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重症肌无力患者呼吸衰竭评估中的陷阱:病例系列

Pitfalls in the Evaluation of Respiratory Failure in Myasthenia Gravis Patients: A Case Series.

作者信息

Uysal Sanem Pinar, Li Yuebing

机构信息

Department of Neurology, Cleveland Clinic, Cleveland, OH, USA.

出版信息

Neurohospitalist. 2024 Aug 31:19418744241280528. doi: 10.1177/19418744241280528.

Abstract

OBJECTIVES

To highlight the importance of recognizing different presentations of respiratory failure due to myasthenic and non-myasthenic etiologies in myasthenia gravis (MG) patients.

METHODS

We describe 3 patients with different presentations of respiratory failure in MG.

CASES

Patient 1 is a 49-year-old female with longstanding MG who presented with lethargy and neck weakness without notable respiratory distress. She was found to be in hypercarbic respiratory failure, which improved with plasmapheresis treatment. Patient 2 is a 58-year-old female who presented with ptosis, dysphagia, and dyspnea requiring intubation. Her hypophonia and dyspnea persisted despite escalation in MG treatment, and further workup revealed glottal stenosis secondary to granulomatosis with polyangiitis. Patient 3 is an 85-year-old female with MG presenting with refractory hypoxia, which was secondary to a large patent foramen ovale resulting in right-to-left shunting.

DISCUSSION

All 3 cases emphasize the role of clinical reasoning and careful analysis based on thorough history taking, detailed neurologic exam and comprehensive laboratory findings to determine the etiologies for respiratory dysfunction in MG and provide appropriate treatment.

CONCLUSION

A lack of overt signs of respiratory distress in MG does not rule out the presence of respiratory failure due to the sedating effect of hypercapnia. There is a need to consider alternative etiologies of hypoxia in MG patients if typical symptoms or signs of MG exacerbations are absent.

摘要

目的

强调认识重症肌无力(MG)患者因重症肌无力及非重症肌无力病因导致的呼吸衰竭不同表现形式的重要性。

方法

我们描述了3例MG患者出现不同表现形式呼吸衰竭的情况。

病例

病例1是一名49岁女性,患有长期MG,表现为嗜睡和颈部无力,无明显呼吸窘迫。她被发现存在高碳酸血症性呼吸衰竭,经血浆置换治疗后病情改善。病例2是一名58岁女性,表现为上睑下垂、吞咽困难和呼吸困难,需要插管。尽管MG治疗升级,她的声音微弱和呼吸困难仍持续存在,进一步检查发现继发于肉芽肿性多血管炎的声门狭窄。病例3是一名85岁患有MG的女性,表现为难治性低氧血症,继发于大型卵圆孔未闭导致的右向左分流。

讨论

所有3例病例都强调了基于全面病史采集、详细神经系统检查和综合实验室检查结果进行临床推理和仔细分析,以确定MG患者呼吸功能障碍病因并提供适当治疗的作用。

结论

MG患者缺乏明显的呼吸窘迫体征并不排除由于高碳酸血症的镇静作用导致呼吸衰竭的存在。如果MG加重的典型症状或体征不存在,有必要考虑MG患者低氧血症的其他病因。

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