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Regression of neuropsychological deficits in early‐treated phenylketonurics during adolescence
Author(s) -
Weglage J.,
Pietsche M.,
Denecke J.,
Sprinz A.,
Feldman R.,
Grenzebach M.,
Ullrich K.
Publication year - 1999
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1023/a:1005587915468
Subject(s) - stroop effect , neuropsychology , medicine , intelligence quotient , phenylalanine , pediatrics , cognition , psychology , psychiatry , biology , biochemistry , amino acid
Even early‐treated phenylketonuric patients suffer from phenylalanine‐associated (mild) neuropsychological impairment. To date it is still unclear whether patients' deficits show a progression on ageing. This unsolved question seems to be an important aspect in the still ongoing debate about how long and how strictly the patients should be maintained on diet. Twenty early‐treated (20±10, 9–30 days) adolescent phenylketonurics (10 boys, 10 girls) and 20 healthy controls, matched for age, sex and IQ, were inves‐tigated twice at a mean ages of 11 and 14 years for their IQ (Culture Fair Intelligence Test–Scale 2; CFT‐20), fine motor abilities (Motor PerformanceTask), sustained (Test d 2 ) and selective attention (Stroop‐Task). At the first test, examinations revealed significant blood phenylalanine‐correlated neuropsychological deficits in PKU patients. In spite of raised blood phenylalanine concentrations during the following 3 years and significantly elevated concurrent blood phenylalanine concentrations, the repeated measurements revealed a significant decrease of patients' deficits compared to controls. Clinical‐neurological status of patients and controls was normal at both test times. The results indicate a decreased vulnerability of PKU‐patients with respect to their neuropsychological functioning against elevated blood phenylalanine levels on ageing.