Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India.
Neuromuscular Laboratory (NML), Neurobiology Research Centre (NBRC) National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India.
Acta Neurol Scand. 2019 May;139(5):428-437. doi: 10.1111/ane.13071. Epub 2019 Feb 19.
Data on antibody profile in myasthenia gravis (MG) from India are limited.
To investigate antibody profile in patients with MG and their clinical correlates.
Patients of MG (n = 85, M:F::1.1:1, mean age: 39.29 ± 17.3 years, mean symptom duration: 72.94 ± 91.8 months) were evaluated for clinical features, MG foundation of America (MGFA) score, response to treatment, and outcome at last follow-up. Antibodies to acetylcholine receptor (AChR), muscle-specific kinase (MUSK), titin and ryanodine receptor (RYR) were analysed using ELISA.
Based on the regional distribution of weakness, the cohort could be categorized as: generalized: 60, ocular: 16 and oculo-bulbar: 9. Sixty patients were followed up for a mean duration of 26.74 ± 13.8 months. Outcome at last follow-up was as follows: remission-22, no remission-33 and dead-5. AChR and MUSK antibodies were detected in 58 and 8 patients, respectively. Frequency of generalized MG, worse MGFA score during the disease course and thymomatous histology significantly correlated with presence of AChR-antibodies, though outcome at last follow-up was comparable between AChR-antibody positive and negative groups. Patients with MUSK antibodies had oculo-bulbar or generalized MG and frequent respiratory crisis, but majority improved or remitted with treatment. Titin antibodies were detected in 31.8% and RYR antibodies in 32.9%. Their presence did not correlate with age at onset of MG, severity or presence of thymoma.
This report highlights the spectrum of antibodies in MG in an Indian cohort. AChR-antibody positivity correlated with clinical severity. Outcome was good in majority of MUSK antibody-positive MG. The role of other antibodies, complementary vs epiphenomenon, remains open.
印度重症肌无力（MG）患者的抗体谱数据有限。
研究 MG 患者的抗体谱及其临床相关性。
评估 85 例 MG 患者（男女比例为 1.1:1，平均年龄为 39.29±17.3 岁，平均症状持续时间为 72.94±91.8 个月）的临床特征、美国重症肌无力基金会（MGFA）评分、治疗反应和末次随访时的结局。采用 ELISA 法检测乙酰胆碱受体（AChR）、肌肉特异性激酶（MUSK）、titin 和 Ryanodine 受体（RYR）抗体。
根据肌无力的区域性分布，该队列可分为：全身性 60 例、眼肌型 16 例和眼肌-球肌型 9 例。60 例患者的平均随访时间为 26.74±13.8 个月。末次随访时的结局如下：缓解 22 例、无缓解 33 例和死亡 5 例。58 例和 8 例患者分别检测到 AChR 和 MUSK 抗体。全身性 MG、疾病过程中更严重的 MGFA 评分和胸腺瘤组织学与 AChR 抗体的存在显著相关，尽管 AChR 抗体阳性和阴性组的结局相似。MUSK 抗体阳性的患者患有眼肌-球肌型或全身性 MG 和频繁的呼吸危象，但大多数患者经治疗后改善或缓解。31.8%的患者检测到 titin 抗体，32.9%的患者检测到 RYR 抗体。它们的存在与 MG 的发病年龄、严重程度或胸腺瘤的存在无关。
本报告强调了印度队列中 MG 的抗体谱。AChR 抗体阳性与临床严重程度相关。大多数 MUSK 抗体阳性的 MG 患者的结局良好。其他抗体的作用，是互补还是表现为伴随现象，仍有待确定。
