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The neuropsychological profile of early and continuously treated phenylketonuria: orienting, vigilance, and maintenance versus manipulation functions of working memory

The neuropsychological profile of early and continuously treated phenylketonuria: orienting, vigilance, and maintenance versus manipulation functions of working memory
The neuropsychological profile of early and continuously treated phenylketonuria: orienting, vigilance, and maintenance versus manipulation functions of working memory
In this paper, we review neuropsychological test results of early and continuously treated Phenylketonuria (PKU) patients. To increase insight into the neuropsychological profile of this population, we have attempted to place the results within an attentional network model [Images of the mind, 1994], which proposes interacting but dissociable attentional networks for orienting, vigilance, and executive control of attention. Executive control of attention is discussed against the background of the process-specific theory of working memory (WM) [Handbook of neuropsychology, 1994], which postulates a distinction between the ‘maintenance’-function of WM and the ‘manipulation and monitoring’-function.
Neuropsychological results are presented for 67 early and continuously treated PKU patients and 73 controls aged 7–14 years. Four neuropsychological tasks were employed to measure orienting, mnemonic processing, interference suppression, and top-down control in visual search. No differences were found in orienting and the maintenance-function of WM. In addition to previously reported impairments in sustained attention/vigilance and inhibition of prepotent responding, PKU patients exhibited deficits when top-down control was required in a visual search task, but showed no impairment when interference suppression was required. It is discussed how the specific neuropsychological impairments in PKU may be a consequence of mid-dorsolateral prefrontal cortex (DLPFC) dysfunctioning due to deficiencies in catecholamine modulation.
phenylketonuria, phenylalanine, tyrosine, selective attention, vigilance, sustained attention, working memory, prefrontal cortex, dopamine, noradrenaline
0149-7634
697-712
Huijbregts, S.C.J.
1268dc4d-9c81-4452-9da6-82cdc9dd39f8
de Sonneville, L.M.J.
38de127a-43ac-48f9-a0a5-95e5ad2baa5d
van Spronsen, F.J.
7f022d8f-cc4d-4ba5-813e-0b2bd2051b96
Licht, R.
f00f825e-915f-4306-a530-413c43765897
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
van Spronsen, F.J.
7f022d8f-cc4d-4ba5-813e-0b2bd2051b96
Licht, R.
f00f825e-915f-4306-a530-413c43765897
Sergeant, J.A.
2f434c2e-8247-4721-b4b0-b0d1849c843f

Huijbregts, S.C.J., de Sonneville, L.M.J., van Spronsen, F.J., Licht, R. and Sergeant, J.A. (2002) The neuropsychological profile of early and continuously treated phenylketonuria: orienting, vigilance, and maintenance versus manipulation functions of working memory. Neuroscience & Biobehavioral Reviews, 26 (6), 697-712. (doi:10.1016/S0149-7634(02)00040-4).

Record type: Article

Abstract

In this paper, we review neuropsychological test results of early and continuously treated Phenylketonuria (PKU) patients. To increase insight into the neuropsychological profile of this population, we have attempted to place the results within an attentional network model [Images of the mind, 1994], which proposes interacting but dissociable attentional networks for orienting, vigilance, and executive control of attention. Executive control of attention is discussed against the background of the process-specific theory of working memory (WM) [Handbook of neuropsychology, 1994], which postulates a distinction between the ‘maintenance’-function of WM and the ‘manipulation and monitoring’-function.
Neuropsychological results are presented for 67 early and continuously treated PKU patients and 73 controls aged 7–14 years. Four neuropsychological tasks were employed to measure orienting, mnemonic processing, interference suppression, and top-down control in visual search. No differences were found in orienting and the maintenance-function of WM. In addition to previously reported impairments in sustained attention/vigilance and inhibition of prepotent responding, PKU patients exhibited deficits when top-down control was required in a visual search task, but showed no impairment when interference suppression was required. It is discussed how the specific neuropsychological impairments in PKU may be a consequence of mid-dorsolateral prefrontal cortex (DLPFC) dysfunctioning due to deficiencies in catecholamine modulation.

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More information

Published date: 2002
Additional Information: Review
Keywords: phenylketonuria, phenylalanine, tyrosine, selective attention, vigilance, sustained attention, working memory, prefrontal cortex, dopamine, noradrenaline
Organisations: Psychology

Identifiers

Local EPrints ID: 18575
URI: http://eprints.soton.ac.uk/id/eprint/18575
ISSN: 0149-7634
PURE UUID: 25287095-d814-452f-babd-7b2f8e2b00ed

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Date deposited: 02 Dec 2005
Last modified: 15 Jul 2019 19:28

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