自身免疫性自主神经节病3例临床分析  

Clinical analysis of three cases of autoimmune autonomic ganglionopathy

作　　者：刘海平 廖少华 毛晓薇 王珺燕 张秀天 毕晓莹 蒋建明 LIU Haiping;LIAO Shaohua;MAO Xiaowei;WANG Junyan;ZHANG Xiutian;BI Xiaoying;JIANG Jianming(Department of Neurology,Changhai Hospital,Shanghai 200433,China)

机构地区：[1]上海长海医院神经内科,200043

出　　处：《中国神经免疫学和神经病学杂志》2022年第5期371-374,共4页Chinese Journal of Neuroimmunology and Neurology

摘　　要：目的总结自身免疫性自主神经节病(autoimmune autonomic ganglionopathy,AAG)的临床特点、诊断、治疗及预后。方法回顾性收集2008年4月至2021年8月上海长海医院确诊的3例AAG患者的临床资料,对其病史、临床表现、辅助检查及治疗进行总结。结果3例患者均为青中年女性,急性起病,均有前驱感染、发热病史,所有患者存在胃肠道动力障碍、排尿障碍、全身排汗障碍,1例(1/3)患者有直立性低血压,2例(2/3)患者存在明显的瞳孔扩大,1例(1/3)患者存在脑脊液蛋白细胞分离现象。3例患者急性期经免疫球蛋白联合糖皮质激素治疗后临床症状改善。结论AAG是一种少见病,具有典型的临床特点,辅助检查无特征性,免疫球蛋白联合糖皮质激素治疗有效,多遗留不同程度后遗症。Objective To summarize the clinical characteristics,diagnosis,treatment and prognosis of autoimmune autonomic ganglionopathy(AAG).Methods The clinical data of 3 patients with AAG who were hospitalized in Changhai Hospital from April 2008 to August 2021 were collected retrospectively.Their medical history,clinical manifestations,auxiliary examination and treatment were summarized.Results All the 3 patients were young or middle-aged women with acute onset and a history of prodromal infection and fever.All the patients had gastrointestinal motility disorder,dysuria and dyshidrosis.One patient(1/3)had orthostatic hypotension.Two patients(2/3)had obvious pupil dilation.One patient(1/3)showed albuminocytologic dissociation.The clinical symptoms of 3 patients were improved after treatment with immunoglobulin combined with glucocorticoid in the acute stage.Conclusions AAG is a rare disease with typical clinical characteristics,no characteristic of adjuvant examination.Intravenous immunoglobulin combined with glucocorticoid is effective.Most patients have sequelae in different degrees.

关 键 词：自主神经系统疾病 急性泛自主神经病 自身免疫性自主神经节病 免疫球蛋白 糖皮质激素 

分 类 号：R745.7[医药卫生—神经病学与精神病学]