Derangement of the dopaminergic system in phenylketonuria: Study of the event‐related potential (P300)

Leuzzi, Vincenzo; Seri, Stefano; Cerquiglini, Antonella; Carducci, Cl.; Carducci, Ca.; Antonozzi, I.

doi:10.1023/a:1005646206348

Cited by 9 publications

(2 citation statements)

References 6 publications

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“…Compromised myelination of neuronal connections, particularly in the first months of gestation, and deficient levels of dopamine and serotonin may affect the development of prefrontal cortical functions in particular (Leuzzi et al 2000; Diamond 2001). A number of studies using magnetic resonance imaging (MRI) and/or Diffusion Tensor Imaging (DTI) indeed have shown abnormalities in the microstructural integrity of the white matter in early-treated PKU patients (Dyer 1999; Cleary et al 1994; Thompson et al 1993; Weglage et al 1997), even in normal appearing white matter (Ding et al 2008; Kono et al 2005; Leuzzi et al 2007; Vermathen et al 2007; Manara et al 2009; Scarabino et al 2009; White et al 2009).…”

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confidence: 99%

Pre‐attentive processing in children with early and continuously‐treated PKU. Effects of concurrent Phe level and lifetime dietary control

Sonneville

Huijbregts

Licht³

et al. 2011

J of Inher Metab Disea

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Sixty-four children, aged 7 to 14 years, with early-treated PKU, were compared with control children on visual evoked potential (VEP) amplitudes and latencies and auditory mismatch negativity (MMN) amplitudes. It was further investigated whether indices of dietary control would be associated with these evoked potentials parameters. There were no significant differences between controls and children with PKU in VEP- and MMN-indices. However, higher lifetime Phe levels were, in varying degree and stronger than concurrent Phe level, related to increased N75 amplitudes, suggesting abnormalities in attention, and longer P110 latencies, indicating a reduction in speed of neural processing, possibly due to deficits in myelination or reduced dopamine levels in brain and retina. Similarly, higher lifetime Phe levels and Index of Dietary Control (IDC) were associated with decreased MMN amplitudes, suggesting a reduced ability to respond to stimulus change and poorer triggering of the frontally mediated attention switch. In summary, the present study in children with PKU investigated bottom-up information processing, i.e., triggered by external events, a fundamental prerequisite for the individual’s responsiveness to the outside world. Results provide evidence that quality of dietary control may affect the optimal development of these pre-attentive processes, and suggest the existence of windows of vulnerability to Phe exposure.

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confidence: 99%

Pre‐attentive processing in children with early and continuously‐treated PKU. Effects of concurrent Phe level and lifetime dietary control

Sonneville

Huijbregts

Licht³

et al. 2011

J of Inher Metab Disea

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“…Both data suggest a specific vulnerability of the network generating this potential to Phe according to patients’ age. A severe derangement of P300 wave under oddball auditory paradigm was found in late-treated PKU patients, while early-treated patients showed an increase of P300 latency and amplitude unrelated to historical and/or concomitant levels of Phe (35). These findings were not confirmed in younger early and continuously treated PKU patients, which were studied with a visual discriminative paradigm (36).…”

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confidence: 89%

Age-Related Psychophysiological Vulnerability to Phenylalanine in Phenylketonuria

et al. 2014

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Background: Phenylketonuria (PKU) is caused by the inherited defect of the phenylalanine hydroxylase enzyme, which converts phenylalanine (Phe) into tyrosine (Tyr). Neonatal screening programs and early treatment have radically changed the natural history of PKU. Nevertheless, an increased risk of neurocognitive and psychiatric problems in adulthood remains a challenging aspect of the disease. In order to assess the vulnerability of complex skills to Phe, we explored: (a) the effect of a rapid increase in blood Phe levels on event-related potentials (ERP) in PKU subjects during their second decade of life; (b) the association (if existing) between psychophysiological and neurocognitive features.Methods: Seventeen early-treated PKU subjects, aged 10–20, underwent ERP [mismatch negativity, auditory P300, contingent negative variation (CNV), and Intensity Dependence of Auditory Evoked Potentials] recording before and 2 h after an oral loading of Phe. Neurocognitive functioning, historical and concurrent biochemical values of blood Phe, Tyr, and Phe/Tyr ratio, were all included in the statistical analysis.Results: Event-related potential components were normally detected in all the subjects. In subjects younger than 13 CNV amplitude, W2-CNV area, P3b latency, and reaction times in motor responses were negatively influenced by Phe-loading. Independently from the psychophysiological vulnerability, some neurocognitive skills were more impaired in younger patients. No correlation was found between biochemical alterations and neurocognitive and psychophysiological findings.Conclusion: The vulnerability of the emerging neurocognitive functions to Phe suggests a strict metabolic control in adolescents affected by PKU and a neurodevelopmental approach in the study of neurocognitive outcome in PKU.

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State regulation and response inhibition in children with ADHD and children with early‐ and continuously treated phenylketonuria: An event‐related potential comparison

Wiersema

Meere²,

Roeyers

et al. 2005

J of Inher Metab Disea

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Derangement of the dopaminergic system in phenylketonuria: Study of the event‐related potential (P300)

Cited by 9 publications

References 6 publications

Pre‐attentive processing in children with early and continuously‐treated PKU. Effects of concurrent Phe level and lifetime dietary control

Pre‐attentive processing in children with early and continuously‐treated PKU. Effects of concurrent Phe level and lifetime dietary control

Age-Related Psychophysiological Vulnerability to Phenylalanine in Phenylketonuria

State regulation and response inhibition in children with ADHD and children with early‐ and continuously treated phenylketonuria: An event‐related potential comparison

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