Total choline intake and working memory performance in adults with phenylketonuria

Area of Science:

• Neuroscience
• Nutritional Science
• Metabolic Disorders

Background:

• Phenylketonuria (PKU) often leads to neurological deficits and executive function impairments, even with dietary management.
• The role of choline intake in these cognitive deficits in PKU has not been previously studied.
• Suboptimal choline levels are a potential contributing factor to cognitive impairments in PKU.

Purpose of the Study:

• To investigate the correlation between choline intake and working memory performance in adults with PKU.
• To determine if this relationship is influenced by PKU diagnosis and metabolic control.
• To assess the impact of choline on cognitive function in individuals with PKU.

Main Methods:

• A cross-sectional study involving 40 adults with PKU and 40 healthy controls.
• Web-based neurocognitive tests assessed working memory.
• Three-day dietary records evaluated nutrient intake, including choline.
• Recent and historical blood phenylalanine (Phe) levels measured metabolic control.

Main Results:

• Individuals with PKU showed lower working memory performance compared to controls, irrespective of choline intake.
• In well-controlled PKU subgroup (Phe ≤ 360 µmol/L), higher choline intake correlated with improved working memory.
• This positive association between choline and working memory was observed after adjusting for intellectual ability and historical Phe levels.

Conclusions:

• Choline intake is crucial for individuals with PKU, particularly those with good metabolic control, for maintaining working memory.
• Clinical monitoring of choline levels is recommended for all PKU patients.
• Further validation in larger, controlled trials is needed to confirm these findings in the PKU population.