目的 探讨抗谷氨酸脱羧酶65(GAD65)抗体相关脑炎患者的临床特征及治疗方案.方法 收集2020年11月—2024年1月广西区内儿科或神经内科就诊的14例抗GAD65抗体相关脑炎的患者的病例资料,分析其临床特点.结果 14例抗GAD65抗体相关脑炎患者中,男性5例,女性9例.就诊时年龄1.5～71岁,平均(33.80±19.83)岁.前驱症状表现为发热、头痛、腹泻、全身乏力、咽痛等.14例均行脑脊液和血液抗GAD65抗体检测,其中11例血清抗GAD65抗体阳性,抗体滴度1∶10～1∶300,9例脑脊液抗GAD65抗体阳性,抗体滴度1∶10～1∶100,2例IgG指数>0.7,6例24 h IgG合成率>10 mg/24 h,1例脑脊液白蛋白指数QAlb>7×10-3.3例患者MRI可见额顶颞叶病灶,为T2WI、T2FLAIR序列斑片状高信号影,部分出现强化.7例患者脑电图表现为尖波、棘慢波.10例患者入院均予激素冲击治疗,1例同时予静脉用免疫球蛋白(IVIg)治疗.1例予利妥昔单抗治疗,其余3例未予激素及免疫调节治疗,5例患者症状在1个月内有改善.结论 抗GAD65抗体相关脑炎患者临床表现多样,部分患者对糖皮质激素治疗有反应.
Clinical features of 14 patients with anti-glutamic acid decarboxylase 65 encephalitis
Objective To investigate the clinical characteristics and treatments of anti-glutamic acid decarboxylase 65(anti-GAD65)encephalitis.Methods The clinical data of 14 patients with anti-GAD65 encephalitis who were hospital-ized in the department of pediatrics or neurology in Guangxi Zhuang Autonomous Region from November 2020 to January 2024 were retrospectively analyzed for their clinical features.Results Of the 14 patients,5 were males and 9 were fe-males.The mean age of them was(33.80±19.83)years,and the mean age of onset was 1.5-71 years.The prodromal symptoms included fever,headache,diarrhea,fatigue,and sore throat.Cerebrospinal fluid and blood GAD65 antibody tests were performed in all these cases.Eleven patients were positive for serum GAD65 antibody,with titers ranging from 1∶10-1∶300;9 patients were positive for cerebrospinal fluid GAD65 antibody,with titers ranging from 1∶10-1∶100;the se-rum IgG index was>0.7 in 2 cases;the 24 h IgG synthesis rate was>10 mg/24 h in 6 cases;the cerebrospinal fluid albumin quotient was>7×10-3 in 1 case.Three patients had lesions in the frontal,parietal,and temporal lobes on MRI,showing high-signal patches on T2WI and T2 FLAIR sequences,some of which showed enhancement.Seven patients showed sharp waves and spike-and-slow waves on electroencephalography.Ten patients were treated with hormone pulse therapy,of whom 1 patient also received intravenous immunoglobulin treatment;one case was given rituximab;three cases were not treated with hormones or immunomodulators.Five patients had improvements within one month.Conclusion Patients with anti-GAD65 encephalitis can have heterogeneous clinical features,with certain response to glucocorticoid treatment in some cases.
万方数据
维普
