Decreased hypoxic ventilatory chemoresponsiveness and clinical features in patients with narcolepsy cataplexy

目的 探讨猝倒型发作性睡病患者呼吸中枢低氧反应性与临床表现的相关性.方法 选择2007年6月至2008年5月北京大学人民医院睡眠中心就诊的伴猝倒且人类白细胞组织相容性抗原(HLA) DQB1* 0602阳性的113例典型发作性睡病患者(发作性睡病组)和128名年龄和性别匹配的健康志愿者(对照组)作为研究对象.分别测定其HLA-DQB1*0602状态、高CO2反应性[分钟通气量(VE)降低量与CO2分压(PCO2)下降量的比值]和低O2反应性[VE降低量与脉氧饱和度(SpO2)下降率的比值].其中93例嗜睡严重的患者在应用盐酸哌甲酯治疗嗜睡、另20例猝倒严重患者应用盐酸氯咪帕明治疗猝倒后分别重复测定了高CO2和低O2反应性.结果 发作性睡病组对低氧的反应性显著低于对照组[(-0.135±0.105)比(-0.223±0.136)L·min-1·％SpO2-1,P＜0.001].经盐酸哌甲酯治疗的93例患者白天嗜睡显著改善,但治疗前后低氧反应性无明显变化[(-0.151±0.111)比(-0.149±0.105)L·min-1·％ SpO2-1,P=0.780];经盐酸氯咪帕明治疗的20例患者猝倒明显改善,但治疗前后低氧反应性亦无明显变化[(-0.114±0.054)比(-0.115±0.065)L·min-1·％SpO2-1,P=0.949].结论 发作性睡病患者呼吸中枢低氧反应性的降低与其临床表现无明显相关.To explore the relationship between hypoxic responsiveness of the patients with narcolepsy-cataplexy and their clinical features.A total of 113 patients with narcolepsy-cataplexy (narcolepsy group) at Peking University People's Hospital from June 2007 to May 2008 and 128 gender-age matched volunteers (control group) were recruited. And their status of human leukocyte antigen (HLA)-DQB1*0602 was examined to differentiate hypercapnic and hypoxic responsiveness. Among them, 93 patients with severe hypersomnolence had hypercapnic and hypoxic responsiveness tested before and after the treatment of methylphenidate and another 20 with severe cataplexy did the same before and after the treatment of chlorimiopramine.Compared with the control group, the narcolepsy group had depressed hypoxic responsiveness ((-0.135 ± 0.105) vs (-0.223 ± 0.136) L×min?1 × %SpO??1, P < 0.001). After the treatment of methylphenidate, sleepiness improved significantly in all 93 patients, but their low hypoxic responsiveness did not change ((-0.151 ± 0.111) vs (-0.149 ± 0.105) L×min?1× % SpO??1, P = 0.780). After the treatment of chlorimiopramine, cataplexy also improved in 20 patients.However their low hypoxic responsiveness had no change ((-0.114 ± 0.054) vs (-0.115 ± 0.065) L×min?1 × %SpO??1, P = 0.949).Lower hypoxic responsiveness in narcolepsy group is not related with the clinical features of disease.教育部高等学校博士学科点专项科研基金; 首都特色临床医学应用发展项目PubMed中文核心期刊要目总览(PKU)中国科技核心期刊(ISTIC)中国科学引文数据库(CSCD)0241870-18739