Department of Neurology, Inje University College of Medicine, Busan Paik Hospital, Busan, Korea.
Department of Neurology, College of Medicine, Hallym University, Seoul, Korea.
J Clin Sleep Med. 2014 Apr 15;10(4):441-2. doi: 10.5664/jcsm.3626.
Fatigability after exercise and restoration of strength by rest are clinical hallmarks of myasthenia gravis (MG). These unique features are originated from a reduced safety factor, which is related to a defect of acetylcholine receptor, and post-exercise exhaustion of neuromuscular junction transmission. Therefore, most patients with MG feel recharged muscle strength and show normal muscle power after nocturnal sleep. As such, worsening of MG symptoms during nighttime is usual. However, in very rare cases, some patients with MG complain fatigability and weakness in the morning, so called "paradoxical weakness (PW)." We report two MG patients with typical PW, diagnosed with OSA by polysomnography. CPAP therapy successfully improved their morning symptoms and quality of life. So far, the detailed mechanism of PW in MG is unknown; however, our report highlights the possible role of sleep disorders in developing a PW in MG and the therapeutic target for life quality of MG.
运动后疲劳和休息时力量恢复是重症肌无力 (MG) 的临床特征。这些独特的特征源于安全系数降低,这与乙酰胆碱受体缺陷以及运动后神经肌肉接头传递衰竭有关。因此,大多数 MG 患者在夜间睡眠后会感到肌肉力量得到恢复,并表现出正常的肌肉力量。因此,MG 症状在夜间恶化是很常见的。然而,在极少数情况下,一些 MG 患者会抱怨晨发性疲劳和无力,即“反常性无力 (PW)。”我们报告了两名经多导睡眠图诊断为 OSA 的典型 PW 型 MG 患者。CPAP 治疗成功改善了他们的晨发性症状和生活质量。迄今为止,MG 中 PW 的详细机制尚不清楚;然而,我们的报告强调了睡眠障碍在 MG 中发展为 PW 以及治疗 MG 生活质量的治疗靶点中的可能作用。