Premium
Neuropsychological effects of subsequent exposure to phenylalanine in adolescents and young adults with eariy‐treated phenylketonuria
Author(s) -
Griffiths P.,
Paterson L.,
Harvie A.
Publication year - 1995
Publication title -
journal of intellectual disability research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.941
H-Index - 104
eISSN - 1365-2788
pISSN - 0964-2633
DOI - 10.1111/j.1365-2788.1995.tb00540.x
Subject(s) - neuropsychology , pediatrics , welsh , cognition , psychology , executive functions , phenylketonurias , medicine , intelligence quotient , psychiatry , phenylalanine , linguistics , philosophy , biochemistry , chemistry , amino acid
Severe mental handicap in phenytketonuHa (PKU) can be prevented if dietary treatment is implemented at birth. Controversy remains about the optimum age for terminating treatment, A group of adolescents and young adults with PKU from the West of Scotland Register was identified which had received early treatment, been well‐controlled on diet, ceased treatment at 10 years old and subsequently were hyperphenyialaninaemic for 3 years or more. They were given a battery of neuropsychologcal tests and their results were compared with those of onndiet subjects with PKU and normal controls. The findings generally supported the view that dietary cessation at age 10 is sufficient to prevent a substantial reduction of cognitive and motor ability, and that the central nervous system is probably mature enough to withstand the toxic effects of high blood phenylaianine by then. However, there were minor indications, in keeping with Welsh et al.'s hypothesis [M.C. Welsh. B.F. Pennington. S. Ozonoff. B. Rouse E.R.B. McCabe (1990) Neuropsychology of early‐treated phenylketonuria: specific executive function deficits. Child Development 61, 1697–713], that subtle intelleaual deficiencies might arise both during and after treatment, possibly in the realm of frontal‐executive functions.