Information processing characteristics and uridine treatment in children with classical galactosemia

• Published in: Nutrition research (New York, N.Y.) N.Y.), 2002, Vol.22 (3), p.257-270
• Main Authors: Widhalm, Kurt, Miranda-da-Cruz, Beatriz, de Sonneville, Leo M.J
• Format: Article
• Language: English
• Subjects: Biological and medical sciences; brain; Children; cognition; Cognitive function; Galactosemia; General and cellular metabolism. Vitamins; Medical sciences; oral administration; patients; Pharmacology. Drug treatments; regression analysis; uridine; Uridine treatment
• ISSN: 0271-5317; 1879-0739
• DOI: 10.1016/S0271-5317(01)00401-8

We postulated that deficiencies of the white matter of the brain associated with galactosemia would result in cognitive slowing. Reaction time tasks were used to evaluate this outcome. It was also investigated whether uridine treatment would favorably affect cognitive function. Seven children (age 12.4 ± 1.3 years) with classical galactosemia (CG children) received an oral dosage of uridine at 150 mg/kg/d for 540 ± 3 days. IQ and reaction time data on neuropsychological function were collected before treatment (controls and CG children) and at the end of treatment (only in CG children). In addition to lower IQ scores, that indicated a reduced cognitive potential, the reaction time data revealed sustained and divided attention deficits. The regression analyses supported the hypothesis that there was cognitive slowing in patients with galactosemia was general, and substantial, with an almost 50% lower pace. The rate of slowing as well as IQ level did not change after 18 months of uridine treatment.