A neuropsychological profile of off-diet adults with phenylketonuria

J Clin Exp Neuropsychol. 2007 May;29(4):436-41. doi: 10.1080/13803390600745829.

Abstract

The metabolic disorder phenylketonuria (PKU) is treated early by a low-phenylalanine diet. While this prevents global cognitive impairment, some patients still experience cognitive and neurophysiological abnormalities. Neuropsychological testing of early treated, currently off-diet, PKU patients attending an adult PKU clinic showed a reduction in the Perceptual Organization Index (POI), Processing Speed Index (PSI) from the Wechsler Adult Intelligence Scale Third Edition (WAIS-III), and Part A of the Trail Making Test for the PKU group relative to controls. Taken together, these results supported a profile of reduced information-processing speed.

Publication types

  • Clinical Trial

MeSH terms

  • Adult
  • Case-Control Studies
  • Female
  • Humans
  • Male
  • Neuropsychological Tests*
  • Phenylalanine / administration & dosage
  • Phenylketonurias / diet therapy*
  • Phenylketonurias / physiopathology*
  • Problem Solving / physiology
  • Wechsler Scales

Substances

  • Phenylalanine