脑干梗死继发Ondine's curse综合征七例临床分析

Ondine's Curse Syndrome Secondary to Brainstem Infarction: Clinical Analysis of Seven Cases

目的探讨继发Ondine's curse综合征的脑干梗死患者的临床和影像学特点。方法回顾性分析近4年在我科住院的7例发生Ondine's curse综合征的脑干梗死患者的临床和影像学资料。结果本组患者脑梗死均累及延髓外侧部,均在病程中出现Ondine's curse综合征,发生时间为病后4 h 20 min至9 d。其中2例抢救无效死亡;4例经立即机械通气意识恢复,且神经系统体征较呼吸停止前无变化;1例继发缺氧缺血性脑病。2例在出现Ondine's curse综合征前有神经功能缺损的加重,影像学证实原有延髓外侧梗死面积扩大或出现新发延髓外侧梗死;余5例未见明显神经功能缺损加重。结论对于延髓外侧梗死的患者除了注重观察临床体征的变化外,还应严密监测血氧,甚至进行长时间监测,特别在患者入睡后,以防止Ondine's curse综合征的发生而导致患者死亡。

Objective To investigate the clinical and imaging characteristics of Ondine＇s curse syndrome secondary to brainstem infarction. Methods The clinical and imaging data of 7 patients with Ondine＇s curse syndrome secondary to brainstem infarctions were analyzed retrospectively. Results Lateral medulla oblongata were all involved among the 7 cases. All the 7 pa- tients developed Ondine＇s curse syndrome during the course of their diseases, from 4 hours and 20 minutes to 9 days after on- set. Of which 2 patients died; 4 survived after emergent intubation and mechanical ventilation, with no new neurological signs; and 1 was complicated with secondary hypoxic ischemic encephatopathy. Before occurrence of Ondine＇s curse 2 patients presented neurologic impairment deterioration, with enlargement of the former lateral medullary infarction or new infarction in the lateral me- dulla oblongata proved by MRI; However, the other 5 had no neurologic impairment deterioration. Conclusion The patients with lateral medullary infarction, besides close observation of changes of clinical signs, should be given closely monitoring of blood oxygen index, especially during their sleep, so as to prevent their death caused by Ondine＇s curse syndrome.