早发型多发性硬化患儿临床特点分析  

Clinical characteristics analysis of early-onset multiple sclerosis in children

作　　者：马永德[1] 王君霞[1] 

机构地区：[1]兰州军区兰州总医院儿科,730050

出　　处：《中国医药》2016年第9期1383-1387,共5页China Medicine

摘　　要：目的总结早发型多发性硬化（EOMS）的临床诊治经验。方法回顾分析2000年5月至2015年5月兰州军区兰州总医院儿科23例EOMS患儿的一般资料、临床特征、辅助检查结果、治疗及预后情况。结果23例患儿中，男11例、女12例；起病年龄为2岁2个月～16岁，平均（7．6±2．8）岁，起病年龄〈10岁14例（60．9％），≥10岁9例（39．1％）；〈10岁患儿中男8例、女6例，男：女为1．3：1，≥10岁患儿中男3例、女6例，男：女为1：2。23例中有诱发因素者5例（21．7％）。急性起病16例（69．6％）、亚急性起病6例（26．1％）、慢性起病1例（4．3％）。首发症状以视力障碍为主，占82．6％（19／23）；其次为捕搐，占52．2％（12／23）。病变侵犯视神经最多，占86．9％（20／23）。合并癫痫9例（39．1％）。21例入院时行脑脊液检查患儿中，白细胞和／或蛋白升高者13例（61．9％），IgG指数〉0．7者8例（38．1％），IgG24h合成率升高者11例（52．4％），寡克隆带阳性者13例（61．9％），免疫指标总阳性者16例（76．2％）。21例行脑电图检查患儿中17例异常（81．0％）。23例患儿头部MRI检查均呈异常，病变部位以额叶、顶叶、颞叶皮质下自质为主[78．3％（18／23）]。行脊髓MRI和视神经MRI检查者分别为14和7例，均呈异常。病灶均为多发。所有患儿急性期均给予糖皮质激素治疗。随访4个月～15年，所有患儿均有复发；19例长期随访患儿中3例死亡，病死率15．8％。结论EONS起病年龄多〈10岁，多无诱发因素，多急性发病，病变多侵犯视神经，MRI检查诊断MS非常重要，该病复发率和病死率高。Objective To investigate the clinical characteristics and follow-up analysis of early onset of multiple sclerosis （EOMS） and review the relative literatures. Methods Clinical data of 23 patients with EOMS, including general data, clinical characteristics, accessory examination, treatment and prognosis were retrospectively analyzed. Results In 23 children, 1 l were males and 12 were females; age of onset was （7.6 ± 2.8） years old （2 years and 2 months-16 years old） , with 14 cases （60. 9% ） 〈 10 years, 9 cases （39. 1% ） ≥ 10 years; in children 〈 10 years old, the ratio of male ： female was 1. 3 ： 1 （8 males and 6 females） , in children i〉 10 years old, the ratio of male ： female was 1 ： 2 （3 males and 6 females）. There were predisposing factors in 5 cases （21.7%）. Sixteen cases （69.6%） were acute onset, 6 cases were subacute （26. 1% ） and 1 case （4. 4% ） was chronic onset. The first symptom was mainly visual impairment [ 82.6% （ 19/23 ）] , followed by seizures [52. 2% （12/23） ]. The optic nerve was commonly involved [ 86. 9% （20/23） ]. There were 9 cases being complicated with epilepsy 9 cases （ 39.1% ）. Twenty one cases underwent examination of cerebrospinal fluid （CSF） , which showed increased white blood cell count and （or） protein in 13 cases （61.9%） , IgG index 〉 0. 7 in 8 cases （38. 1% ）, increased IgG 24 h synthesis rate in 11 cases （52.4%）, positive oligoelonal in 13 cases （61.9%）, and the total positive rate of immune parameters was 76. 2% （ 16/21 ）. Twenty one cases underwent electroencephalograph, among them 17 was abnormal. All the patients underwent head MRI and were all abnormal; the lesions ~e^e mainly situated at subcortical white matter of frontal, parietal and temporal lobe [ 78.3% （ 18/23 ） ] ;14 patients underwent spinal MRI and 7 eases underwent optic nerve MRI, all were abnormal. Gluocorticoid therapy was given in acute stage in all patients. All the patients had recurrence in follow

关 键 词：早发型 多发性硬化 磁共振成像 治疗 随访 

分 类 号：R744.5[医药卫生—神经病学与精神病学]