Honda T
Brain Dev. 1984;6(1):27-32. doi: 10.1016/s0387-7604(84)80006-6.
Free amino acid patterns of cerebrospinal fluid in infants and children with various types of convulsive disorders were compared with those in age-matched normal subjects. The total free amino levels in Lennox syndrome were higher than the normal values, and those in infantile spasms controlled by ACTH were higher than those in uncontrolled infantile spasms. Although the levels of only one or two amino acids in tonic-clonic seizure, focal seizure and febrile seizure were higher or lower than those of the controls, the levels of 8 amino acids in infantile spasms were lower and those of 10 amino acids in Lennox syndrome were generally higher compared to the controls. Among amino acids in CSF of children with tonic-clonic seizure, infantile spasms or Lennox syndrome, only the ornithine level was commonly lower than that of the controls. After the treatment, in tonic-clonic seizure, the levels of taurine, asparagine and glycine were increased, and in infantile spasms, those of asparagine, glutamine, glycine, alanine, phenylalanine, lysine and arginine were increased while that of taurine was decreased. These results suggest that each type of convulsive disorder shows the specific amino acid pattern, and the effects of anticonvulsants may be partially understood through the changes of the free amino acid patterns in the brain.
将各类惊厥性疾病患儿脑脊液中的游离氨基酸模式与年龄匹配的正常受试者进行了比较。Lennox综合征中的游离氨基酸总量高于正常值,而促肾上腺皮质激素控制的婴儿痉挛症患儿的游离氨基酸总量高于未控制的婴儿痉挛症患儿。虽然强直阵挛性发作、局灶性发作和热性惊厥中仅一两种氨基酸的水平高于或低于对照组,但与对照组相比,婴儿痉挛症中有8种氨基酸水平较低,Lennox综合征中有10种氨基酸水平普遍较高。在强直阵挛性发作、婴儿痉挛症或Lennox综合征患儿的脑脊液氨基酸中,只有鸟氨酸水平通常低于对照组。治疗后,强直阵挛性发作中牛磺酸、天冬酰胺和甘氨酸水平升高;婴儿痉挛症中,天冬酰胺、谷氨酰胺、甘氨酸、丙氨酸、苯丙氨酸、赖氨酸和精氨酸水平升高,而牛磺酸水平降低。这些结果表明,每种惊厥性疾病都表现出特定的氨基酸模式,通过大脑中游离氨基酸模式的变化可以部分理解抗惊厥药物的作用。