Neuromuscular and Rare Disease Center, Department of Neuroscience, Mental Health and Sensory Organs (NESMOS), Faculty of Medicine and Psychology, SAPIENZA University of Rome, Sant'Andrea Hospital, Via di Grottarossa 1035-1039, 00189, Rome, Italy.
Department of Neurology, E. Agnelli Hospital, Pinerolo, Turin, Italy.
J Neurol. 2021 Aug;268(8):3011-3019. doi: 10.1007/s00415-021-10485-x. Epub 2021 Feb 27.
To evaluate, in a prospective study, high-resolution ultrasound (HRUS) changes of nerve segments in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and their relationships with clinical and electrodiagnostic (EDX) characteristics.
Twenty-three consecutive patients with CIDP were included in a 3-year follow-up (FU) study. Each patient underwent neurologic examination, EDX and HRUS study. HRUS was performed on median, ulnar and peroneal nerves, yielding a total of 319 scanned nerve segments. INCAT and MRC-sum scores, motor nerve conduction velocity (NCV), compound muscle action potential (cMAP) amplitude, and nerve cross-sectional area (NCSA) were collected at baseline and at FU end, and were used for statistical analysis. Twenty-two healthy individuals, matched to patients for age and BMI, served as controls.
NCSA was higher in patients than in controls (p < 0.0001) and showed significant direct correlation with disease severity, and inverse correlation with NCV and cMAP amplitude, both at baseline and at FU end. Disease duration, clinical scores and EDX were predictors of NCSA enlargement at both time points. During FU, NCSA increased in 51% of nerve segments (p = 0.006), in correlation with INCAT increase and with NCV and cMAP reduction. Considering EDX changes in subgroups that reflect the different types of nerve damage, NCSA significantly increased in those nerve segments that from normal EDX switched to prevalent myelinopathic EDX characteristics.
Peripheral nerve size tends to increase over time in patients with CIDP, in correlation with clinical and EDX changes, in particular in those nerve segments that undergo a predominantly demyelinating damage.
前瞻性研究评估慢性炎症性脱髓鞘性多发性神经病（CIDP）中神经节段的高分辨率超声（HRUS）变化及其与临床和电诊断（EDX）特征的关系。
23 例连续 CIDP 患者纳入 3 年随访（FU）研究。每位患者均接受神经病学检查、EDX 和 HRUS 研究。对正中神经、尺神经和腓总神经进行 HRUS，共扫描 319 个神经节段。在基线和 FU 结束时收集 INCAT 和 MRC 总和评分、运动神经传导速度（NCV）、复合肌肉动作电位（cMAP）幅度和神经横截面积（NCSA），并进行统计学分析。22 名年龄和 BMI 与患者匹配的健康个体作为对照。
NCSA 在患者中高于对照组（p<0.0001），且与疾病严重程度呈显著直接相关，与 NCV 和 cMAP 幅度呈显著负相关，在基线和 FU 结束时均如此。疾病持续时间、临床评分和 EDX 是两个时间点 NCSA 增大的预测因素。在 FU 期间，51%的神经节段的 NCSA 增加（p=0.006），与 INCAT 增加以及 NCV 和 cMAP 降低相关。考虑到反映不同类型神经损伤的 EDX 变化，在从正常 EDX 转变为主要脱髓鞘 EDX 特征的神经节段中，NCSA 显著增加。
CIDP 患者的周围神经大小随时间推移呈增加趋势，与临床和 EDX 变化相关，特别是在那些发生主要脱髓鞘损伤的神经节段中。
