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颅神经 IX 和 X 无力:重症肌无力的不常见初始表现。

Cranial Nerve IX and X Weakness: An Unusual Initial Presentation of Myasthenia Gravis.

机构信息

Department of Internal Medicine, Overlook Medical Center, Summit, NJ, USA.

St. George's University, School of Medicine, True Blue, West Indies, Grenada.

出版信息

Am J Case Rep. 2020 Aug 10;21:e923750. doi: 10.12659/AJCR.923750.

DOI:10.12659/AJCR.923750
PMID:32773733
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7440743/
Abstract

BACKGROUND Myasthenia gravis (MG) is an autoimmune disease characterized by antibodies binding skeletal muscle acetylcholine receptors (AChR). Rarely does the disease manifest with orolaryngeal symptoms before ocular ones. We present a case of MG that on initial presentation had symptoms of cranial nerves (CN) IX and X weakness, including dysphagia and dysphonia. CASE REPORT A 51-year-old woman with panic attacks presented to the Emergency Department (ED) with complaints of her throat closing, swallowing difficulty, and hoarse voice. Multiple ED visits revealed no etiology. However, she developed stridor, which prompted further evaluation. Laryngoscopy and imaging studies revealed no gross abnormalities; therefore, her symptoms of dysphonia and difficulty breathing were attributed to anxiety. Her hospital course was complicated by a cardiac arrest requiring intubation. ECHO, CTA chest, and MRI brain were unremarkable. Her cardiac arrest was hypothesized as being secondary to laryngeal spasm. During her ICU course, she failed extubation multiple times due to acute respiratory failure. An autoimmune etiology was suspected, prompting a paraneoplastic screen, which revealed elevated levels of AChR antibodies at 124 mmol/L. MG was diagnosed and treatment with plasmapheresis and steroids was initiated. However, complications of thrombocytopenia, anemia, and ARDS ensued, so MG treatment was discontinued. The patient was eventually transferred to a LTACH. Thereafter, at outpatient followup, her MG was treated with mycophenolate and prednisone, which led to significant symptom improvement. CONCLUSIONS MG commonly presents in the third decade with clinical features of ptosis, diplopia, and facial weakness. However, initial and isolated symptoms of dysphagia and dysphonia are rare, leading to missed diagnoses. Our case of a middle-aged woman posed a diagnostic challenge because of her uncommon presentation and comorbidities of panic attacks and obesity. This case highlights the crucial need for a high index of clinical suspicion for MG in any patient presenting with symptoms of CN IX and X weakness.

摘要

背景

重症肌无力(MG)是一种自身免疫性疾病,其特征是抗体结合骨骼肌乙酰胆碱受体(AChR)。该疾病很少在眼部症状之前出现口咽症状。我们报告一例 MG 病例,该病例最初表现为颅神经(CN)IX 和 X 无力的症状,包括吞咽困难和声音嘶哑。

病例报告

一名 51 岁女性因惊恐发作就诊于急诊科(ED),主诉喉咙紧闭、吞咽困难和声音嘶哑。多次 ED 就诊未发现病因。然而,她出现了喘鸣,促使进一步评估。喉镜和影像学检查未发现明显异常;因此,她的声音嘶哑和呼吸困难症状归因于焦虑。她的住院过程因需要插管的心脏骤停而复杂化。ECHO、CTA 胸部和 MRI 脑部均无明显异常。她的心脏骤停被认为是喉痉挛的继发原因。在 ICU 期间,由于急性呼吸衰竭,她多次尝试拔管失败。怀疑存在自身免疫性病因,促使进行副肿瘤筛查,发现乙酰胆碱受体抗体水平升高至 124mmol/L。诊断为重症肌无力,并开始进行血浆置换和类固醇治疗。然而,随后出现血小板减少症、贫血和 ARDS 等并发症,因此停止了 MG 治疗。患者最终转入 LTACH。此后,在门诊随访时,她接受了霉酚酸酯和泼尼松治疗,症状显著改善。

结论

MG 通常在第三十年出现,临床特征为上睑下垂、复视和面部无力。然而,吞咽困难和声音嘶哑的初始和孤立症状较为罕见,导致误诊。我们报告的这名中年女性的病例因不常见的表现和惊恐发作和肥胖等合并症而构成诊断挑战。该病例强调了在任何出现 CN IX 和 X 无力症状的患者中,对 MG 进行高度临床怀疑的重要性。

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