摘要
目的对髓鞘少突胶质细胞糖蛋白抗体相关疾病(myelin-oligodendrocyte glycoprotein-IgG associated disorders,MOGAD)的临床及影像特征进行分析。方法回顾性分析2019年2月至2022年7月在我院确诊的18例MOGAD患者临床资料。结果18例患者,男、女各9例,年龄14~68岁(中位数37岁)。最常见临床症状为认知功能下降和肢体无力,各11例(61.1%),其次是头痛和癫痫发作各8例(44.4%),感觉异常6例(33.3%),视神经炎及失语、构音障碍各5例(27.8%),发热4例(22.2%)等。腰穿检查5例(27.8%)脑脊液压力升高,11例(61.1%)脑脊液白细胞升高,6例(33.3%)脑脊液蛋白升高。影像上,累及大脑皮质9例(50.0%),其次为累及脊髓7例(38.9%)、脑干4例(22.2%)。急性期治疗,9例应用大剂量激素冲击,8例应用大剂量激素联合免疫球蛋白,1例仅用免疫球蛋白。3例患者在复发时加用吗替麦考酚酯口服。11例(61.1%)为首次发病,7例(38.9%)存在复发,复发时间2个月至7年(中位数7个月)。结论MOGAD影像上常累及大脑皮质、脊髓、脑干,并出现相应受累部位的神经系统症状,脑脊液可见白细胞、蛋白升高。急性期首选大剂量激素冲击治疗,约38.9%的患者存在复发,慢性期是否长期应用免疫抑制剂,需要根据患者病情综合判断。
Objective To summarize the clinical and imaging manifestations of myelin oligodendrocyte glycoprotein-IgG associated disorders(MOGAD).Methods From February 2019 to July 2022,18 patients with MOGAD,who were included in our hospital were retrospectively analyzed.Results Eighteen patients including 9 males and 9 females were recruited,the patient's age were from 14 to 68 years old(median 37 years old).The most common symptoms were cognitive decline and extremity weakness,each in 11 cases(61.1%).Patients also experienced headaches and epilepsy each in 8 cases(44.4%),paresthesia in 6 cases(33.3%),optic neuritis,aphasia or dysarthria each in 5 cases(27.8%),fever in 4 cases(22.2%).Cerebrospinal fluid showed elevated cranial pressure in 5 cases(27.8%),11 cases(61.1%)and 6 cases(33.3%)with elevated leukocyte and protein levels.Brain MRI revealed cortex lesions in 9 cases(50.0%),myelitis in 7 cases(38.9%)and the brainstem lesions in 4 cases(22.2%).9 patients were treated with high dose steroids in the acute phase,8 patients received steroids combing with immunoglobulins,1 patient was only treated with immunoglobulins.Only 3 patients received oral stilling during the remission period.11 cases(61.1%)had first attacked and 7 cases(38.9%)had a recurrence,the relapse occurred from 2 months to 7 years(median 7 months).Conclusion The image features of MOGAD often involved the cerebral cortex,spinal cord and brainstem,with corresponding neurological symptoms.Cerebrospinal fluid analysis revealed pleocytosis and elevated proteins.High dose steroids were preferred in the acute phase of MOGAD.38.9%patients experienced recurrence.When choosing a long-term immunosuppressive therapy in the chronic period,the patient's comprehensive condition should be considered.
作者
杨丹
王向波
闫鶴立
马红梅
刘晴
YANG Dan;WANG Xiangbo;YAN Heli;MA Hongmei;LIU Qing(Department of Neurology Xuanwu Hospital,Capital Medical University,Beijing,China)
出处
《中国神经精神疾病杂志》
CAS
CSCD
北大核心
2023年第6期331-339,共9页
Chinese Journal of Nervous and Mental Diseases
关键词
脱髓鞘疾病
髓鞘少突胶质细胞糖蛋白抗体
皮质脑炎
脊髓炎
磁共振成像
视神经炎
Demyelinating disease
Myelin oligodendrocyte glycoprotein antibody
Cortical encephalitis
Myelitis
Magnetic resonance imaging Optic neuritis
