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为何并不总是焦虑:僵人综合征的艰难诊断

Why It Is Not Always Anxiety: A Tough Diagnosis of Stiff Person Syndrome.

作者信息

Cervantes Carmen Elena, Lee Lau Hsien, Binazir Tina Ataian, O'Brien Keith O, Cross Jonathan S

机构信息

Department of Medicine, Aventura Hospital and Medical Center, Aventura, FL, USA.

出版信息

Case Rep Neurol Med. 2017;2017:7431092. doi: 10.1155/2017/7431092. Epub 2017 Aug 14.

Abstract

Anxiety disorder is a commonly used diagnosis that may mask underlying conditions. Stiff person syndrome (SPS) is a rare neuroimmunological disorder characterized by progressive rigidity and painful muscle spasms affecting axial and lower extremity musculature. These episodes can be triggered by sudden movement, noise, or emotional stress, which may present as a psychiatric condition. We report the case of a 30-year-old female who presented with recurrent panic attacks with multiple prior hospital admissions for anxiety, rigidity, and difficulty in walking. Previous electroencephalogram (EEG) and brain and cervical spine magnetic resonance imaging (MRI) were unremarkable. She was empirically treated with diazepam and beta-blockers for SPS, which was confirmed by positive glutamic acid decarboxylase (GAD) antibodies. The patient's symptoms became refractory to benzodiazepines and required steroids with intravenous immunoglobulin (IVIG). Her rigidity subsequently responded to plasmapheresis. In SPS, antibodies in the cerebrospinal fluid (CSF) most commonly target the GAD antigen on gamma-aminobutyric acid (GABA) neurons. The goal of treatment is to ameliorate symptoms and improve quality of life. Our case of SPS was masked as generalized anxiety disorder for at least six years since onset of symptoms. The criteria for both diagnoses may overlap as seen in this patient.

摘要

焦虑症是一种常用的诊断,可能掩盖潜在疾病。僵人综合征(SPS)是一种罕见的神经免疫性疾病,其特征为进行性僵硬和影响躯干及下肢肌肉组织的疼痛性肌肉痉挛。这些发作可由突然运动、噪音或情绪应激引发,可能表现为精神疾病状态。我们报告一例30岁女性病例,该患者反复出现惊恐发作,此前因焦虑、僵硬和行走困难多次住院。既往脑电图(EEG)及脑和颈椎磁共振成像(MRI)均无异常。她经验性地接受了地西泮和β受体阻滞剂治疗SPS,谷氨酸脱羧酶(GAD)抗体阳性证实了该病。患者症状对苯二氮䓬类药物难治,需要使用类固醇及静脉注射免疫球蛋白(IVIG)。她的僵硬随后对血浆置换有反应。在SPS中,脑脊液(CSF)中的抗体最常靶向γ-氨基丁酸(GABA)神经元上的GAD抗原。治疗目标是改善症状和提高生活质量。我们的SPS病例自症状出现后至少六年被误诊为广泛性焦虑症。这两种诊断的标准可能重叠,正如该患者所见。

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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。
J Neurol Neurosurg Psychiatry. 2015 Aug;86(8):840-8. doi: 10.1136/jnnp-2014-309201. Epub 2014 Dec 15.
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Stiff person syndrome masquerading as panic attacks.伪装成惊恐发作的僵人综合征。
Lancet. 2014 Feb 15;383(9917):668. doi: 10.1016/S0140-6736(14)60127-6.
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Stiff-person syndrome: commonly mistaken for hysterical paralysis.僵人综合征:常被误诊为癔症性瘫痪。
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