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联合免疫球蛋白和糖皮质激素治疗僵硬肢体综合征患者的疗效:病例报告及文献复习

Effectiveness of Combined Immunoglobulin and Glucocorticoid Treatments in a Patient With Stiff Limb Syndrome: Case Report and Review of the Literature.

作者信息

Huang Juan, Meng Huan-Yu, Duan Xian, Li Wen-Wen, Xu Hong-Wei, Zhou Ya-Fang, Zhou Lin

机构信息

Department of Geriatric Neurology, Xiangya Hospital, Central South University, Changsha, China.

National Clinical Research Center for Geriatric Disorders, Xiangya Hospital, Central South University, Changsha, China.

出版信息

Front Neurol. 2020 May 8;11:284. doi: 10.3389/fneur.2020.00284. eCollection 2020.

DOI:10.3389/fneur.2020.00284
PMID:32477232
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7232827/
Abstract

Stiff limb syndrome (SLS) is a rare autoimmune-related central nervous system disorder, resulting in stiffness and spasms of limbs since onset with rare involvement of the truncal muscles. However, SLS patients will gain notable effects by appropriate therapy focusing on symptomatic treatment and immunotherapy. We reported on a 55-year-old female who showed typical painful spasms in both lower limbs and abduction of the right eyeball that partially responded to low-dose diazepam and had high-titer anti-glutamic acid decarboxylase (anti-GAD) antibody. Electromyography (EMG) only showed continuous motor unit activity (CMUA) in the anterior tibialis and right triceps. Eventually, our patient was diagnosed with SLS and treated with intravenous immunoglobulin (IVIG) and glucocorticoid combined simultaneously. She obtained notable effects. We also review and summarize the current literature on clinical characteristics, coexisting disease, treatment, and outcome of 40 patients with SLS. We hope that this report will provide a basis for further understanding of SLS and promote the formation of more advanced diagnosis and treatment processes.

摘要

僵肢综合征(SLS)是一种罕见的自身免疫相关的中枢神经系统疾病,自发病起即导致肢体僵硬和痉挛,躯干肌肉受累罕见。然而,通过以对症治疗和免疫治疗为主的适当治疗,SLS患者会取得显著疗效。我们报告了一名55岁女性,她双下肢出现典型的疼痛性痉挛,右眼球外展,对低剂量地西泮部分有效,且抗谷氨酸脱羧酶(抗GAD)抗体滴度高。肌电图(EMG)仅显示胫前肌和右三头肌有持续运动单位活动(CMUA)。最终,我们的患者被诊断为SLS,并同时接受静脉注射免疫球蛋白(IVIG)和糖皮质激素治疗。她取得了显著疗效。我们还回顾并总结了40例SLS患者的临床特征、并存疾病、治疗及预后的当前文献。我们希望本报告将为进一步了解SLS提供依据,并促进更先进的诊断和治疗流程的形成。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a99a/7232827/86eabb8da2a5/fneur-11-00284-g0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a99a/7232827/e97aec5e7004/fneur-11-00284-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a99a/7232827/86eabb8da2a5/fneur-11-00284-g0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a99a/7232827/e97aec5e7004/fneur-11-00284-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a99a/7232827/86eabb8da2a5/fneur-11-00284-g0002.jpg

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本文引用的文献

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[A case of stiff-limb syndrome with anti-glycine receptor antibody].[一例伴有抗甘氨酸受体抗体的僵肢综合征病例]
Rinsho Shinkeigaku. 2019 Feb 23;59(2):98-101. doi: 10.5692/clinicalneurol.cn-001239. Epub 2019 Jan 31.
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Stiff Limb Syndrome Mimicking Corticobasal Syndrome.酷似皮质基底节综合征的僵肢综合征
Mov Disord Clin Pract. 2014 Oct 28;1(4):354-356. doi: 10.1002/mdc3.12059. eCollection 2014 Dec.
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Pragmatic Treatment of Stiff Person Spectrum Disorders.僵人谱系障碍的实用治疗
Mov Disord Clin Pract. 2018 Jul 19;5(4):394-401. doi: 10.1002/mdc3.12629. eCollection 2018 Jul-Aug.
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Pseudo spastic gait can reveal a Stiff Leg Syndrome (SLS).假性痉挛步态可能提示僵腿综合征(SLS)。
Clin Neurol Neurosurg. 2016 Aug;147:108-9. doi: 10.1016/j.clineuro.2016.05.026. Epub 2016 Jun 13.
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Stiff person syndrome and other immune-mediated movement disorders - new insights.僵人综合征及其他免疫介导的运动障碍——新见解
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Clinical and Immunologic Investigations in Patients With Stiff-Person Spectrum Disorder.硬人综合征患者的临床和免疫研究。
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Clinical Spectrum of Stiff Person Syndrome: A Review of Recent Reports.僵人综合征的临床谱:近期报告综述
Tremor Other Hyperkinet Mov (N Y). 2016 Mar 4;6:340. doi: 10.7916/D85M65GD. eCollection 2016.
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Recent Advances and Review on Treatment of Stiff Person Syndrome in Adults and Pediatric Patients.成人及儿童僵人综合征治疗的最新进展与综述
Cureus. 2015 Dec 22;7(12):e427. doi: 10.7759/cureus.427.
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Stiff-person syndrome: insights into a complex autoimmune disorder.僵人综合征:对一种复杂自身免疫性疾病的深入了解。
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Antiglycine receptor-related stiff limb syndrome in a patient with chronic lymphocytic leukaemia.一名慢性淋巴细胞白血病患者的抗甘氨酸受体相关僵肢综合征
BMJ Case Rep. 2013 May 20;2013:bcr2013008667. doi: 10.1136/bcr-2013-008667.