胸腺切除治疗重症肌无力远期疗效及影响因素的分析

Diagnostic and surgical experience of secondary syringohydromyelia

目的评价胸腺切除治疗重症肌无力远期疗效及影响因素。方法对１９９０年５月到１９９７年５月间１１４例重症肌无力患者进行了信访和门诊随访，疗效评价分为缓解、改善（以上二项称为有效）、无效和恶化。对年龄、性别、血浆乙酰胆碱受体（ＡｃｈＲ）抗体效价、病程、临床分型、随访期等因素进行统计分析。结果本组男４７例，女６７例，年龄４～７２岁。术后随访１０～８２个月（平均４２８个月）。缓解率２７２％，有效率６７５％，晚期病死率７０％（８例）。结论疗效评价需长期随访（＞５年）。患者性别、血浆乙酰胆碱受体（ＡｃｈＲ）抗体效价与疗效无关；病程大于５年的患者缓解率低；１６～３９岁、Ⅱ型、胸腺增生患者手术效果好。其它伴发疾病不影响疗效。

Objective To analyse the diagnostic and surgical experience in 17 patients with secondary syringohydromyelia. Methods Physical examination and MRI were carried out before operation. Surgical treatments were selected according to their different oringinal disorders. Results In three of 8 patients, intramedullary tumors were completely removed, 4 subtotally and 1 partially. Pathological examination showed maimly ependymoma. The tumor and syrinx on MRI were shown along the central axis of the spinal cord, but this was not the same on other 9 of the 17 patients with extramedullary disorders, which were treated for their oringinal disorders. The syrinx was shrunken in all 5 patients takeing MRI for countercheck. Conclusions The diagnosis of secondary syringohydromyelia mainlys depends on MRI. Surgical treatment should aim at their original disorders. Syringohydromyelia includes two pathological types: hydromyelia and syringomyelia. Those secondary to intramedullary ependymoma are mostly hydromyelia, which may be caused by expanding of tumor along the central canal of the spinal cord.