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

Gao Shanyu; Lu Jun; Zhao Chongbo

doi:10.24920/003658

Your Location：

Home >

Browse articles >

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

Chinese Medical Sciences Journal Vol. 35, Issue 4, Pages: 387-390(2020)

Case Report | Updated：2024-04-10

- Stiff-Person Syndrome Associated with Anti-Glutamic Acid Decarboxylase Autoimmune Encephalitis in a Young Woman: A Case Report
- 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
  CLC：
- Received：12 September 2019，
  
  Accepted：2020-2-24，
  
  Published Online：31 December 2020，
  
  Published：31 December 2020
- Accepted：
Scan QR Code
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：

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.

关键词

Keywords

references

Malter MP , Helmstaedter C , Urbach H , et al . Antibodies to glutamic acid decarboxylase define a form of limbic encephalitis . Ann Neurol 2010 ; 67 : 471 - 8 . doi: 10.1002/ana.21917 10.1002/ana.21917 .

Sarva H , Deik A , Ullah A , et al . Clinical spectrum of stiff person syndrome: a review of recent reports . Tremor Other Hyperkinet Mov (N Y) 2016 ; 6 : 340 . doi: 10.7916/D85M65GD 10.7916/D85M65GD .

Chang T , Alexopoulos H , McMenamin M , et al . Neuronal surface and glutamic acid decarboxylase autoantibodies in nonparaneoplastic stiff person syndrome . JAMA Neurol 2013 ; 70 ( 90 ): 1140 - 9 . doi: 10.1001/jamaneurol.2013.3499 10.1001/jamaneurol.2013.3499 . DOI: 10.1001/jamaneurol.2013.3499 http://doi.org/10.1001/jamaneurol.2013.3499 http://archneur.jamanetwork.com/article.aspx?doi=10.1001/jamaneurol.2013.3499 http://archneur.jamanetwork.com/article.aspx?doi=10.1001/jamaneurol.2013.3499

Baumgartner A , Rauer S , Mader I , et al . Cerebral PDG-PET and MRI findings in autoimmune limbic encephalitis: correlation with autoantibody types . J Neurol 2013 ; 260 ( 11 ): 2744 - 53 . doi: 10.1007/s00415-013-7048-2 10.1007/s00415-013-7048-2 . DOI: 10.1007/s00415-013-7048-2 http://doi.org/10.1007/s00415-013-7048-2 https://www.ncbi.nlm.nih.gov/pubmed/23900756 https://www.ncbi.nlm.nih.gov/pubmed/23900756

Rangan K , Ora M , Jain N , et al . Multimodality imaging of anti-contactin-associated protein-like 2 antibody-induced nonparaneoplastic limbic encephalitis: complementary role of fluorine-18 fluorodeoxyglucose positron emissin tomography and magnetic resonance imaging . Indian J Nucl Med 2020 ; 35 : 54 - 7 . doi: 10.103/ijnm.IJNM_178_19 10.103/ijnm.IJNM_178_19 . DOI: 10.4103/ijnm.IJNM_178_19 http://doi.org/10.4103/ijnm.IJNM_178_19 https://www.ncbi.nlm.nih.gov/pubmed/31949370 https://www.ncbi.nlm.nih.gov/pubmed/31949370

Sloviter RS , Dichter MA , Rachinsky TL , et al . Basal expression and induction of glutamate decarboxylase and GABA in excitatory granule cells of the rat and monkey hippocampal dentate gyrus . J Comp Neurol 1996 ; 373 ( 4 ): 593 - 618 . doi: 10.1002/(SICI)1096-9861(19960930)373:4<593::AID-CNE8>3.0.CO 10.1002/(SICI)1096-9861(19960930)373:4<593::AID-CNE8>3.0.CO ;2-X. DOI: 10.1002/(SICI)1096-9861(19960930)373:4<593::AID-CNE8>3.0.CO;2-X http://doi.org/10.1002/(SICI)1096-9861(19960930)373:4<593::AID-CNE8>3.0.CO;2-X https://www.ncbi.nlm.nih.gov/pubmed/8889946 https://www.ncbi.nlm.nih.gov/pubmed/8889946

Huang J , Li H , Zhou R , et al . Clinical heterogeneity in patients with glutamate decarboxylase antibody . Neroimmunomodulation 2019 ; 26 ( 5 ): 234 - 8 . doi: 10.1159/000502695 10.1159/000502695 .

Martinez-Hernandez E , Ariño H , Mckeon A , et al . Clinical and immunologic investigations in patients with stiff-person spectrum disorder . JAMA Neurol 2016 ; 73 ( 6 ): 714 - 20 . doi: 10.1001/jamaneurol.2016.0133 10.1001/jamaneurol.2016.0133 . DOI: 10.1001/jamaneurol.2016.0133 http://doi.org/10.1001/jamaneurol.2016.0133 https://www.ncbi.nlm.nih.gov/pubmed/27065452 https://www.ncbi.nlm.nih.gov/pubmed/27065452

Balint B , Meinck HM . Pragmatic treatment of stiff person spectrum disorders . Mov Disod Clin Pract 2018 ; 5 ( 4 ): 394 - 401 . doi: 10.1002/mdc3.12629 10.1002/mdc3.12629 .

Spatola M , Dalmau J . Seizures and risk of epilepsy in autoimmune and other inflammatory encephalitis . Curr Opin Neurol 2017 ; 30 ( 3 ): 345 - 53 . doi: 10.1097/WCO.0000000000000449 10.1097/WCO.0000000000000449 . DOI: 10.1097/WCO.0000000000000449 http://doi.org/10.1097/WCO.0000000000000449 https://www.ncbi.nlm.nih.gov/pubmed/28234800 https://www.ncbi.nlm.nih.gov/pubmed/28234800

Dalakas MC , Rakocevic G , Dambrosia JM , et al . A double-blind, placebo-controlled study of rituximab in patients with stiff person syndrome . Ann Neurol 2017 ; 82 ( 2 ): 271 - 7 . doi: 10.1002/ana.25002 10.1002/ana.25002 . https://www.ncbi.nlm.nih.gov/pubmed/28749549 https://www.ncbi.nlm.nih.gov/pubmed/28749549

Views

468

Downloads

CSCD

Alert me when the article has been cited

Submit

Publicity Resources

No data

Related Author

No data

Related Institution

No data

⁰