Child Neuropsychiatry Unit, G. Gaslini Institute, Largo G. Gaslini 5, 16147, Genoa, Italy,
Neuroradiology. 2013 Oct;55(10):1233-9. doi: 10.1007/s00234-013-1240-z. Epub 2013 Jul 27.
This study aims to report on serial magnetic resonance imaging (MRI) studies and clinical features in a cohort of children with chronic inflammatory demyelinating polyneuropathy (CIDP).
Clinical, neuroradiological, and statistical investigations performed on nine children with CIDP were retrospectively reviewed. Pathological nerve root enhancement was categorized according to severity, extension, and morphology. A MRI score was thus obtained, and correlations with the clinical picture and disease course were explored.
Intrathecal nerve root enhancement (NRE) of varying degrees was seen in a high percentage of patients. There was no significant correlation between the total MRI score at the first MRI study and either severity or course of the disease. However, we found a significant difference (p = 0.002) in NRE of patients with improving CIDP with respect to those with stable or progressing disease at the time of follow-up MRI.
Contrast-enhanced MRI plays a pivotal role in children with CIDP, both for the initial diagnosis as well as a biomarker of clinical evolution, and should be performed in all children with suspected CIDP both at initial presentation and during follow-up. Further multicenter studies on larger cohorts are awaited to determine the ideal timing for follow-up MRI.
本研究旨在报告一组慢性炎症性脱髓鞘性多发性神经病（CIDP）患儿的系列磁共振成像（MRI）研究和临床特征。
回顾性分析了 9 例 CIDP 患儿的临床、神经影像学和统计学资料。根据严重程度、范围和形态对神经根病理性强化进行分类。因此获得了 MRI 评分，并探讨了其与临床表现和病程的相关性。
在大多数患者中观察到不同程度的鞘内神经根强化（NRE）。首次 MRI 研究的总 MRI 评分与疾病的严重程度或病程之间无显著相关性。然而，我们发现随访 MRI 时，CIDP 改善的患者的 NRE 与稳定或进展的患者有显著差异（p=0.002）。
增强 MRI 在儿童 CIDP 中具有重要作用，不仅对初始诊断具有重要意义，而且对临床演变的生物标志物也具有重要意义，对于疑似 CIDP 的所有儿童，无论是在初始表现还是在随访期间，均应进行增强 MRI。需要进行更多的多中心大样本研究，以确定随访 MRI 的最佳时机。
