Cognitive functions and the antioxidant system in phenylketonuric patients.

Abstract

The authors studied the relationship between the antioxidant system and cognitive functions in a group of 36 early and continuously treated phenylketonuric (PKU) patients (mean age=9.7 years) and 29 controls. The authors measured antioxidant cofactors and free radical damage markers in plasma (selenium, retinol, tocopherol, coenzyme Q10, malondialdehide) and antioxidant enzymes in red blood cells (glutathione peroxidase, catalase, superoxide dismutase). The authors used neuropsychological tests to screen for several cognitive functions. PKU patients showed significantly lower values of selenium, coenzyme Q10, and catalase, and significantly higher levels of malondialdehide. PKU patients showed a significantly negative correlation between plasma selenium concentrations and several Conner's Continuous Performance Test measures (more omission errors, fluctuating attention and inconsistency of response times, and slowing reaction time as the test progressed). Selenium deficiency was thus associated with a worsened performance on the Conner's Continuous Performance Test among PKU patients. In conclusion, it is important not only to control blood Phe levels in PKU but also other nutritional components such as selenium. Selenium status seems to be associated with attention functions in these PKU patients.