线粒体脑肌病伴高乳酸血症和卒中样发作患者的血管自主调节功能  

Evaluation of vascular autoregulation in mitochondrial myopathy, encephalopathy, lactic acidosis,and stroke-like episodes patients

作　　者：陈靖[1] 赵丹华[1] 王朝霞[1] 彭清[1] 袁云[1] 黄一宁[1] 

机构地区：[1]北京大学第一医院神经内科,100034

出　　处：《中华神经科杂志》2012年第4期264-267,共4页Chinese Journal of Neurology

基　　金：国家自然科学基金资助项目（30870864）

摘　　要：目的评价线粒体脑肌病伴高乳酸血症和卒中样发作（MELAS）患者的脑血管二氧化碳（CO2）反应性和血管内皮功能，以分析患者在卒中样发作缓解期的血管自主调节功能变化。方法以经基因诊断证实的29例MELAS为患者组，检查时间距离末次卒中样发作的时间大于2周；健康志愿者28名为对照组。应用颈部血管彩超和头颅磁共振血管成像（MRA）评价颈部及颅内大血管形态。血管自主调节功能检测包括：①应用经颅多普勒超声进行呼吸抑制试验，计算呼吸抑制指数（BHI），评价脑血管CO2反应性；②肱动脉法评价内皮依赖性血管舒张功能（FMD）和非内皮依赖性血管舒张功能。对两组结果进行独立样本t检验。结果MELAS患者和健康志愿者的颈部血管超声及头颅MRA检查均未见异常。MELAS组的BHI（1．36±0．52）、FMD（11．0％±4．8％）均低于健康对照组（1．81±0．26，t=-3．693，P〈0．01；15．8％±5．8％，t=-3．390，P〈0．01）。结论MELAS患者存在脑血管CO，反应性受损及血管内皮功能障碍。Objective To investigate the cerebral vascular autoregulation in patients with mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes （MELAS） during the remission of stroke-like episodes, including cercbrovascular CO2 reactivity and vascular endothelial function. Methods Twenty-nine MELAS patients confirmed by genetic analysis were recruited in this study. They underwent the examination at least 2 weeks after the onset of last stroke-like episode. Twenty-eight healthy people were collected as healthy controls. Carotid ultrasound and brain magnetic resonance angiogram （MRA） were done to evaluate the cervical and intracranial appearance of large arteries. Evaluation of vascular autoregulation included： （1） the cerebrovascular CO2 reactivity with breath-holding test by transcranial Doppler and calculating breath holding index （ BHI）, and （2） flow-mediated dilation （ FMD ） and nitroglycerin-mediated dilation with ultrasound assessment of humeral artery. Independent-samples t test was done between the results of two groups. Results Carotid ultrasound and cranial MRA revealed no abnormalities in both MELAS patients and healthy controls. The BHI of MELAS patients was significantly decreased than that of normal controls （ 1.36 ＋ O. 52 vs 1.81 -＋ O. 26, t = - 3. 693, P 〈 O. O1 ）, and the FMD of MELAS patients was also significantly lower than that of normal controls （11.0% -＋ 4. 8% vs 15.8% ＋ 5.8%, t = -3. 390,P 〈 O. 01 ）. Conclusion The function of vascular autoregulation, including cerebrovascular CO2 reactivity and FMD, is impaired in MELAS patients.

关 键 词：MELAS综合征 二氧化碳 内皮 血管 血管舒张 脑 局部血流 

分 类 号：R746[医药卫生—神经病学与精神病学]