Nyberg-Hansen R, Gjerstad L
Department of Neurology, University of Oslo, Norway.
Acta Neurol Scand. 1988 Apr;77(4):307-13. doi: 10.1111/j.1600-0404.1988.tb05914.x.
Six patients (4 females, 2 males) with generalized myasthenia gravis (MG) have been treated in an open study with ciclosporin (5-10 mg/kg/day), originally for 28 weeks. The age of the patients ranged from 16-61 years and the duration of symptoms from 5-30 years. Four of the patients had been thymectomized. Ciclosporin was added to prednisolone in 5 of the patients. Three of these had in addition previously used azathioprine without satisfactory improvement. The efficacy was measured by scoring the degree of muscle weakness on clinical examination and by scoring of the patients' personal assessment of their fatigability and muscle strength. In 5 patients there was a moderate to marked improvement in muscle strength and fatigueability on ciclosporin therapy. In one patient the effect was slight to moderate. The degree of improvement was similar in the one patient who received ciclosporin alone and in those also using prednisolone. The levels of anti ACh-r antibodies decreased in 3 patients, increased in 2, and were unchanged in one patient. Side effects known to occur on ciclosporin therapy were noticed, but they were tolerable in all patients. An increase in serum creatinine (mean 10%) and decrease in creatinine clearance (mean 21%) were seen in all patients. The treatment with ciclosporin has been continued in all 6 patients, but the dosage has been reduced to 5 mg/kg/day. Deterioration 3-4 weeks after discontinuation of ciclosporin was seen in one case, but resumption of therapy resulted in improvement as before. The total treatment period is now 24-34 months (March, 1987).(ABSTRACT TRUNCATED AT 250 WORDS)
6例全身性重症肌无力(MG)患者(4例女性,2例男性)接受了环孢素(5-10毫克/千克/天)的开放性研究治疗,最初为期28周。患者年龄在16至61岁之间,症状持续时间为5至30年。其中4例患者已接受胸腺切除术。5例患者在泼尼松龙基础上加用了环孢素。其中3例患者此前还使用过硫唑嘌呤,但病情改善不明显。通过临床检查对肌肉无力程度进行评分以及患者对自身疲劳感和肌肉力量的自我评估评分来衡量疗效。5例患者在接受环孢素治疗后肌肉力量和疲劳感有中度至明显改善。1例患者效果为轻度至中度。单独接受环孢素治疗的1例患者与同时使用泼尼松龙的患者改善程度相似。3例患者抗乙酰胆碱受体(ACh-r)抗体水平下降,2例上升,1例无变化。观察到环孢素治疗已知会出现的副作用,但所有患者均可耐受。所有患者血清肌酐均升高(平均10%),肌酐清除率均下降(平均21%)。所有6例患者均继续接受环孢素治疗,但剂量已减至5毫克/千克/天。1例患者在停用环孢素3-4周后病情恶化,但恢复治疗后如前改善。目前总治疗期为24至34个月(1987年3月)。(摘要截选至250字)