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对死于遗传性舞蹈症病人进行尸检,其脑内有的部位γ氨基丁酸显著减少。本病可能由于正常利用γ氨基丁酸作为抑制性神经介质的神经原的损失。遗传性舞蹈症的很多症状可能是γ氨基丁酸和其它神经介质之间的严重不平衡所致,因此,增加脑内γ氨基丁酸水平对本病可能有缓解作用。异烟肼的作用是γ氨基丁酸氨基转移酶的抑制剂,它是γ氨基丁酸降解过程中的第一个酶。应用异烟肼的量大于治疗人结核病的量时,能提高动物脑内γ氨基丁酸含量。作者应用异烟肼治疗遗传性舞蹈症,获得初步令人鼓舞的效果。对诊断明确的6例遗传性舞蹈症病人进行了异烟肼治疗。每日每公斤体重10～21毫克(加维生素B_6
An autopsy of a patient who dies from hereditary chorea shows a significant reduction of gamma-aminobutyric acid in some areas of the brain. The disease may be due to the normal use of gamma-aminobutyric acid as an inhibitory neurotrophic neuron loss. Many of the symptoms of genetic chorea may be due to a serious imbalance between gamma-aminobutyric acid and other neurotransmitters and therefore increasing gamma-aminobutyric acid levels in the brain may have a beneficial effect on the disease. Isoniazid acts as an inhibitor of γ-aminobutyrate aminotransferase, the first enzyme in the degradation of γ-aminobutyric acid. Application of isoniazid is greater than the amount of treatment of human tuberculosis, the brain can increase the content of γ aminobutyric acid. The authors apply isoniazid for the treatment of inherited chorea with initial encouraging results. 6 cases of diagnosed idiopathic chorea were treated with isoniazid. Daily weight per kilogram of 10 to 21 mg (plus vitamin B_6