重症肌无力2385例的临床资料研究

Myasthenia gravis: clinical study in 2 385 patients

目的 研究重症肌无力（ＭＧ） 的临床特点。方法 回顾性分析１９５６ ～１９９８ 年间诊治的２ ３８５ 例ＭＧ病例资料。结果 本组ＭＧ 患者性别差异较小（男∶女＝１∶１ ．０１） ，发病年龄以儿童期为主（４７．８％ ），以眼睑下垂首发者多见（７７．４ ％ ），按改良Ｏｓｓｅｒｍａｎ 分型以Ⅰ型为主（６０ ．１％ ） ，危象发生率低（８．１％ ） ，激素中剂量冲击、小剂量维持疗法和胸腺切除术疗效好。结论 本组病例的临床资料与国外报道有较大差异，提示我国ＭＧ的临床特点有其独特之处，充分认识其临床特点将有助于临床的诊断和治疗。

Objective To study the clinical characteristics of myasthenia gravis(MG). Methods Retrospective study was done on 2 385 patients who were diagnosed at Tongji Hospital from 1956 to 1998. Results (1) There was little difference of morbidity of MG between the male and the female MG patients. The sex ratio was 1∶1.01 M∶F,but in the onset age group of 20～40 years old,there were more female patients than male ones,and the male ones were more common in the patients with their onset after 40 years old. (2)The average onset age was 17.7 years old, and the most common onset age group was between the youngest to nine years old. The morbidity decreased as the age of onset got older. (3)The extraocular muscles are most frequently involved as the first symptom of MG. According to the modified Osserman′s criteria, 1 434 patients (60.1%) were classified as groupⅠ. The patients in groupⅡincreased with the age of onset getting older. (4) Thymectomy was performed in 124 patients, only one thymoma was present in 85 cases and 4 malignant thymomas were found in 4 cases, 30 cases had thymic hyperplasia. (5) 194 patients(8.1%)had evident myasthenia crisis. The average age of the first crisis was 30.9 years old,and the mean interval from the onset of symptom to first crisis was 2.3 years. (6) With corticosteroids therapy of moderate dose impacting followed by small dose maintaining in 1 420 patients, initial exacerbations occurred in 5.9% of patients, remission in 29.5%, improvement in 59.5% and 11.0% remained unchanged. With the canonical perioperative phase management of thymectomy in 124 patients, remission occurred in 39.5%, improvement was seen in 41.9%, and the total mortality rate was 7.3%. Conclusion The clinical data in this group were evidently different from those reports from abroad. It suggested that MG in China had its distinct characteristics. The important information of the clinical manifestations available in this study may facilitate the diagnosis and therapy of MG in China.