Uemura Sota, Fujiwara Satoru, Sasada Tsuyoshi, Kohara Nobuo, Kawamoto Michi
Department of Neurology, Kobe City Medical Center General Hospital, Kobe, JPN.
Department of Respiratory Medicine, Kobe City Medical Center General Hospital, Kobe, JPN.
Cureus. 2025 Jan 31;17(1):e78284. doi: 10.7759/cureus.78284. eCollection 2025 Jan.
Lambert-Eaton myasthenic syndrome (LEMS) is a paraneoplastic or autoimmune neuromuscular disorder that typically presents with limb weakness or autonomic dysfunction. Here, we report a rare case of LEMS with acute type 2 respiratory failure as the initial symptom. A 79-year-old woman was admitted with acute disturbance of consciousness and type 2 respiratory failure. On admission, she presented with confusion, seizures, and respiratory acidosis requiring non-invasive positive pressure ventilation. Cerebrospinal fluid analysis revealed pleocytosis and elevated protein levels without infection. Electroencephalography showed nonspecific slowing, while imaging revealed no abnormalities. A nerve conduction study on day 10 confirmed LEMS, with findings of low-amplitude compound motor action potentials and facilitation on high-frequency stimulation. Coexisting autoimmune encephalitis was diagnosed based on clinical presentation and serum antibody positivity for voltage-gated calcium channels and gamma-aminobutyric acid B receptors. Positron emission tomography/CT identified small-cell lung cancer, confirmed by biopsy. The patient and family declined invasive cancer therapies. Then, the patient passed away on day 60. This case highlights the diagnostic challenge of atypical presentations of paraneoplastic neurological syndromes, including LEMS and autoimmune encephalitis. Early recognition of LEMS in patients with unexplained type 2 respiratory failure through nerve conduction studies is critical. This report underscores the importance of considering paraneoplastic etiologies in patients with multiple neurological syndromes.
兰伯特-伊顿肌无力综合征(LEMS)是一种副肿瘤性或自身免疫性神经肌肉疾病,通常表现为肢体无力或自主神经功能障碍。在此,我们报告一例罕见的以急性2型呼吸衰竭为首发症状的LEMS病例。一名79岁女性因急性意识障碍和2型呼吸衰竭入院。入院时,她出现意识模糊、癫痫发作和呼吸性酸中毒,需要无创正压通气。脑脊液分析显示有细胞增多和蛋白水平升高,但无感染迹象。脑电图显示非特异性减慢,而影像学检查未发现异常。第10天的神经传导研究确诊为LEMS,结果显示复合运动动作电位波幅降低以及高频刺激时出现易化现象。根据临床表现以及血清中电压门控钙通道和γ-氨基丁酸B受体抗体阳性,诊断为合并自身免疫性脑炎。正电子发射断层扫描/计算机断层扫描(PET/CT)发现了小细胞肺癌,活检得以证实。患者及其家属拒绝接受侵入性癌症治疗。随后,患者在第60天去世。该病例突出了副肿瘤性神经综合征非典型表现(包括LEMS和自身免疫性脑炎)的诊断挑战。通过神经传导研究早期识别不明原因的2型呼吸衰竭患者中的LEMS至关重要。本报告强调了在患有多种神经综合征的患者中考虑副肿瘤病因的重要性。