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; 陆珺; 赵重波

doi:10.24920/003658

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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

中国医学科学杂志（英文版） 2020年35卷第4期页码：387-390

Case Report | Updated：2024-04-10

- 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
- Affiliations：
  
  1. 1Department of Neurology, Changshu No.2 Hospital, Yangzhou University, Changshu, Jiangsu 215500, China
  2. 2Department of Neurology, Huashan Hospital, Fudan University, Shanghai 200040, China
- Author bio：
  
  * E-mail: lujun_huashan@163.com.
- Funds：
- DOI：10.24920/003658
  中图分类号：
- 收稿日期：2019-09-12，
  
  录用日期：2020-2-24，
  
  网络出版日期：2020-12-31，
  
  纸质出版日期：2020-12-31
- Accepted：
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高善玉, 陆珺, 赵重波. 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[J]. 中国医学科学杂志（英文版）, 2020,35(4):387-390.

Gao Shanyu, Lu Jun, Zhao Chongbo. Stiff-Person Syndrome Associated with Anti-Glutamic Acid Decarboxylase Autoimmune Encephalitis in a Young Woman: A Case Report[J]. Chinese medical sciences journal, 2020, 35(4): 387-390.
高善玉, 陆珺, 赵重波. 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[J]. 中国医学科学杂志（英文版）, 2020,35(4):387-390. DOI： 10.24920/003658.

Gao Shanyu, Lu Jun, Zhao Chongbo. Stiff-Person Syndrome Associated with Anti-Glutamic Acid Decarboxylase Autoimmune Encephalitis in a Young Woman: A Case Report[J]. Chinese medical sciences journal, 2020, 35(4): 387-390. DOI： 10.24920/003658.

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。

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.

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Keywords

references

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