先天性中枢性肺换气不足综合征患者对引起血管收缩的冷刺激的异常神经反应

先天性中枢性肺换气不足综合征患者对引起血管收缩的冷刺激
的异常神经反应
Macey P.M.;Macey K.E.;Woo M.A.;高蕊
【期刊名称】《世界核心医学期刊文摘：儿科学分册》
【年(卷),期】2005(000)008
【摘要】Patients with congenital central hypoventilation syndrome (CCHS), a condition characterized by impaired ventilatory responses to chemoreceptor stimulation, d o not show the normal increase in respiratory rate and respiratory- related hea rt rate variation to cold forehead stimulation, a challenge that bypasses centra l chemoreceptors. We hypothesized that a forehead cold pressor challenge would r eveal abnormal neural response patterns, as assessed by functional magnetic reso nance imaging, in brain regions that are responsible for the integration of cold afferent stimulation with respiratory and cardiovascular output in patients wit h CCHS. Primary sensory thalamic and cortical areas for the forehead showed dimi nished responses in 13 patients with CCHS (ventilator dependent during sleep but not waking, no Hirschsprung’s disease) compared with 14 control subjects, des pite initial signal changes in the cortex being similar in both groups. Cerebell ar cortex and deep nuclei; basal ganglia; and middle to posterior cingulate, ins ular, frontal, and temporal cortices showed reduced signal rises in patients wit h CCHS. Areas within the frontal and anterior cingulate cortices exhibited marke d
signal declines in control subjects but little change in patients with CCHS. N o response occurred in either group in the dorsal medulla, but medial and ventra l medullary areas showed enhanced signals in patients with CCHS. The cold presso r stimulation did not recruit dorsal medullary sites that would be affected by P HOX2B (a mutation of which is associated with the syndrome) expression in either group but demonstrated deficient cerebellar and medial medullary influences tha t, by action on rostral sites, may underlie the loss of respiratory responses.
【总页数】1页(P56-56)
【作者】Macey P.M.;Macey K.E.;Woo M.A.;高蕊
【作者单位】Department of Neurobiology Univ. of California at Los Angeles Los Angeles CA 90095- 1763 United States Dr.
【正文语种】中文
【中图分类】R748
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