Ware Tyson L, Kornberg Andrew J, Rodriguez-Casero M Victoria, Ryan Monique M
1Department of Neurology, The Royal Children's Hospital, Melbourne, Australia.
J Child Neurol. 2014 Jan;29(1):43-8. doi: 10.1177/0883073812471719. Epub 2013 Jan 29.
Chronic inflammatory demyelinating polyneuropathy is a rare condition in children. In this article, we report our experience in the management of 10 cases of childhood chronic inflammatory demyelinating polyneuropathy in a single center, in the era of contrast-enhanced magnetic resonance imaging (MRI), genetic microarray, and chronic inflammatory demyelinating polyneuropathy disease activity status. Robust neurophysiologic abnormalities were present in all cases and both MRI and lumbar puncture were useful adjuncts in diagnosis. Genetic microarray is a simple technique useful in excluding the most common hereditary demyelinating neuropathy. Intravenous immunoglobulin was an effective first-line therapy in most cases, with refractory cases responding to corticosteroids and rituximab. We found the chronic inflammatory demyelinating polyneuropathy disease activity status useful for assessing outcome at final follow-up, whereas the modified Rankin score was better for assessing peak motor disability.
慢性炎症性脱髓鞘性多发性神经病在儿童中是一种罕见疾病。在本文中，我们报告了在单中心管理10例儿童慢性炎症性脱髓鞘性多发性神经病的经验，此经验处于对比增强磁共振成像（MRI）、基因微阵列以及慢性炎症性脱髓鞘性多发性神经病疾病活动状态的时代。所有病例均存在明显的神经生理异常，MRI和腰椎穿刺在诊断中均为有用的辅助手段。基因微阵列是一项有助于排除最常见遗传性脱髓鞘性神经病的简单技术。静脉注射免疫球蛋白在大多数病例中是有效的一线治疗方法，难治性病例对皮质类固醇和利妥昔单抗有反应。我们发现慢性炎症性脱髓鞘性多发性神经病疾病活动状态有助于评估最终随访时的结果，而改良Rankin评分在评估运动功能障碍峰值方面效果更佳。
