Oh Hyeonjun, Kang Seong Woong, Choi Won Ah, Lee Jang Woo, Suh Miri, Kim Eun Young
Department of Rehabilitation Medicine, Gangnam Severance Hospital, Seoul, Korea.
Rehabilitation Institute of Neuromuscular Disease, Yonsei University College of Medicine, Seoul, Korea.
Ann Rehabil Med. 2017 Apr;41(2):328-331. doi: 10.5535/arm.2017.41.2.328. Epub 2017 Apr 27.
Motor neuron diseases (MNDs) refer to a heterogeneous group of progressive neurologic disorders caused by degeneration of motor neurons. The diseases affect either the upper motor neurons, lower motor neurons, or both, and are characterized by weakness, atrophy, fasciculation, spasticity, and respiratory failure. We report a case of a 61-year-old male patient with no past history of cardiovascular or pulmonary disease, who presented with only dyspnea, and no indication of any other symptom such as muscle weakness, atrophy, or bulbar dysfunction. Neuromuscular conduction study, including a study of the phrenic nerve, confirmed the diagnosis of MND. The patient greatly improved giving respiratory assistance at night, using a noninvasive ventilator. This case indicates that MNDs should be considered as differential diagnoses for patients showing acute respiratory failure of unknown causes. This report will aid in the prompt diagnosis and treatment of MNDs.
运动神经元病(MNDs)是指由运动神经元变性引起的一组异质性进行性神经疾病。这些疾病可影响上运动神经元、下运动神经元或两者,其特征为肌无力、萎缩、肌束震颤、痉挛和呼吸衰竭。我们报告一例61岁男性患者,既往无心血管或肺部疾病史,仅表现为呼吸困难,无肌无力、萎缩或延髓功能障碍等其他任何症状。神经肌肉传导研究,包括膈神经研究,确诊为MND。该患者通过夜间使用无创呼吸机给予呼吸支持后病情明显改善。该病例表明,对于不明原因急性呼吸衰竭的患者,应将MNDs列为鉴别诊断。本报告将有助于MNDs的及时诊断和治疗。