). Markedly prolonged distal motor latencies were also present in autoimmune nodopathy.
Patients with autoimmune nodopathies had distinct distributions of peripheral nerve enlargement revealed by ultrasound, as well as distinct NCS patterns, which were different from CIDP. This suggests the potential utility of nerve ultrasound and NCS to supplement clinical characteristics for distinguishing nodopathies from CIDP.
简介/目的:自身免疫性结节病和慢性炎症性脱髓鞘性多发性神经病(CIDP)中已有神经增粗的描述。然而,自身免疫性结节病和 CIDP 之间的增粗分布比较尚未得到很好的描述。为了填补这一空白,我们探讨了自身免疫性结节病和 CIDP 之间超声和电生理特征的差异。
2015 年 3 月至 2023 年 6 月期间,纳入符合 CIDP 诊断标准的患者;其中,对节点-旁节细胞黏附分子抗体阳性的患者被区分出自自身免疫性结节病。进行神经超声和神经传导研究(NCS)。
共纳入 114 例 CIDP 患者和 13 例自身免疫性结节病患者。与健康对照组相比,CIDP 和自身免疫性结节病患者所有部位的横截面积(CSA)均更大。抗神经束蛋白 155(NF155)、抗接触蛋白 1(CNTN1)和抗接触蛋白相关蛋白 1(CASPR1)抗体的患者臂丛神经根和干的 CSA 显著更大。抗 NF186 抗体的患者臂丛神经无增粗。神经传导研究显示,自身免疫性结节病中 Erb 点更常出现可能的传导阻滞(正中神经 61.9%,尺神经 52.4%),而 CIDP 中为 36.6%和 39.5%。自身免疫性结节病还存在明显的远端运动潜伏期延长。
自身免疫性结节病患者的外周神经超声显示出明显不同的增粗分布,以及明显不同的 NCS 模式,与 CIDP 不同。这表明神经超声和 NCS 可能有助于补充临床特征,以区分结节病和 CIDP。