儿童伴胼胝体压部可逆性病灶的临床症状轻微的脑炎/脑病的特点

Features of clinically mild encephalitis/encephalopathy with a reversible splenial lesion in children

目的探讨儿童伴有胼胝体压部可逆性病灶的临床症状轻微的脑炎/脑病(MERS)的特点。方法回顾性分析1例儿童MERS病例的临床、头颅磁共振(MRI)特点及随访结果,并检索2004年1月至2015年5月国内外期刊数据库收录的论文,收集59例儿童MERS患者的临床资料,并进行分析。结果本例MERS的前驱症状为发热、咳嗽,病程第2天出现丛集性全面性惊厥发作,头颅MRI表现为胼胝体压部、膝部和体部病灶,脑电图、脑脊液和血液学检查未见异常,共住院9 d,4周后复查MRI完全恢复,随访6个月,无神经系统后遗症。59例MERS患儿日本籍占56例,前驱疾病以呼吸道和消化道病毒感染多见,神经系统症状以意识障碍(31例)、精神异常(25例)和惊厥(24例)最多见;头颅MRI表现为孤立胼胝体压部可逆性病变(33例),或伴胼胝体膝部、皮层下白质、侧脑室周围、半卵圆中心等部位的广泛损害(26例),25例患儿伴有低钠血症,56例行脑脊液检查,其中细胞数升高者17例、蛋白升高3例、糖浓度均正常,脑电图以非特异性弥漫性慢波为主要表现。本病预后良好,临床症状及影像多可完全恢复正常。结论儿童MERS表现为在急性炎症疾病过程中出现轻微脑炎/脑病症状,头颅MRI表现为单独胼胝体压部或联合其他部位脑白质可逆性病变,可伴有低钠血症,本病预后良好。

Objective To investigate the characteristics of clinically mild encephalitis/encephalopathy with a reversible splenial lesion（MERS） in children. Methods The clinical, cerebral magnetic resonance imaging and follow-up results of a pediatric patient with the diagnosis of MERS were retrospectively analyzed, and the data of 59 MERS pediatric patients from January 2004 to May 2015 were summarized. Results The precursor symptoms of the patient before the onset cluster convulsions were fever and cough. Brain MRI indicated lesions in the splenium, genus and body of corpus callosum, these lesions recovered completely within 4 weeks, electroencephalogram（EEG） and cerebrospinal fluid（CSF） test were normal. 94.9% of the 59 children diagnosed with MERS were Japanese, and associated with respiratory and gastrointestinal infectious diseases by virus. Symptoms of nervous system included consciousness disturbance（52.5%）, delirium（42.4%） and convulsions（40.7%）. Cerebral MRI indicated solitary lesion in splenium（55.9%） or combined with genu of corpus callosum, subcortical white matters and centrum semiovate（44.1%）. Hyponatremia was found in 51% of MERS children. Examination of the CSF showed pleocytosis（30.4%）, increased protein concentration（5.4%） and normal glucose concentration. EEG showed nonspecific diffused slow wave in 60% of patients. Conclusion As a new type of clinico-radiological entity, MERS characterized by transient splenial lesions with high-signal intensity on diffusion-weighted MRI, a mild clinical course, and a good outcome.