Sanfield J A, Linares O A, Cahalan D D, Forrester J M, Halter J B, Rosen S G
Department of Internal Medicine, University of Michigan, Ann Arbor.
Arch Neurol. 1989 Jan;46(1):53-7. doi: 10.1001/archneur.1989.00520370055019.
We studied a 66-year-old woman with spontaneous periodic hypothermia (Shapiro's syndrome) to determine the mechanisms that result in increased plasma norepinephrine (NE) levels. In comparison with age-matched control subjects, compartmental analysis of NE kinetics revealed an increased NE release rate into the extravascular compartment and decreases in NE clearance and volume of distribution of NE in the intravascular compartment. Clonidine therapy was associated with an initial dramatic decrease in the frequency of diaphoretic episodes as well as with a fall in NE release rate and increases in NE clearance and volume of distribution. We conclude that increased NE release and decreased plasma NE clearance result in elevated plasma NE levels in Shapiro's syndrome. Clonidine, which was associated with changes in NE kinetics, may provide effective treatment for this disorder.
我们对一名患有自发性周期性体温过低（夏皮罗综合征）的66岁女性进行了研究，以确定导致血浆去甲肾上腺素（NE）水平升高的机制。与年龄匹配的对照受试者相比，对NE动力学进行的房室分析显示，NE向血管外间隙的释放速率增加，而血管内间隙中NE的清除率和分布容积降低。可乐定治疗与发汗发作频率最初显著降低相关，同时NE释放速率下降，NE清除率和分布容积增加。我们得出结论，NE释放增加和血浆NE清除率降低导致夏皮罗综合征患者血浆NE水平升高。与NE动力学变化相关的可乐定可能为这种疾病提供有效的治疗方法。
