Wolfson L I, Sharpless N S, Thal L J, Waltz J M, Shapiro K
Neurology. 1983 Mar;33(3):369-72. doi: 10.1212/wnl.33.3.369.
3-methoxy-4-hydroxyphenylglycol (MHPG), the primary brain metabolite of norepinephrine (NE), was measured in ventricular fluid from 51 patients with dystonia, other movement disorders, or hydrocephalus. The dystonic patients were divided into three categories: childhood-onset form with early limb dystonia and rapid progression to generalized symptoms, more localized and benign adult-onset dystonia, and symptomatic dystonia. Patients with the childhood form had significantly lower ventricular fluid MHPG levels (8.7 +/- 0.6 ng per milliliter) than other dystonic patients (11.4 +/- 1 ng per milliliter), age-matched controls with neurologic disease (11.7 +/- 1.1 ng per milliliter), or other movement disorders (11.8 +/- 0.7 ng per milliliter). Decreased ventricular fluid MHPG levels suggest a possible abnormality of brain NE function in childhood dystonia.
3-甲氧基-4-羟基苯乙二醇（MHPG）是去甲肾上腺素（NE）的主要脑代谢产物，对51例患有肌张力障碍、其他运动障碍或脑积水的患者的脑室液进行了检测。肌张力障碍患者分为三类：儿童期起病型，早期出现肢体肌张力障碍并迅速进展为全身性症状；更为局限且良性的成人起病型肌张力障碍；症状性肌张力障碍。儿童期起病型患者的脑室液MHPG水平（8.7±0.6纳克/毫升）显著低于其他肌张力障碍患者（11.4±1纳克/毫升）、年龄匹配的神经疾病对照组（11.7±1.1纳克/毫升）或其他运动障碍患者（11.8±0.7纳克/毫升）。脑室液MHPG水平降低提示儿童期肌张力障碍可能存在脑NE功能异常。
