|
| 儿童抗NF155抗体阳性的自身免疫性郎飞结病1例报道 |
| Anti-neurofascin-155 antibody autoimmune positive nodopathy in a child |
| 投稿时间：2024-12-25 修订日期：2024-12-25 |
| DOI： |
| 中文关键词: 儿童 自身免疫性郎飞结病 抗神经束蛋白155抗体 |
| 英文关键词: children autoimmnue nodopathy anti-neurofascin-155 |
| 基金项目: |
|
| 摘要点击次数: 253 |
| 全文下载次数: 0 |
| 中文摘要: |
| 目的：探讨抗神经束蛋白（NF）155抗体阳性的自身免疫性郎飞结病（AN）患儿的临床特点及预后。方法：报道我院1例确诊为抗NF155抗体阳性的自身免疫性郎飞结病女性患儿并结合文献回顾性分析。结果：本例患儿临床特点为肢体无力，下肢重于上肢，伴感觉异常，无震颤。脑脊液提示蛋白细胞分离，肌电图示运动及感觉均受累，主要以脱髓鞘损害为主。头颅及全脊柱核磁检查未见异常。给予静脉注射丙种球蛋白和激素治疗后症状较前改善。搜索既往报道的113例抗NF155抗体阳性的自身免疫性郎飞结病患儿，临床特点为亚急性或慢性起病，远端肢体无力为主，伴有感觉异常、共济失调及震颤。部分患者经丙种球蛋白及激素治疗后症状改善不佳，可尽早使用利妥昔单抗改善预后。 |
| 英文摘要: |
| Objective:To analyze the clinical features and prognosis of anti-neurofascin-155(NF155) antibody positive autoimmnue nodopathy in children.Method：We reported a female child diagnosed with anti-NF155 antibody positive nodopathy at Wuhan Children's Hospital. Results:The clinical characteristics of this child were limb weakness with more severe in the distal and the lower extremities than in the upper extremities, paraesthesia and without tremor.Her cerebrospinal fluid showed albuminocytologic dissociation.Her electrophysiological examination showed motor and sensory nerve damage.The brain and spinal cord magentic resonance imagings were normal. Her symptoms significantly improved after using intravenous immunoglobulin (IVIG) and steroids. A total of 133 children with anti-NF155 antibody positive nodopathy were reported in the past and the clinical characteristic were subacute or chronic onset, manily distal limb weakness, paraesthesia, ataxia and tremor. Some patients have poor improvement after IVIG and steroids,and rituximab can be used as soon as possible. |
|
View Fulltext
查看/发表评论 下载PDF阅读器 |
| 关闭 |