僵人谱系障碍的实用治疗
Pragmatic Treatment of Stiff Person Spectrum Disorders.
作者信息
Balint Bettina, Meinck Hans-Michael
机构信息
Sobell Department of Motor Neuroscience and Movement Disorders UCL Institute of Neurology Queen Square, London UK.
Neuroimmunology Group, Nuffield Department of Clinical Neurosciences John Radcliffe Hospital Oxford UK.
出版信息
Mov Disord Clin Pract. 2018 Jul 19;5(4):394-401. doi: 10.1002/mdc3.12629. eCollection 2018 Jul-Aug.
Abstract
BACKGROUND
Stiff person spectrum disorders (SPSD) are a group of rare conditions clinically characterized by stiffness, spasms, and heightened stimulus sensitivity. They also share a spectrum of antibodies.
METHODS
We reviewed the literature and our own experience with the aim of providing a practical approach to the treatment of SPSD.
RESULTS
Because of the rarity of SPSD, there is little evidence to guide treatment decisions. The treatment of SPSD is based on the triad of symptomatic treatment, immunotherapy, and tumor treatment where appropriate. Moreover, the management involves continuous and appropriate monitoring of the symptoms of the disease, its autoimmune associations, and potential treatment side effects.
CONCLUSIONS
Here we delineated a pragmatic treatment approach to SPSD, based on our experience and existing literature. We also highlighted how our understanding of neuronal antibodies and their implications reflects on management considerations.
摘要
背景
僵人谱系障碍（SPSD）是一组罕见疾病，临床特征为僵硬、痉挛和刺激敏感性增强。它们还存在一系列抗体。
方法
我们回顾了文献及自身经验，旨在提供一种治疗SPSD的实用方法。
结果
由于SPSD罕见，几乎没有证据可指导治疗决策。SPSD的治疗基于对症治疗、免疫治疗及必要时的肿瘤治疗这三者。此外，管理还包括持续且适当地监测疾病症状、其自身免疫关联以及潜在的治疗副作用。
结论
在此，我们根据自身经验和现有文献，阐述了一种针对SPSD的实用治疗方法。我们还强调了我们对神经元抗体及其影响的理解如何反映在管理考量中。
相似文献
1
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.
2
Therapeutic plasma exchange in the management of stiff person syndrome spectrum disorders: a case series and review of the literature.治疗性血浆置换在僵人综合征谱系障碍管理中的应用：病例系列及文献综述
Ther Adv Neurol Disord. 2023 Jul 24;16:17562864231180736. doi: 10.1177/17562864231180736. eCollection 2023.
3
[From stiff man syndrome to stiff person spectrum disorders].[从僵人综合征到僵人谱系障碍]
Nervenarzt. 2018 Feb;89(2):207-218. doi: 10.1007/s00115-017-0480-2.
4
Stiff person syndrome spectrum disorders; more than meets the eye.僵人综合征谱障碍；比想象的更复杂。
J Neuroimmunol. 2022 Aug 15;369:577915. doi: 10.1016/j.jneuroim.2022.577915. Epub 2022 Jun 12.
5
Stiff Person Spectrum Disorders-An Update and Outlook on Clinical, Pathophysiological and Treatment Perspectives.僵人谱系障碍——临床、病理生理及治疗前景的最新进展与展望
Biomedicines. 2023 Sep 10;11(9):2500. doi: 10.3390/biomedicines11092500.
6
Prevalence of non-neurological autoantibodies and related comorbidities in stiff person spectrum disorders.僵人谱系障碍中非神经自身抗体及相关共病的患病率。
Front Neurol. 2023 Nov 22;14:1289460. doi: 10.3389/fneur.2023.1289460. eCollection 2023.
7
Expanding clinical profiles and prognostic markers in stiff person syndrome spectrum disorders.扩展僵硬人综合征谱障碍的临床特征和预后标志物。
J Neurol. 2024 Apr;271(4):1861-1872. doi: 10.1007/s00415-023-12123-0. Epub 2023 Dec 11.
8
Review of 23 patients affected by the stiff man syndrome: clinical subdivision into stiff trunk (man) syndrome, stiff limb syndrome, and progressive encephalomyelitis with rigidity.对23例僵人综合征患者的回顾：临床分为僵躯干（人）综合征、僵肢综合征和进行性脑脊髓炎伴强直。
J Neurol Neurosurg Psychiatry. 1998 Nov;65(5):633-40. doi: 10.1136/jnnp.65.5.633.
9
The many faces of gastrointestinal dysfunction in stiff person syndrome spectrum disorders.僵人综合征谱系障碍中胃肠功能障碍的多种表现
Front Neurol. 2023 Oct 6;14:1273256. doi: 10.3389/fneur.2023.1273256. eCollection 2023.
10
A case of stiff dog syndrome associated with anti-glutamic acid decarboxylase antibodies.一例与抗谷氨酸脱羧酶抗体相关的僵狗综合征病例。
J Clin Mov Disord. 2017 May 10;4:5. doi: 10.1186/s40734-017-0053-3. eCollection 2017.
引用本文的文献
1
Stiff-person syndrome and related disorders - diagnosis, mechanisms and therapies.僵人综合征及相关疾病的诊断、发病机制与治疗。
Nat Rev Neurol. 2024 Oct;20(10):587-601. doi: 10.1038/s41582-024-01012-3. Epub 2024 Sep 3.
