Shimizu T, Hayashi H, Hayashi M, Kato S, Tanabe H
Department of Neurology, Tokyo Metropolitan Neurological Hospital, Japan.
Clin Auton Res. 1995 Jun;5(3):165-9.
Intravenous norepinephrine infusion test was performed in eight patients with amyotrophic lateral sclerosis (ALS) supported by respirators and nine control subjects, to examine alpha-adrenoceptor function of peripheral resistant blood vessels. Baseline plasma norepinephrine concentrations in ALS patients were significantly higher than those in control subjects, indicating basal sympathetic hyperactivity (normal 218.2 +/- 59.7 pg/ml; ALS 450.0 +/- 288.4 pg/ml). The stimulus-response curves in the patients were similar to those in control subjects, and there were no significant differences between mean gains of the stimulus-response curves in both groups (normal 18.7 +/- 5.5; ALS 15.2 +/- 11.2). However, three ALS patients, two of whom had circulatory fluctuation and sympathetic hyperactivity, revealed lower gain levels than the mean minus 2 SD in control subjects (4.7, 1.1 and 3.7). This indicates hyposensitivity or down-regulation of the alpha-adrenoceptor function of peripheral blood vessels in these ALS patients. For early detection of sympathetic hyperactivity and prediction of circulatory collapse, it would be useful to measure the plasma norepinephrine concentration and the gain of the norepinephrine infusion curve in respirator-dependent ALS patients.
对8例依靠呼吸机维持的肌萎缩侧索硬化症（ALS）患者和9例对照者进行了静脉注射去甲肾上腺素输注试验，以检测外周阻力血管的α-肾上腺素能受体功能。ALS患者的基线血浆去甲肾上腺素浓度显著高于对照者，表明基础交感神经功能亢进（正常为218.2±59.7 pg/ml；ALS为450.0±288.4 pg/ml）。患者的刺激-反应曲线与对照者相似，两组刺激-反应曲线的平均增益无显著差异（正常为18.7±5.5；ALS为15.2±11.2）。然而，3例ALS患者，其中2例有循环波动和交感神经功能亢进，其增益水平低于对照者平均减2个标准差（4.7、1.1和3.7）。这表明这些ALS患者外周血管的α-肾上腺素能受体功能存在低敏或下调。对于早期检测交感神经功能亢进和预测循环衰竭，检测依赖呼吸机的ALS患者的血浆去甲肾上腺素浓度和去甲肾上腺素输注曲线的增益将是有用的。
