Mygland A, Vincent A, Newsom-Davis J, Kaminski H, Zorzato F, Agius M, Gilhus N E, Aarli J A
Department of Neurology, University of Bergen, Norway.
Arch Neurol. 2000 Apr;57(4):527-31. doi: 10.1001/archneur.57.4.527.
About 50% of patients with thymoma have paraneoplastic myasthenia gravis (MG). Myositis and myocarditis or neuromyotonia (NMT) will also develop in some. Patients with thymoma-associated MG produce autoantibodies to a variety of neuromuscular antigens, particularly acetylcholine receptor (AChR), titin, skeletal muscle calcium release channel (ryanodine receptor [RyR]), and voltage-gated potassium channels (VGKC).
To examine whether neuromuscular autoantibodies in patients with thymoma correlate with specific clinical syndromes.
Serum and plasma samples from 19 patients with thymoma-associated MG, of whom 5 had myositis and 6 had NMT, underwent testing for antibodies to AChR, titin, RyR, and VGKC.
Antibodies to AChR and titin were found in 19 and 17 patients, respectively. Antibodies to RyR correlated with the presence of myositis (P = .03); they were found in all 5 patients with myositis and in only 1 patient with NMT, but also in 4 of 8 patients with neither disease. Antibodies to VGKC were found in 4 patients with NMT, 1 of 3 patients undergoing testing for myositis, and 2 of 7 patients undergoing testing with neither comorbidity. Presence of RyR antibodies correlated with high levels of titin antibodies.
The results appear to distinguish partially between 3 groups of patients with thymoma-associated MG: the first with RyR antibodies and myositis or myocarditis, the second with NMT without RyR antibodies, and the third without RyR antibodies, myositis, or NMT. Differences in the thymoma may underlie these pathologic associations.
约50%的胸腺瘤患者患有副肿瘤性重症肌无力(MG)。部分患者还会发展为肌炎、心肌炎或神经性肌强直(NMT)。胸腺瘤相关MG患者会产生针对多种神经肌肉抗原的自身抗体,尤其是乙酰胆碱受体(AChR)、肌联蛋白、骨骼肌钙释放通道(兰尼碱受体[RyR])和电压门控钾通道(VGKC)。
研究胸腺瘤患者的神经肌肉自身抗体是否与特定临床综合征相关。
对19例胸腺瘤相关MG患者的血清和血浆样本进行检测,其中5例患有肌炎,6例患有NMT,检测其针对AChR、肌联蛋白、RyR和VGKC的抗体。
分别在19例和17例患者中发现了AChR抗体和肌联蛋白抗体。RyR抗体与肌炎的存在相关(P = 0.03);在所有5例肌炎患者中均发现了RyR抗体,仅1例NMT患者中发现了该抗体,但在8例既无肌炎也无NMT的患者中有4例也发现了该抗体。在4例NMT患者、3例接受肌炎检测的患者中有1例以及7例未合并任何疾病接受检测的患者中有2例发现了VGKC抗体。RyR抗体的存在与高水平的肌联蛋白抗体相关。
结果似乎部分区分了3组胸腺瘤相关MG患者:第一组有RyR抗体且患有肌炎或心肌炎;第二组有NMT但无RyR抗体;第三组无RyR抗体、肌炎或NMT。胸腺瘤的差异可能是这些病理关联的基础。