2
Progressive encephalomyelitis with rigidity and myoclonus: a pediatric case report and literature review.进行性脑脊髓炎伴僵硬和肌阵挛：一例儿科病例报告及文献复习。
BMC Pediatr. 2024 Jul 3;24(1):427. doi: 10.1186/s12887-024-04899-5.
3
Axial dystonia as a manifestation of stiff-person syndrome in a paediatric patient.轴性肌张力障碍作为儿童僵人综合征的一种表现。
BMJ Case Rep. 2024 Feb 15;17(2):e256448. doi: 10.1136/bcr-2023-256448.
4
Stiff Person Spectrum Disorders-An Update and Outlook on Clinical, Pathophysiological and Treatment Perspectives.僵人谱系障碍——临床、病理生理及治疗前景的最新进展与展望
Biomedicines. 2023 Sep 10;11(9):2500. doi: 10.3390/biomedicines11092500.
5
Relevance of Antibody Testing in Movement Disorders.抗体检测在运动障碍中的相关性。
Mov Disord Clin Pract. 2023 Jun 12;10(Suppl 2):S32-S35. doi: 10.1002/mdc3.13772. eCollection 2023 Aug.
6
COVID-19 Still Surprising Us-A Rare Movement Disorder Induced by Infection.新冠病毒仍让我们惊讶——一种由感染引发的罕见运动障碍
Brain Sci. 2022 Dec 17;12(12):1733. doi: 10.3390/brainsci12121733.
7
One in a Million: A Case Report of Stiff Person Syndrome.百万分之一：僵人综合征病例报告
Case Rep Rheumatol. 2022 Jan 13;2022:7741545. doi: 10.1155/2022/7741545. eCollection 2022.
8
Treatable Hyperkinetic Movement Disorders Not to Be Missed.不可漏诊的可治疗性运动亢进性疾病
Front Neurol. 2021 Dec 1;12:659805. doi: 10.3389/fneur.2021.659805. eCollection 2021.
9
From Collar to Coccyx: Truncal Movement Disorders: A Clinical Review.从颈部到尾骨：躯干运动障碍：临床综述
Mov Disord Clin Pract. 2021 Aug 26;8(7):1027-1033. doi: 10.1002/mdc3.13325. eCollection 2021 Oct.
10
Paraneoplastic neurological syndrome: an evolving story.副肿瘤性神经系统综合征：一个不断演变的故事。
Neurooncol Pract. 2021 Feb 24;8(4):362-374. doi: 10.1093/nop/npab002. eCollection 2021 Aug.
本文引用的文献
1
Glycine receptor modulating antibody predicting treatable stiff-person spectrum disorders.预测可治疗的僵人综合征谱系障碍的甘氨酸受体调节抗体。
Neurol Neuroimmunol Neuroinflamm. 2018 Jan 23;5(2):e438. doi: 10.1212/NXI.0000000000000438. eCollection 2018 Mar.
2
Movement disorders with neuronal antibodies: syndromic approach, genetic parallels and pathophysiology.神经元抗体相关运动障碍：综合征方法、遗传相似性和病理生理学。
Brain. 2018 Jan 1;141(1):13-36. doi: 10.1093/brain/awx189.
3
A double-blind, placebo-controlled study of rituximab in patients with stiff person syndrome.一项关于利妥昔单抗治疗僵人综合征患者的双盲、安慰剂对照研究。
Ann Neurol. 2017 Aug;82(2):271-277. doi: 10.1002/ana.25002. Epub 2017 Aug 9.
4
Paraneoplastic cerebellar degeneration associated with anti-ITPR1 antibodies.与抗ITPR1抗体相关的副肿瘤性小脑变性
Neurol Neuroimmunol Neuroinflamm. 2017 Feb 3;4(2):e326. doi: 10.1212/NXI.0000000000000326. eCollection 2017 Mar.
5
Whole-Body F-FDG PET and F-FDG PET/CT in Patients with Suspected Paraneoplastic Syndrome: A Systematic Review and Meta-Analysis of Diagnostic Accuracy.全身 F-FDG PET 和 F-FDG PET/CT 在疑似副肿瘤综合征患者中的应用：系统评价和诊断准确性的荟萃分析。
J Nucl Med. 2017 Jul;58(7):1031-1036. doi: 10.2967/jnumed.116.183905. Epub 2016 Dec 15.
6
Serotonin-norepinephrine reuptake inhibitors may exacerbate stiff-person syndrome.血清素-去甲肾上腺素再摄取抑制剂可能会加重僵人综合征。
Neurol Neuroimmunol Neuroinflamm. 2016 Aug 29;3(5):e281. doi: 10.1212/NXI.0000000000000281. eCollection 2016 Oct.
7
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
Curr Opin Neurol. 2016 Aug;29(4):496-506. doi: 10.1097/WCO.0000000000000351.
8
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。
JAMA Neurol. 2016 Jun 1;73(6):714-20. doi: 10.1001/jamaneurol.2016.0133.
9
Pediatric stiff-person syndrome with renal failure.小儿僵人综合征伴肾衰竭
J Neurosci Rural Pract. 2016 Jan-Mar;7(1):147-9. doi: 10.4103/0976-3147.165352.
10
Early-onset immunotherapy by intravenous immunoglobulin and corticosteroids in well characterized onconeural-antibody-positive paraneoplastic neurological syndrome.在特征明确的肿瘤神经抗体阳性副肿瘤性神经系统综合征中，通过静脉注射免疫球蛋白和皮质类固醇进行早期免疫治疗。
Clin Exp Immunol. 2014 Dec;178 Suppl 1(Suppl 1):127-9. doi: 10.1111/cei.12539.
Zotero 插件