Mygland A, Aarli J A, Matre R, Gilhus N E
Department of Neurology, Gade Institute, University of Bergen, Norway.
J Neurol Neurosurg Psychiatry. 1994 Jul;57(7):843-6. doi: 10.1136/jnnp.57.7.843.
Ryanodine receptor (RyR) antibodies are detected in about 50% of patients with myasthenia gravis who have a thymoma. The RyR is a calcium release channel involved in the mechanism of excitation-contraction coupling in striated muscle. In this study the severity of myasthenia gravis assessed by a five point disability score was compared between 12 patients with myasthenia gravis, a thymoma, and RyR antibodies and 10 patients with myasthenia gravis and a thymoma but without such antibodies. Symptoms of myasthenia gravis were significantly more severe in patients with RyR antibodies. The mean (SD) disability scores were 3.7(0.5) in patients with antibodies and 2.7 (0.9) in those without at peak of illness, (p = 0.01) and 3.4(1.4) v 1.6(0.7) at the end of an average observation period of five years (p = 0.002). The number of deaths due to myasthenia gravis was five of 12 RyR antibody positive patients, and none of 10 RyR antibody negative patients (p = 0.04). RyR antibody levels correlated positively with severity of myasthenia gravis. The presence of circulating RyR antibodies seems to be associated with a severe form of thymoma associated myasthenia gravis.
在患有胸腺瘤的重症肌无力患者中,约50%可检测到兰尼碱受体(RyR)抗体。RyR是一种钙释放通道,参与横纹肌兴奋-收缩偶联机制。在本研究中,对12例患有胸腺瘤且伴有RyR抗体的重症肌无力患者和10例患有胸腺瘤但无此类抗体的重症肌无力患者,采用五点残疾评分法评估重症肌无力的严重程度。伴有RyR抗体的患者,其重症肌无力症状明显更严重。在疾病高峰期,有抗体患者的平均(标准差)残疾评分为3.7(0.5),无抗体患者为2.7(0.9),(p = 0.01);在平均五年的观察期结束时,分别为3.4(1.4)和1.6(0.7),(p = 0.002)。因重症肌无力死亡的人数,12例RyR抗体阳性患者中有5例,10例RyR抗体阴性患者中无1例(p = 0.04)。RyR抗体水平与重症肌无力的严重程度呈正相关。循环中RyR抗体的存在似乎与一种严重形式的胸腺瘤相关性重症肌无力有关。