Markowa E D, Hausmanowa-Petrusewicz I, Alijewa L M, Friedman A, Insarowa N G
Neurol Neurochir Pol. 1981 Jul-Aug;15(4):403-6.
The authors report the results of treatment of hereditary extrapyramidal diseases with new preparations acting upon neurotransmitter systems. Patients with torsion dystonia, Huntington's chorea, Parkinson's disease, hereditary tremor, myoclonic epilepsy were followed-up for several years.. The best results in akinetic-rigidity syndromes (Parkinson's disease, rigid froms of torsion dystonia, Hallevorden-Spatz disease) were obtained with L-DOPA (including Sinemet, Nacom, Madopar) and in many patients these preparations were given in combination with other drugs (cholinolytic agents, Midantan) which contributed to compensation of the disturbed equilibrium of neurotransmitter systems and reduction of side effects. For decreasing the side effects of L-DOPA (hyperkineses of dystonic type, chorea and myoclonia) preparations from the group of phenothiazine and diazepine were given. In many cases improvement was achieved by slover increase of L-DOPA doses. In the hyperkinetic syndromes (Huntington's chorea, idiopathic tremor, myoclonic epilepsy, hyperkinetic torsion dystonia) preparations of phenothiazine, butyrophenone and new drugs active on the GABAergic system (Baclophen, Lyoresal, Pantogam) and diazepine (Clonazepam) were used. The analysis of the results shows that disturbed equilibrium of central neurotransmitters plays and important role in the pathogenesis of hereditary extrapyramidal system diseases.
作者报告了使用作用于神经递质系统的新制剂治疗遗传性锥体外系疾病的结果。对患有扭转性肌张力障碍、亨廷顿舞蹈病、帕金森病、遗传性震颤、肌阵挛性癫痫的患者进行了数年的随访。在运动不能-强直综合征（帕金森病、扭转性肌张力障碍的强直型、哈勒沃登-施帕茨病）中，左旋多巴（包括心宁美、纳康、美多芭）取得了最佳效果，并且在许多患者中，这些制剂与其他药物（抗胆碱能药物、咪坦坦）联合使用，有助于补偿神经递质系统的平衡紊乱并减少副作用。为了减轻左旋多巴的副作用（肌张力障碍型运动过多、舞蹈病和肌阵挛），给予了吩噻嗪类和二氮䓬类制剂。在许多情况下，通过缓慢增加左旋多巴剂量实现了病情改善。在运动过多综合征（亨廷顿舞蹈病、特发性震颤、肌阵挛性癫痫、运动过多性扭转性肌张力障碍）中，使用了吩噻嗪类、丁酰苯类以及对γ-氨基丁酸能系统有活性的新药（巴氯芬、氯苯氨丁酸、丙戊酰胺）和二氮䓬类（氯硝西泮）。结果分析表明，中枢神经递质的平衡紊乱在遗传性锥体外系疾病的发病机制中起重要作用。
