Jordan M K, Brunner R L, Hunt M M, Berry H K
Dev Med Child Neurol. 1985 Feb;27(1):33-9. doi: 10.1111/j.1469-8749.1985.tb04522.x.
Recent behavioral data have demonstrated the importance of maintaining low phenylalanine concentrations beyond early childhood in patients with phenylketonuria, which can be a difficult task, particularly during adolescence. Administration of certain large neutral amino-acids (valine, isoleucine, leucine--VIL) appears to reduce phenylalanine concentrations in the cerebrospinal fluid of humans and in the brain of rats. The present study compared neuropsychological test-performance of six patients with phenylketonuria during periods of VIL administration and periods when this supplement was not given. Although individual responses to VIL were variable, there was an over-all improvement of about 1 1/2 SD in neuropsychological test performance during VIL treatment. Abstract reasoning and tactile motor problem-solving increased more than pure motor performance.
近期行为学数据表明，对于苯丙酮尿症患者而言，在幼儿期之后维持低苯丙氨酸浓度至关重要，而这可能是一项艰巨的任务，尤其是在青春期。给予某些大中性氨基酸（缬氨酸、异亮氨酸、亮氨酸——VIL）似乎能降低人类脑脊液和大鼠大脑中的苯丙氨酸浓度。本研究比较了6例苯丙酮尿症患者在服用VIL期间和未服用该补充剂期间的神经心理测试表现。尽管个体对VIL的反应各不相同，但在VIL治疗期间，神经心理测试表现总体上提高了约1.5个标准差。抽象推理和触觉运动问题解决能力的提高幅度大于单纯的运动表现。
