Plaitakis A, Berl S
J Neural Transm Suppl. 1983;19:65-74.
Altered metabolism of neuroexcitatory amino acids has been described in patients with a form of olivopontocerebellar atrophy (OPCA) associated with glutamate dehydrogenase (GDH) deficiency. To further investigate the specificity of these results, oral glutamate loading tests were performed in healthy controls, patients with GDH deficient OPCA as well as patients with non-GDH deficient degenerative disorders affecting primarily the function of the cerebellum and/or the basal ganglia. Following oral intake of monosodium glutamate, plasma levels of glutamate, aspartate and taurine increased significantly in controls and similar increases also occurred in patients with non-GDH deficient disorders. However, patients with GDH-deficient OPCA showed much greater elevations in plasma glutamate and aspartate and a rather flat taurine curve.
在患有与谷氨酸脱氢酶（GDH）缺乏相关的橄榄体脑桥小脑萎缩（OPCA）的患者中，已发现神经兴奋性氨基酸的代谢发生改变。为了进一步研究这些结果的特异性，对健康对照者、GDH缺乏的OPCA患者以及主要影响小脑和/或基底神经节功能的非GDH缺乏的退行性疾病患者进行了口服谷氨酸负荷试验。口服谷氨酸钠后，对照组血浆谷氨酸、天冬氨酸和牛磺酸水平显著升高，非GDH缺乏疾病患者也出现类似升高。然而，GDH缺乏的OPCA患者血浆谷氨酸和天冬氨酸升高幅度更大，牛磺酸曲线较为平缓。
