眼肌型重症肌无力干预后状态

Postintervention status in ocular myasthenia gravis： effects of treatment

目的评估溴吡斯的明、激素、胸腺切除、胸腺切除-激素联合4种治疗对于以眼肌型重症肌无力（OMG）起病的患者干预后状态的差异。方法对180例OMG患者进行回顾加前瞻性临床研究，患者起病年龄≥15岁，根据患者接受的治疗非随机性入组，分为手术组60例、激素组39例、联合组31例、对症组50例。按照美国重症肌无力基金会标准以干预后状态仅余“轻微异常”为理想状态。结果①激素组治疗后3～6个月，睑下垂、眼肌麻痹、总体理想状态率相对高于其他3组，其中，42．1％（16／38）的患者治疗3个月睑下垂达到理想状态，高于对症组（7／48，14．6％，x^2=8．200，P=0．004）。②联合组、手术组从治疗后1年起，各项理想状态率呈上升趋势，以联合组略高于手术组；观察期终末21．7％（13／60）手术组患者达到完全缓解；纵向配对比较，手术组治疗2年后睑下垂（22／40，55．0％）、眼肌麻痹（16／27，59．3％）和总体效果（20／40，50．0％）达理想状态率均显著高于治疗3个月后[11／59，18．6％；11／44，25．0％；9／60，15．0％；P=0．002、0．031、0．000（此处无统计值，仅有P值）]。③选择对症治疗的患者平均起病年龄（51．9±18．0）岁，高于其他3组（F=10．563，P=0．000）。④伴有眼肌麻痹的OMG患者更倾向于选择激素或联合治疗，联合组眼肌麻痹率高于对症、手术2组（x^2=12．939、14．380，均P=0．000），激素组眼肌麻痹率高于手术组（x^2=8．017，P=0．005）。结论对于以OMG起病的青中年患者，激素治疗起效快，胸腺切除、联合治疗长期疗效好，激素甚或联合治疗可能是伴有眼肌麻痹者较好的选择。

Objective To assess the differences of short- and long-term postintervention status on ocular and systemic symptoms for patients with ocular myasthenia gravis （OMG） after pyridostigmine bromide, corticosteroid, thymectomy, or thymectomy-corticosteroid combination therapy （ combination ）. Methods This retrospective plus prospective study included 180 OMG patients, whose age of onset ≥ 15 years, treated non-randomly with above therapies separately： thymectomy group （60 cases ） , corticosteroid group （39 cases）, combination group （ 31 cases ）, symptomatic group （ 50 cases ）. Postintervention status complying with Myasthenia Gravis Foundation of America （MGFA） ＂ complete stable remission ＂, ＂pharmacologic remission＂, or ＂minimal manifestations＂ was considered as desirable response, which was used as statistical indicator. Results （1）Corticosteroid group showed higher desirable response rates on ptosis, ophthalmoplegia and general weakness at 3-6 months after treatment than other groups, and 42. 1% （ 16/38 ） of them at 3 months achieved the desired state of ptosis, higher than the symptomatic group （7/48, 14. 6% ,x^2 = 8. 200, P = 0. 004）. （2）Ascending ideal rates had been presented in both combination and thymectomy groups since 1 year after treatments, while a little bit higher rate was presented in the former. At the end of observation, 21.7% （13/60）of patients in thymectomy group achieved complete stable remission. By paired longitudinal comparisons, thymectomy group showed higher ideal rates on ptosis （22/40, 55.0% ）, ophthalmoplegia （ 16/27,59. 3% ） and general weakness （20/40,50. 0% ） at 2 years than that at 3 months （11/59,18.6% ;11/44,25.0% ;9/60,15.0% ;P = 0. 002, 0. 031, 0. 000）. （3）For those patients by symptomatic treatment, the average age of onset was （51.9 ± 18.0） years, higher than that by other 3 therapies （F = 10. 563 ,P = 0. 000）. （4）OMG patients with ophthalmoplegia more likely select corticosteroid or combined therapy. Ophthalmoplegia in combination group was higher than that in symptomatic and surgery groups（ x^2 = 12. 939,14. 380, P = 0. 000 in both）. Ophthalmoplegia in corticosteroid group was higher than that in surgery group （x^ 2= 8. 017, P = 0. 005 ）. Conclusions Corticosteroid appears to early overcome ptosis, ocular motor dysfunction and general weakness for patient with OMG in early-to-middle adulthood. Thymectomy and surgery-corticosteroid combination therapies both show long-term effect on them. Corticosteroid or combination therapy may be the better choice for those OMG patients with ophthalmoplegia.