Chinese Medical Sciences Journal ›› 2020, Vol. 35 ›› Issue (4): 387-390.doi: 10.24920/003658

• • 上一篇    

一例合并抗谷氨酸脱羧酶相关脑炎的僵人综合征的年轻女性

高善玉1,陆珺2,*(),赵重波2   

  1. 1扬州大学第五临床医学院常熟市第二人民医院神经内科,江苏 常熟 215500,中国
    2复旦大学附属华山医院神经内科,上海 200040,中国
  • 收稿日期:2019-09-12 接受日期:2020-02-24 出版日期:2020-12-31 发布日期:2021-01-08
  • 通讯作者: 陆珺 E-mail:lujun_huashan@163.com

Stiff-Person Syndrome Associated with Anti-Glutamic Acid Decarboxylase Autoimmune Encephalitis in a Young Woman: A Case Report

Shanyu Gao1,Jun Lu2,*(),Chongbo Zhao2   

  1. 1Department of Neurology, Changshu No.2 Hospital, Yangzhou University, Changshu, Jiangsu 215500, China
    2Department of Neurology, Huashan Hospital, Fudan University, Shanghai 200040, China
  • Received:2019-09-12 Accepted:2020-02-24 Online:2020-12-31 Published:2021-01-08
  • Contact: Jun Lu E-mail:lujun_huashan@163.com

摘要:

本例报道是一名34岁女性僵人综合征(stiff-person syndrome, SPS)患者。该患者20个月前曾因短时记忆障碍在当地医院就诊,查脑脊液抗谷氨酸脱羧酶(glutamic acid decarboxylase, GAD)抗体阳性而诊断为抗GAD相关脑炎。给予免疫球蛋白及甲强龙冲击治疗后,患者症状没有明显改善。2个月前患者出现步态不稳,双下肢僵硬伴疼痛来我院就诊,诊断为合并抗GAD相关脑炎的僵人综合征。给予高剂量γ-氨基丁酸受体激动剂及血浆置换治疗后,患者的临床症状显著改善。合并抗GAD相关脑炎的僵人综合征临床极为罕见。γ-氨基丁酸受体激动剂及适当的免疫治疗可以改善边缘性脑炎合并僵人综合征患者的神经功能。

关键词: 僵人综合征, 抗谷氨酸脱羧酶抗体, 自身免疫性脑炎

Abstract:

A 34-year-old female with stiff-person syndrome (SPS) is reported in this paper. She experienced short-term memory impairment and was diagnosed with anti-glutamic acid decarboxylase (GAD) autoimmune encephalitis (AE) at the local hospital. However, after the treatment with intravenous immunoglobulin and high-dose glucocorticoids, her symptoms unchanged. Two months later, she was admitted to our hospital due to an unstable gait and persistent leg stiffness, at which point she was diagnosed as anti-GAD AE concomitant with SPS. Her clinical symptoms improved with an increased dose of γ-aminobutyric acid (GABA)-enhancing drug and plasma exchange. Anti-GAD antibody-associated AE combined with SPS is extremely rare. Treatment with GABA-enhancing drugs and appropriate immunotherapy can improve the neurological function of patients suffering from the combination of SPS and limbic encephalitis.

Key words: stiff-person syndrome, anti-glutamic acid decarboxylase, autoimmune encephalitis

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