儿童抗N-甲基-D-天门冬氨酸受体脑炎的诊断与鉴别诊断

Diagnosis and differential diagnosis of anti-N-methyl-D-aspartate receptor encephalitis in children

摘要:

自身免疫性脑炎相对少见，且临床表现多种多样，很难在疾病早期给予确诊。因此，儿科临床医生应该熟悉自身免疫性脑炎的临床症状、体征、辅助检查特点、影像学改变、免疫学特征以及鉴别诊断要点。为正确诊断抗N-甲基-D-天门冬氨酸受体（NMDAR）脑炎，患儿应该进行包括寡克隆带检测的脑脊液检查、头颅磁共振扫描以及普通脑电图和/ 或24 h 视频脑电图检查。对于早期出现精神症状和癫癎发作的患儿尤其注意进行血液和脑脊液中抗NMDAR 抗体测定。脑电图出现“δ 刷”时对该病临床诊断有帮助。脑脊液NMDAR 抗体阳性是诊断该病的金标准。抗NMDAR 脑炎的鉴别诊断非常宽泛，儿童患者应该主要与单纯疱疹病毒性脑炎、其他自身免疫性脑炎及精神病相鉴别。

Abstract:

Autoimmune encephalitis is rare and has various clinical manifestations, which may hamper the correct diagnosis. Therefore, the pediatrician should be familiar with the clinical symptoms, signs, laboratory features, neuroimaging changes, immunological characteristics, and differential diagnosis of this disease. In order to correctly diagnose anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, cerebrospinal fluid (CSF) examinations including detection of oligoclonal bands, brain MRI scanning, and routine EEG and/or 24 hours video EEG should be performed in children. For highly suspected cases, particularly children presenting with psychiatric symptoms and epileptic seizures, examinations should be done to detect anti-NMDAR antibodies (Abs) in serum and CSF. A notable feature in children is the EEG pattern named "extreme delta brush", which may help confirm the clinical diagnosis. Anti-NMDAR Abs in CSF is the diagnostic "gold-standard" for this disease. The differential diagnosis of anti-NMDAR encephalitis is broad. In pediatric patients, the differential diagnosis should be made mainly with herpes simplex virus encephalitis, other autoimmune encephalitis, and psychosis.