头颅MRI以脑膜增厚伴强化表现的儿童自身免疫性脑炎  

Pediatric autoimmune encephalitis with brain MRI showing meningeal thickening and enhancement

作　　者：侯若琳[1] 吴静[1] 李玲[1] HOU Ruolin;WU Jing;LI Ling(Department of Pediatric Neurology,Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine,Shanghai 200092,China)

机构地区：[1]上海交通大学医学院附属新华医院儿神经内科,上海200092

出　　处：《临床儿科杂志》2023年第9期674-679,691,共7页Journal of Clinical Pediatrics

基　　金：国家自然科学基金项目(No.82071350,81873760)。

摘　　要：目的探讨儿童自身免疫性脑炎(AE)伴脑膜增厚强化的临床特点。方法回顾性收集并分析2019年12月-2022年2月诊断为AE,且头颅磁共振成像(MRI)表现为脑膜增厚伴强化患儿的临床资料及随访预后。结果2例患儿均为男性,发病年龄分别为5岁、12岁,均以癫痫持续状态起病,伴意识障碍。例1在发病前有感染症状,病程中反复抽搐,并逐渐出现语言障碍及精神行为异常,脑脊液细胞数轻度升高,蛋白定量(691.5 mg/L)、免疫球蛋白(Ig G68.9 mg/L,IgA 5.32 mg/L,IgM 3.72 mg/L)明显升高,血清及脑脊液中AE相关抗体均阴性。例2伴中枢性呼吸衰竭,其脑脊液细胞数轻度升高,蛋白定量(617.6 mg/L)及IgA(3.17 mg/L)升高,血清抗AMPAR1抗体IgG阳性。2例患儿血清及脑脊液病原学二代测序均阴性;头颅MRI均提示脑膜增厚伴强化;脑电图均提示背景慢波,其中例1伴痫样放电。2例患儿诊断为AE,且其影像学表现符合肥厚性硬脑膜炎特征。经免疫治疗及抗癫痫药物治疗后,均预后良好。结论对头颅MRI表现为脑膜增厚伴强化的患儿,可及时完善AE相关抗体检测,早期诊断、及时治疗及提高预后具有重要意义。Objective To investigate the clinical characteristics of autoimmune encephalitis(AE)in children with meningeal thickening and enhancement.Methods The clinical data of children diagnosed with AE and their brain magnetic resonance imaging(MRI)showing meningeal thickening with enhancement from December 2019 to February 2022 were retrospectively collected and analyzed,and the prognosis was followed up.Results A total of two boys were enrolled with onset age of 5 and 12 years old,respectively.The initial symptom of both patients was status epilepticus and disturbance of consciousness.Of them,case 1 had preceding infection,and still experienced repeated seizures during hospitalization,accompanied by gradual speech dysfunction and psycho-behavioral abnormality.The cerebrospinal fluid(CSF)results of case 1 indicated slightly leukocytosis,elevated protein(691.5 mg/L)and significantly increased immunoglobulins(IgG 68.9 mg/L,IgA 5.32 mg/L and IgM 3.72 mg/L).The antibodies associated with AE was negative in either serum or CSF.Case 2 was accompanied by central respiratory failure.The CSF results indicated slightly leukocytosis,elevated protein(617.6 mg/L)and IgA(3.17 mg/L),and positive anti-AMPAR 1 antibody in serum.The serum and CSF next generation sequencing of pathogen was negative in both patients.Brain MRI of both patients showed meningeal thickening and enhancement.The electroencephalogram of the two patients showed background slow wave,and case 1 was accompanied by epileptic discharge.Two cases were diagnosed with AE.Simultaneously,the MRI findings of both was consistent with the feature of hypertrophic pachymeningitis(HP).Both of them obtained good prognosis after immunotherapy and anti-epileptic drug treatment.Conclusions For the children with meningeal thickening and enhancement on head MRI,the detection of AE-related antibodies can be completed in time.Early diagnosis and timely treatment are of great significance for improving prognosis.

关 键 词：自身免疫性脑炎 头颅磁共振成像 脑膜增厚伴强化 临床特点 儿童 

分 类 号：R742.9[医药卫生—神经病学与精神病学] R445.2[医药卫生—临床医学]