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Sustained attention and inhibition of cognitive interference in treated phenylketonuria: associations with concurrent and lifetime phenylalanine concentrations

Sustained attention and inhibition of cognitive interference in treated phenylketonuria: associations with concurrent and lifetime phenylalanine concentrations
Sustained attention and inhibition of cognitive interference in treated phenylketonuria: associations with concurrent and lifetime phenylalanine concentrations
Fifty-seven 7–14-year-old early- and continuously treated phenylketonuria (PKU) patients and 65 matched controls performed a sustained attention task. PKU patients with plasma phenylalanine (phe) levels higher than 360 ?mol/l at the time of testing exhibited, compared to controls, lower speed of information processing, a lower ability to inhibit task-induced cognitive interference, less consistent performance, and a stronger decrease of performance level over time. Patients with concurrent phe levels lower than 360 ?mol/l did not differ from controls and were significantly better than patients with levels higher than 360 ?mol/l. Strong relationships were found with task performance for phe levels during the pre-school years and between ages 5 and 7. These correlations were stronger than those between concurrent phe level and task performance. Significant multiple regression models were found with age accounting for the largest proportion of variance of tempo and tempo fluctuation, and lifetime phe levels (particularly phe level between ages 5 and 7) accounting for the largest proportion of variance of the relative number of inhibition errors and its increase over time. Phe level between ages 5 and 7 also contributed significantly to the variance of tempo and tempo fluctuation. Neuropsychological outcome was independent of IQ. The results indicate that strict dietary adherence during these periods is beneficial to attentional control later in life. We suggest that phe levels should be maintained under 360 ?mol/l until approximately age 12, when development of attentional control approaches an adult level.
0028-3932
7-15
Huijbregts, S.C.J.
1268dc4d-9c81-4452-9da6-82cdc9dd39f8
de Sonneville, L.M.J.
38de127a-43ac-48f9-a0a5-95e5ad2baa5d
Licht, R.
f00f825e-915f-4306-a530-413c43765897
van Spronsen, F.J.
7f022d8f-cc4d-4ba5-813e-0b2bd2051b96
Verkerk, P.H.
9e6e1fa8-9ef8-4189-af98-b75b8f636c97
Sergeant, J.A.
2f434c2e-8247-4721-b4b0-b0d1849c843f
Huijbregts, S.C.J.
1268dc4d-9c81-4452-9da6-82cdc9dd39f8
de Sonneville, L.M.J.
38de127a-43ac-48f9-a0a5-95e5ad2baa5d
Licht, R.
f00f825e-915f-4306-a530-413c43765897
van Spronsen, F.J.
7f022d8f-cc4d-4ba5-813e-0b2bd2051b96
Verkerk, P.H.
9e6e1fa8-9ef8-4189-af98-b75b8f636c97
Sergeant, J.A.
2f434c2e-8247-4721-b4b0-b0d1849c843f

Huijbregts, S.C.J., de Sonneville, L.M.J., Licht, R., van Spronsen, F.J., Verkerk, P.H. and Sergeant, J.A. (2002) Sustained attention and inhibition of cognitive interference in treated phenylketonuria: associations with concurrent and lifetime phenylalanine concentrations. Neuropsychologia, 40 (1), 7-15. (doi:10.1016/S0028-3932(01)00078-1).

Record type: Article

Abstract

Fifty-seven 7–14-year-old early- and continuously treated phenylketonuria (PKU) patients and 65 matched controls performed a sustained attention task. PKU patients with plasma phenylalanine (phe) levels higher than 360 ?mol/l at the time of testing exhibited, compared to controls, lower speed of information processing, a lower ability to inhibit task-induced cognitive interference, less consistent performance, and a stronger decrease of performance level over time. Patients with concurrent phe levels lower than 360 ?mol/l did not differ from controls and were significantly better than patients with levels higher than 360 ?mol/l. Strong relationships were found with task performance for phe levels during the pre-school years and between ages 5 and 7. These correlations were stronger than those between concurrent phe level and task performance. Significant multiple regression models were found with age accounting for the largest proportion of variance of tempo and tempo fluctuation, and lifetime phe levels (particularly phe level between ages 5 and 7) accounting for the largest proportion of variance of the relative number of inhibition errors and its increase over time. Phe level between ages 5 and 7 also contributed significantly to the variance of tempo and tempo fluctuation. Neuropsychological outcome was independent of IQ. The results indicate that strict dietary adherence during these periods is beneficial to attentional control later in life. We suggest that phe levels should be maintained under 360 ?mol/l until approximately age 12, when development of attentional control approaches an adult level.

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Published date: 2002

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Local EPrints ID: 18578
URI: http://eprints.soton.ac.uk/id/eprint/18578
ISSN: 0028-3932
PURE UUID: 45580df5-6c67-4fec-bf9c-ce03c138a2f5

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Date deposited: 02 Dec 2005
Last modified: 15 Mar 2024 06:06

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Contributors

Author: S.C.J. Huijbregts
Author: L.M.J. de Sonneville
Author: R. Licht
Author: F.J. van Spronsen
Author: P.H. Verkerk
Author: J.A. Sergeant

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