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作 者:刘崇 郑华城[1] 王欣[1] Liu Chong;Zheng Huacheng;Wang Xin(Department of Neurology,Children's Hospital of Hebei Province,Shijiazhuang 050031,China)
机构地区:[1]河北省儿童医院神经内科,河北省小儿癫痫与神经疾病重点实验室,石家庄050031
出 处:《脑与神经疾病杂志》2024年第11期701-705,共5页Journal of Brain and Nervous Diseases
基 金:河北省医学科学研究课题计划(20220723,20230178)。
摘 要:目的通过对2例头颅磁共振(MRI)以双侧对称性病变为首发表现的MOG抗体相关疾病的病历报道,以增加对此类疾病的认识及鉴别。方法分析2例头颅MRI以双侧对称病变为首发表现且确诊为MOG抗体相关疾病的儿童患者的临床资料,并结合文献复习。结果2例患儿头颅MRI出现双侧基底核、前额对称性病变(皮质肿胀),血尿代谢筛查及基因检测未发现与之相关的变异,CBA法检测血清MOG-IgG阳性,及时给予免疫调节治疗后临床表现及头颅MRI明显好转。结论患者头颅影像学出现双侧基底核、前额对称性Flair/T2高信号,需及时送检血清MOG-IgG,早期诊断及规范治疗。Objective To improve the differentiation and understanding of MOG antibody-associated diseases(MOGAD)by exploring two cases in which bilateral symmetric lesions on cranial magnetic resonance imaging(MRI)were the initial manifestations.Methods We analyzed the clinical data of two pediatric patients with bilateral symmetric lesions on cranial MRI as the initial manifestation and confirmed diagnosis of MOGAD,and combined with a literature review.Results Both children presented with bilateral symmetric lesions in the basal ganglia and frontal lobes on cranial MRI(swelling of cerebral cortex),no relevant metabolic disorders were detected through blood and urine metabolic screening,and genetic testing revealed no mutation,serum MOG-IgG was positive as detected by cell-based assay(CBA).Timely immunomodulatory treatment led to significant improvement in clinical manifestations and cranial MRI findings.Conclusion When bilateral symmetric hyperintense signals in the basal ganglia and frontal lobes are observed on cranial imaging,timely testing for serum MOG-IgG is necessary for early diagnosis and standardized treatment.
关 键 词:MOG抗体相关疾病 儿童 头颅磁共振 遗传代谢病
分 类 号:R741[医药卫生—神经病学与精神病学]
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