Barchatowa W P, Milewska D, Wehr H
Neurol Neurochir Pol. 1981 Jul-Aug;15(4):381-6.
The authors describe the results of determinations of the main metabolites of dopamine (DA), serotonin (S) and noradrenaline (NA) in the cerebrospinal fluid (ventricular and lumbar) in patients with various extrapyramidal system diseases. A profound decrease was demonstrated in the concentration of homovanillic acid (HVA)--the end metabolite of DA in parkinsonism, reflecting damage to DA--containing pathways and reduced DA synthesis in basal ganglia. Treatment with L-DOPA raises considerably the HVA level in the cerebrospinal fluid evidencing increased DA metabolism in the brain during administration of its precursor L-DOPA. In torsion dystrophy a statistically significant difference was found in HVA concentrations in the ventricular fluid depending on the clinical manifestations of the disease. In the patients with local muscular rigidity HVA level was much lower than in patients with the hyperkinetic form of the disease. It is concluded that the character of changes in the cerebral dopaminergic systems differs phenotypically in the form of torsion dystonia. In hepatolenticular degeneration (Wilson's disease) the level of all studied metabolites was decreased, which could be an evidence of deficient cerebral metabolism of their precursors--amines. In cases of Huntington's chorea a low level of HVA was found in the ventricular fluid, reflecting decreased total amount of DA in the brain due to damage to the corresponding neurons. Absence of detectable changes in MHPG concentration (the main cerebral metabolite of NA) indicates that this amine plays a lower role than DA and S in the biochemical mechanisms of the pathogenesis of extrapyramidal motor disturbances. The obtained data are important for the understanding of the pathogenesis and for evolving therapeutic methods in extrapyramidal diseases.
作者描述了对患有各种锥体外系疾病患者脑脊液（脑室和腰椎穿刺）中多巴胺（DA）、5-羟色胺（S）和去甲肾上腺素（NA）主要代谢产物的测定结果。在帕金森病中，DA的终末代谢产物高香草酸（HVA）浓度显著降低，这反映了含DA通路的损伤以及基底神经节中DA合成的减少。左旋多巴治疗可使脑脊液中HVA水平显著升高，这表明在给予其前体左旋多巴时，大脑中DA代谢增加。在扭转性肌张力障碍中，根据疾病的临床表现，发现脑室液中HVA浓度存在统计学上的显著差异。局部肌肉僵硬的患者HVA水平远低于疾病多动型患者。得出的结论是，扭转性肌张力障碍形式的大脑多巴胺能系统变化特征在表型上有所不同。在肝豆状核变性（威尔逊病）中，所有研究代谢产物的水平均降低，这可能证明其前体——胺类在大脑中的代谢不足。在亨廷顿舞蹈病病例中，脑室液中发现HVA水平较低，这反映了由于相应神经元受损，大脑中DA总量减少。未检测到3-甲氧基-4-羟基苯乙二醇（MHPG，NA的主要脑代谢产物）浓度的变化，这表明在锥体外系运动障碍发病机制的生化机制中，这种胺类的作用低于DA和S。所获得的数据对于理解锥体外系疾病的发病机制以及制定治疗方法具有重要意义。
