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

Why It Is Not Always Anxiety: A Tough Diagnosis of Stiff Person Syndrome
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DOI:
10.1155/2017/7431092
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发表时间:
2017-01-01
影响因子:
0.9
通讯作者:
Cross, Jonathan S.
Cross, Jonathan S.
中科院分区:
其他
文献类型:
--
作者:
Cervantes, Carmen Elena;Lau, Hsien Lee;Cross, Jonathan S.

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焦虑症是一种常用的诊断方法,它可能会掩盖潜在的情况。僵直人综合征(SPS)是一种罕见的神经免疫学疾病,其特征是渐进性僵直和肌肉痉挛疼痛,影响轴心和下肢肌肉系统。这些发作可以由突然的运动、噪音或情绪压力引发,这可能是一种精神状态。我们报告一位30岁女性,因焦虑、僵硬和行走困难而多次住院治疗,表现为反复恐慌症发作。以前的脑电(EEG)和脑和颈椎磁共振成像(MRI)并不明显。她接受了安定和β-受体阻滞剂的经验性治疗,谷氨酸脱羧酶(GAD)抗体阳性证实了这一点。患者的症状对苯二氮卓类药物无效,需要类固醇和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)中的抗体最常针对伽马氨基丁酸(GABA)神经元上的GAD抗原。治疗的目的是改善症状,提高生活质量。我们的SPS病例自症状出现以来被掩盖为广泛性焦虑症至少六年。如本例所示,两种诊断标准可能有重叠。
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.