儿童慢性炎性脱髓鞘性多发性神经病15例报告并文献复习

发布时间：2018-03-27 18:10

本文选题：慢性炎性脱髓鞘性多发性神经病　切入点：临床分析　出处：《中国循证儿科杂志》2017年03期

【摘要】：目的探讨儿童慢性炎性脱髓鞘性多发性神经病(CIDP)的临床特点和预后。方法收集2008年1月至2017年1月复旦大学附属儿科医院收治的CIDP患儿的临床资料,检索PubMed数据库1991年1月至2017年2月的儿童CIDP病例,将文献检索和本文的病例汇总,根据到达疾病高峰期时间分组,4~8周为亚急性起病组,8周为慢性起病组,比较两组的临床表现、实验室检查、治疗和预后。结果 (1)符合本文纳入和排除标准的CIDP患儿15例,男6例,女9例;1~3岁4例,~7岁4例,~15岁7例;起病年龄1岁1月至11岁9月;7例有前驱感染史;到达疾病高峰期时间为4周至13月,平均(12.9±13.6)周。15例的首发症状均为运动障碍,4例有感觉障碍;疾病高峰期改良Rankin量表(MRS)评分:3分8例,4分6例,5分1例;病程呈单向型3例,进展型5例,复发型7例;15例神经传导检查均有神经源性损害,脑脊液检查均有细胞蛋白分离现象。14例予糖皮质激素治疗,1例予静脉丙种球蛋白治疗;1例家属放弃治疗出院,其余14例住院时间7~17 d,出院时临床症状均有不同程度好转。随访中7例患儿复发。(2)共有14篇文献报道87例CIDP患儿与本文15例合并行文献分析(n=102),亚急性起病组38例,慢性起病组64例。两组性别、起病年龄和运动障碍差异无统计学意义,感觉障碍(57.1%vs 23.5%,P=0.002)和颅神经异常(25.7%vs 7.8%,P=0.023)亚急性起病组高于慢性起病组,且更易反复发作(62.2%vs 34.0%,P=0.010),慢性起病组单相发作形式多(50.8%vs 27.8%,P=0.026)。结论 CIDP亚急性起病患儿感觉障碍和颅神经异常多于慢性起病患儿,并且疾病发展过程中容易复发。儿童CIDP运动障碍常见,较少出现颅神经损害和呼吸衰竭等,脑脊液细胞蛋白分离现象多见,电生理改变明显,早期治疗则预后较好。
[Abstract]:Objective to investigate the clinical features and prognosis of chronic inflammatory demyelinating polyneuropathy (CIDP) in children. Methods the clinical data of children with CIDP were collected from January 2008 to January 2017 in the Pediatrics Hospital of Fudan University. From January 1991 to February 2017, children with CIDP were searched in PubMed database. The literature was searched and the cases collected in this paper. According to the time of reaching the peak of disease, the patients were divided into subacute onset group for 8 weeks and chronic onset group for 8 weeks. The clinical manifestations of the two groups were compared. Results 1) Fifteen children with CIDP, including 6 males and 9 females, 1 / 3 years old, 4 / 7 years old and 7 / 7 years old, who met the criteria of inclusion and exclusion of CIDP, had a history of prodromal infection from 1 year old to 11 years old, and 7 cases from 1 year old to 11 year old, 7 cases were diagnosed by laboratory examination, treatment and prognosis. The initial symptoms of the 15 cases were all dyskinesia in 4 cases with sensory disturbance, and the scores of 3 points in 8 cases, 4 cases in 4 cases and 5 points in 1 case, and the course of disease was unidirectional in 3 cases. Neurogenic damage was found in 5 cases of progressive type, 7 cases of recurrent type and 15 cases of nerve conduction. Cerebrospinal fluid (CSF) examination showed the phenomenon of cell protein separation. 14 cases were treated with glucocorticoid, 1 case was treated with intravenous immunoglobulin and 1 case was discharged from hospital. The remaining 14 cases were hospitalized for 7 days and their clinical symptoms were improved in varying degrees at the time of discharge. There were 14 articles about 87 cases of CIDP and 15 cases of this article combined with literature analysis, 38 cases in subacute onset group. There was no significant difference in sex, onset age and motor disorder between the two groups. The subacute onset group was higher than the chronic onset group (57.1 vs 23.5P0. 002) and the cranial nerve abnormality 25. 7 vs 7. 8 Pn0. 023). And it is more likely to repeat attack 62.2% vs 34.0% P0.010%, and 50.8% vs 27.8% P0.026 in chronic onset group. Conclusion the sensory disorders and cranial nerve abnormalities in children with subacute onset of CIDP are more than those in patients with chronic onset, and it is easy to recur in the course of disease development. CIDP motility disorder is common in children. There were few cranial nerve damage and respiratory failure, and the separation of cerebrospinal fluid cell proteins was more common, electrophysiological changes were obvious, and early treatment had a better prognosis.
【作者单位】：复旦大学附属儿科医院神经科;
【分类号】：R744.5

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