眼肌型重症肌无力缓解的预后预测因子。
Prognostic predictors of remission in ocular myasthenia gravis.
机构信息
Department of Neurology, Ege University Medical School, 35100, Izmir, Turkey.
Department of Neurology, Celal Bayar University Medical School, 45000, Manisa, Turkey.
出版信息
Acta Neurol Belg. 2023 Oct;123(5):1927-1932. doi: 10.1007/s13760-022-02151-4. Epub 2022 Dec 6.
BACKGROUND
Ocular myasthenia gravis (OMG) constitutes 15% of all myasthenia gravis patients.
METHODS
One hundred eight patients with OMG followed-up for over 36 months were retrospectively evaluated regarding factors associated with remission. Demographic features, neuro-ophthalmologic findings at onset, acetylcholine receptor (AChR Ab) and muscle-specifc tyrosine kinase antibodies (MuSK Ab), thymic status, single fiber electromyography (SFEMG) results were the variables considered.
RESULTS
Median age of disease onset was 57 years (range 18-82 years). Clinical features at onset was isolated ptosis in 55 (50.9%) and isolated diplopia in 33 (30.6%) patients. Combined ptosis and diplopia were present in 20 (18.5%) patients. Among 75 patients with ptosis, it was unilateral in 65 (86.7%) and bilateral in 10 (13.3%). AChR Abs were found in 66 (61.1%) and MuSK Abs in 2 (1.9%) patients. SFEMG abnormality was detected in 74 (68.5%) patients. Thymoma was present in 16 (14.8%) and thymic hyperplasia in 6 (5.6%) patients. Forty-one patients (37.9%) had been treated with pyridostigmine alone. Sixty-seven (62%) patients were given immunosupressive drugs. In 53 (49.1%) prednisone was used and in 14 (12.9%) patients it was combined with azathioprine. Thymectomy was performed in all 16 patients with thymoma. Complete stable remission (CSR) was achieved in 49 (45.4%) patients. Fifty-nine (54.6%) patients had reached minimal manifestation (MM) status; 32 (29.6%) having a status of MM-1 and 27 (25%) a status of MM-3.
CONCLUSIONS
The presence of AchR Abs (p = 0.034) and an abnormal SFEMG (p = 0.006) at onset as increased risk factors for the presence of ongoing signs necessitating medical treatment.
背景
眼肌型重症肌无力（OMG）占所有重症肌无力患者的 15%。
方法
对 108 例随访时间超过 36 个月的 OMG 患者进行回顾性评估，以确定与缓解相关的因素。考虑的变量包括人口统计学特征、发病时的神经眼科发现、乙酰胆碱受体（AChR Ab）和肌肉特异性酪氨酸激酶抗体（MuSK Ab）、胸腺瘤状态、单纤维肌电图（SFEMG）结果。
结果
疾病发病的中位年龄为 57 岁（范围 18-82 岁）。发病时的临床特征为 55 例（50.9%）单纯上睑下垂和 33 例（30.6%）单纯复视。20 例（18.5%）患者同时存在上睑下垂和复视。在 75 例有上睑下垂的患者中，单侧 65 例（86.7%），双侧 10 例（13.3%）。AChR Ab 阳性 66 例（61.1%），MuSK Ab 阳性 2 例（1.9%）。SFEMG 异常 74 例（68.5%）。胸腺瘤 16 例（14.8%），胸腺增生 6 例（5.6%）。41 例（37.9%）患者单独接受吡啶斯的明治疗。67 例（62%）患者给予免疫抑制剂治疗。53 例（49.1%）患者使用泼尼松，14 例（12.9%）患者联合使用硫唑嘌呤。16 例胸腺瘤患者均行胸腺切除术。49 例（45.4%）患者达到完全稳定缓解（CSR）。59 例（54.6%）患者达到最小表现（MM）状态；32 例（29.6%）为 MM-1 状态，27 例（25%）为 MM-3 状态。
结论
发病时存在 AchR Ab（p=0.034）和异常的 SFEMG（p=0.006）是存在持续需要药物治疗的体征的危险因素。
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