Scoppetta C, Tonali P, Evoli A, David P, Crucitti F, Vaccario M L
J Neurol. 1979;222(1):11-21. doi: 10.1007/BF00313263.
In the treatment of myasthenia gravis (MG) considerable progress has recently been achieved. Our experience is based on the observation of 139 patients with an average follow-up of 3 years and 4 months. A treatment plan and results are presented. Indications for thymectomy: all cases of MG in adult life, apart from ocular myasthenia without radiological thymoma and without electrophysiological and pharmacological signs of generalization; before puberty only cases with radiological thymoma and severely incapacitating or life-threatening signs. Median sternotomy is preferable for thymoma, the transcervical approach with a sternal split for non-neoplastic thymus. Mediastinal radiotherapy is indicated after removal of an invasive or adhesive thymoma. Indications for corticosteroids: 1) before thymectomy: respiratory weakness; 2) soon after thymectomy: life-threatening signs; 3) later after thymectomy: incapacitating or life-threatening signs; 4) as an alternative to thymectomy: when surgery cannot be performed or it is not indicated. Oral Prednisone was nearly always preferred: alternate-day high single dose (75 to 115 mg) has given good results in most cases even if in some cases a small dose was required in the "off day"; inversely a lower alternate-day or daily dose was often sufficient. Long-term results: following this schedule for adult patients good results were scored in 67% of thymomas, in 94% of hyperplasias, and in 62% of unthymectomized patients: in prepuberal life the few cases of severe MG have all shown a favorable evolution.
在重症肌无力(MG)的治疗方面,最近已取得了相当大的进展。我们的经验基于对139例患者的观察,平均随访时间为3年零4个月。本文介绍了一种治疗方案及结果。胸腺切除术的指征:成年期所有MG病例,但不包括无放射学胸腺肿瘤、无电生理和药理学全身化迹象的眼肌型MG;青春期前仅适用于有放射学胸腺肿瘤以及有严重功能障碍或危及生命迹象的病例。对于胸腺肿瘤,正中胸骨切开术更为可取;对于非肿瘤性胸腺,采用胸骨劈开的经颈入路。对于侵袭性或粘连性胸腺肿瘤,在切除后需进行纵隔放疗。皮质类固醇的指征:1)胸腺切除术前:呼吸肌无力;2)胸腺切除术后不久:危及生命的体征;3)胸腺切除术后后期:功能障碍或危及生命的体征;4)作为胸腺切除术的替代方法:无法进行手术或不适合手术时。几乎总是优先选择口服泼尼松:隔日单次大剂量(75至115毫克)在大多数情况下都取得了良好效果,即使在某些情况下“非用药日”需要小剂量;相反,较低的隔日或每日剂量通常也足够。长期结果:按照该方案治疗,成年患者中,胸腺肿瘤患者的良好结果比例为67%,增生患者为94%,未进行胸腺切除术的患者为62%;在青春期前,少数重症MG病例均显示出良好的病情发展。