Mel'nichuk P V, Khokhlov A P, Khaĭlova I M
Zh Nevropatol Psikhiatr Im S S Korsakova. 1979;79(10):1324-8.
Pheylalanine metabolism was studied in 56 children with various forms of hyperkinesias. It was found that in the development of slow and fast hyperkinesias a certain role belongs to dihydroxyphenylalanine (DOPA). It is probable, that in patients with Turett's syndrome the synthesis of DOPA is increased while in patients the excretion of phenylacetylglutamine was found to be disturbed: it was decreased in the patients with the fast and increased in the children with the slow hyperkinesias. Phenylalanine load led to a lowering of the DOPA level in the patients with Turett's syndrome; an intensification of the synthesis of phenylacetylglutamine and diminution of the intensity of hyperkinesias. L-glutamine load resulted in detoxication of the toxic phenylalanine metabolites which inhibited the DOPA synthesis, as well as in a short-time increase in the phenylacetylglutamine excretion and a moderation of tonic hyperkinesias that manifested by athetosis and dystonia. All this points out that in the development of various forms of hyperkinesias a certain role belongs to amino acid metabolites.
对56名患有各种形式多动症的儿童的苯丙氨酸代谢进行了研究。结果发现,在缓慢型和快速型多动症的发展过程中,二羟基苯丙氨酸(多巴)起到了一定作用。在图雷特综合征患者中,多巴的合成可能增加,而在患者中发现苯乙酰谷氨酰胺的排泄受到干扰:在快速型患者中其排泄减少,而在缓慢型多动症儿童中其排泄增加。苯丙氨酸负荷导致图雷特综合征患者的多巴水平降低;苯乙酰谷氨酰胺的合成增强,多动症的强度减弱。L-谷氨酰胺负荷导致抑制多巴合成的有毒苯丙氨酸代谢产物解毒,以及苯乙酰谷氨酰胺排泄短期内增加,以手足徐动症和肌张力障碍表现的强直性多动症得到缓解。所有这些都表明,在各种形式多动症的发展过程中,氨基酸代谢产物起到了一定作用。