延髓病变继发Ondine’s curse综合征临床分析

Clinical analysis of ondine's curse syndrome secondary to medulla oblongata lesions

目的探讨延髓病变继发Ondine’s curse综合征的临床特点、治疗及预后,以提高对本病的认识。方法收集5 y来我院临床证实的延髓病变250例,其中双侧延髓病变8例作为研究组,8例中6例继发Ondine’s curse综合征,单侧延髓病变242例作为对照组,无1例继发Ondine’s curse综合征,对两组临床特点,影像学资料及诊治预后进行回顾性分析。结果研究组与对照组在继发Ondine’s curse综合征的发生率及预后方面比较差异有统计学意义(P<0.01);研究组6例继发Ondine’s curse综合征中2例治愈,其中1例通过抢救,症状恢复,影像学病灶可逆;另1例通过逐渐延长停机间歇时间,最终脱机,恢复正常呼吸;其它4例中2例自动出院,2例死亡。结论双侧延髓病变比单侧延髓病变更易继发Ondine s curse综合征,一旦出现则病情重预后差,应提高警惕,及时诊治;单侧延髓病变继发Ondine s curse综合征相对较少,预后相对较好。

Objective To investigate clinical characteristic,treatment and prognosis of ondine＇s curse syndrome secondary to medulla oblongata lesions. Methods 250 patients of medulla oblongata lesions were collected in our hospital in 5 years. Among them,8cases with bilateral medulla oblongata lesions were studied group and 6cases of them were complicated with ondine＇s curse syndrome. 242 cases with unilateral lesions were control group,and none of them was with ondine＇s curse syndrome. Clinical manifestations,imaging tests,treatment and prognosis were analyzed retrospectively. Results There was significant statistical difference between studied and control group in incidence of ondine＇s curse syndrome and prognosis（ P〈 0. 01） 2 of the 6 cases with ondine＇s curse in studied group were cured Between them one recovered by timely rescue,whose imaging changes were reversible,and one regained normal respiration after free from resisted ventilation by gradually prolonging the time of infermission. Among the other 4cases,2left hospital automatically and two died. Conclusion Compared to the unilateral lesions,patients with bilateral medulla oblongata lesions are more easily to be suffered from Ondine＇s curse syndrome,whose condition is more serious and prognosis is poor. Altogether we highlight that more emphasis must be put on this type of ondine＇s curse syndrome and timely diagnosis and effective treatment are of great necessity.