长时间横膈膜麻痹后出现的运动神经元疾病

Joerg Steier , Caroline Jolley , P.Nigel Leigh , Michael I. Polkey , John Moxham
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引用次数: 0

摘要

膈肌麻痹可能是运动神经元疾病(MND)的表现特征,但可归因于该疾病的体征通常在当时或随后一个月内发现。我们报告了两例因孤立性膈肌麻痹而出现呼吸短促的患者,经膈肌压力测量和颈磁刺激膈神经证实了这一点。一位65岁男性在出现膈肌麻痹症状6年后出现高碳酸血症和进行性肌肉萎缩。一年后发现构音障碍,右侧腓总神经麻痹和舌部束动,并诊断为MND。一名47岁男性首次出现高碳酸血症性呼吸衰竭(PaCO2 18kpa),并插管和通气。患者有2年的呼吸困难病史,确诊为孤立性双侧膈肌麻痹。9个月后出现了MND的其他症状。这些病例提醒我们,MND可能是膈肌麻痹的潜在诊断,有时可能需要数年才能发展出更典型的特征。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Motor neuron disease presenting following prolonged diaphragm paralysis

Diaphragm paralysis may be the presenting feature of motor neuron disease (MND), but signs attributable to the disease are usually found at that time or within the following month.

We present two patients who developed shortness of breath due to isolated diaphragm paralysis, which was confirmed by transdiaphragmatic pressure measurements and cervical magnetic stimulation of the phrenic nerves.

A 65-year-old man developed hypercapnia and progressive muscle wasting 6 years after first symptoms of diaphragm paralysis. Dysarthria, common peroneal nerve palsy on the right and fasciculations of the tongue were found a year later and MND was diagnosed.

A 47-year-old-man first presented with hypercapnic respiratory failure (PaCO2 18 kPa) and was intubated and ventilated. He had a 2-year history of dyspnoea and isolated bilateral diaphragm paralysis was confirmed. Other symptoms of MND developed 9 months later.

These cases serve as a reminder that MND may be the underlying diagnosis of diaphragm paralysis and may occasionally take years to develop more typical features.

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