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以急性呼吸衰竭为首发表现的不对称性膈神经损伤的肌萎缩侧索硬化症罕见病例。

A Rare Case of Amyotrophic Lateral Sclerosis With Asymmetrical Phrenic Nerve Lesion Presenting With Acute Respiratory Failure as an Initial Manifestation.

作者信息

Blanco Rubén, Pichardo Jessie, Abdullah Hassan

机构信息

Neurology, Pontificia Universidad Católica Madre y Maestra, Santiago, DOM.

Medicine, Pontificia Universidad Católica Madre y Maestra, Santiago, DOM.

出版信息

Cureus. 2021 Jan 7;13(1):e12547. doi: 10.7759/cureus.12547.

DOI:10.7759/cureus.12547
PMID:33564541
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7863083/
Abstract

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that causes muscle weakness, disability, and eventually, death. Respiratory failure is the leading cause of death in ALS. It is common in the advanced stages of the disease. However, acute respiratory failure is a presenting symptom in only a small number of patients, such as in our case. Here, we present the case of a 54-year-old woman with ALS presenting with respiratory failure due to unilateral diaphragm paralysis as the first manifestation. Although rare, respiratory muscle function failure can be the first symptom of motor neuron disease. Therefore, a motor neuron disease such as ALS, which leads to respiratory muscle weakness and diaphragm paralysis, should be considered in cases of unexplained acute respiratory failure.

摘要

肌萎缩侧索硬化症(ALS)是一种进行性神经退行性疾病,会导致肌肉无力、残疾,最终导致死亡。呼吸衰竭是ALS患者的主要死因。这在疾病晚期很常见。然而,急性呼吸衰竭仅在少数患者中作为首发症状出现,比如我们的病例。在此,我们报告一例54岁女性ALS患者,以单侧膈肌麻痹导致的呼吸衰竭为首发表现。虽然罕见,但呼吸肌功能衰竭可能是运动神经元病的首发症状。因此,对于不明原因的急性呼吸衰竭病例,应考虑像ALS这样导致呼吸肌无力和膈肌麻痹的运动神经元病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/03d2/7863083/c03e35566755/cureus-0013-00000012547-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/03d2/7863083/c1caf8ea92e3/cureus-0013-00000012547-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/03d2/7863083/8821b54bbc42/cureus-0013-00000012547-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/03d2/7863083/94d97148e2dd/cureus-0013-00000012547-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/03d2/7863083/04e9933b217c/cureus-0013-00000012547-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/03d2/7863083/f3cd4fef26cd/cureus-0013-00000012547-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/03d2/7863083/c03e35566755/cureus-0013-00000012547-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/03d2/7863083/c1caf8ea92e3/cureus-0013-00000012547-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/03d2/7863083/8821b54bbc42/cureus-0013-00000012547-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/03d2/7863083/94d97148e2dd/cureus-0013-00000012547-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/03d2/7863083/04e9933b217c/cureus-0013-00000012547-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/03d2/7863083/f3cd4fef26cd/cureus-0013-00000012547-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/03d2/7863083/c03e35566755/cureus-0013-00000012547-i06.jpg

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本文引用的文献

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Early and rapidly progressing respiratory failure in a patient with amyotrophic lateral sclerosis: when FVC% is misleading.肌萎缩侧索硬化症患者早期快速进展的呼吸衰竭:当用力肺活量百分比产生误导时。
Neurol Sci. 2019 Feb;40(2):421-422. doi: 10.1007/s10072-018-3590-0. Epub 2018 Oct 4.
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Diaphragmatic Palsy.膈肌麻痹
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