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经治疗的苯丙酮尿症患者的神经心理功能

Neuropsychological functioning in treated phenylketonuria.

作者信息

Faust D, Libon D, Pueschel S

出版信息

Int J Psychiatry Med. 1986;16(2):169-77. doi: 10.2190/2209-39kw-bekl-w80j.

Abstract

Comprehensive neuropsychological assessment was performed on a series of patients with treated classical phenylketonuria. All patients showed a similar cluster of performance deficits in motor and visual-motor functions, spatial operations, academic skill areas, and select areas of higher level reasoning. The results are consistent with other neuropsychological studies of patients with early and late diet termination that have used differing assessment methods, thus allowing for greater confidence in the findings. Examination of underlying factors indicates that poor motor coordination, limits in the complexity of spatial problem solving, and limits in the amount of information that can be "processed" simultaneously may account for most or all of the performance deficits observed among these patients. Suggestions for future research and both clinical and theoretical implications of the findings are discussed.

摘要

对一系列接受过治疗的经典苯丙酮尿症患者进行了全面的神经心理学评估。所有患者在运动和视觉运动功能、空间操作、学术技能领域以及某些高级推理领域都表现出类似的一组表现缺陷。这些结果与其他针对饮食提前或延迟终止的患者进行的神经心理学研究一致,尽管那些研究使用了不同的评估方法,但这使得对这些发现更有信心。对潜在因素的检查表明,运动协调能力差、空间问题解决复杂性的限制以及同时“处理”信息数量的限制可能是这些患者中观察到的大部分或全部表现缺陷的原因。文中还讨论了对未来研究的建议以及这些发现的临床和理论意义。

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