儿童抗N-甲基-D-天冬氨酸受体脑炎15例临床分析

发布时间：2018-02-08 13:29

本文关键词： 抗N-甲基-D-天冬氨酸受体脑炎儿童临床特征　出处：《中国实用儿科杂志》2017年07期 　论文类型：期刊论文

【摘要】：目的探讨儿童抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的临床特点。方法对2015年4月至12月复旦大学附属儿科医院神经内科收治的15例确诊为抗NMDAR脑炎患儿资料进行回顾性分析。结果 15例中男8例、女7例,年龄8个月至12岁,中位发病年龄7岁。临床症状:首发症状意识障碍伴惊厥3例(20.0%),精神行为异常4例(26.7%)。15例均有惊厥发作,睡眠障碍5例(33.3%);发生惊厥持续状态3例;意识障碍10例(66.7%),昏迷3例;肢体或颜面部不自主运动12例(80.0%);尿潴留7例(46.7%)。中枢性低通气3例(20.0%)。继发于感染后2例(13.3%)。辅助检查:均行脑脊液检查,5例(33.3%)白细胞轻度升高,2例(13.3%)蛋白明显升高;脑脊液抗NMDAR抗体均阳性;其中10例行IgG指数检查,4例(40.0%)Ig G指数升高。10例(66.7%)头颅磁共振成像(MRI)异常。脑电图均为背景活动变慢,9例(60.0%)痫样放电,未发现δ刷。仅1例(6.7%)发现卵巢占位性病变。治疗和随访:均应用静脉甲泼尼龙加丙种球蛋白免疫治疗,1例(6.7%)给予血浆置换。随访1~6个月,9例改良Rankin量表评级为0~2级。结论抗NMDAR脑炎临床表现和实验室检查缺乏特异性,初期诊断困难。脑脊液特异性抗NMDAR抗体阳性可确诊该病。儿童抗NMDAR脑炎患者肿瘤发生率低,脑电图δ刷少见。早期诊断和及时应用免疫抑制剂治疗,预后良好。
[Abstract]:Objective to investigate the anti N- methyl -D- aspartate receptor (NMDAR) encephalitis clinical features. Methods 15 cases from April 2015 to December in Paediatrics Hospital Affiliated to Fudan University from the Department of Neurology for anti NMDAR encephalitis were retrospectively analyzed. Results among the 15 cases, male 8 cases, female 7 cases, aged 8 months to 12 years in the median age was 7 years old. The clinical symptoms: symptoms of disturbance of consciousness with convulsion in 3 cases (20%), 4 cases of abnormal mental behavior (26.7%).15 cases were seizure onset, sleep disorders in 5 cases (33.3%) occurred in 3 cases; status epilepticus; 10 cases of disturbance of consciousness (66.7%), 3 cases of coma or limb; the face of involuntary movement in 12 cases (80%); 7 cases of urinary retention (46.7%). 3 cases of central hypoventilation (20%). Secondary to infection in 2 cases (13.3%). Auxiliary examination: 5 cases underwent the examination of cerebrospinal fluid, (33.3%) white blood cells increased slightly, 2 cases (13.3%) was significantly elevated CSF; anti NMDAR antibody Positive; 10 cases underwent IgG examination index, 4 cases (40% cases).10 Ig G index was significantly increased (66.7%) brain magnetic resonance imaging (MRI) abnormalities. EEG background activity was slow, 9 cases (60%) of epileptic discharge, did not find the delta brush only 1 cases (6.7%) found that ovarian lesions lesions. Treatment and follow-up: intravenous injection of methylprednisolone plus gamma globulin immune therapy, 1 cases (6.7%) received plasma exchange. The follow-up of 1~6 months, 9 cases of modified Rankin scale rating of 0~2. Conclusion anti NMDAR encephalitis clinical manifestation and laboratory examination of lack of specificity, the early diagnosis of cerebral spinal fluid. The specificity of anti NMDAR antibody positive can be diagnosed the disease in children. The incidence of tumors in patients with anti NMDAR encephalitis is low, EEG delta brush rare. Early diagnosis and timely application of immunosuppressive therapy, the prognosis is good.

【作者单位】：复旦大学附属儿科医院神经内科;扬州大学附属医院(原扬州市第一人民医院)儿科;
【分类号】：R742.9

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