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The Association between EEG Abnormality and Behavioral Disorder: Developmental Delay in Phenylketonuria

Background. Brain defect leading to developmental delay is one of the clinical manifestations of phenylketonuria. The aim of this study was to evaluate the association between EEG abnormality and developmental delay/behavioral disorders in phenylketonuria. Patients and Methods. 105 phenylketonuria p...

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Autores principales: Karimzadeh, Parvaneh, Alaee, Mohammad Reza, Zarafshan, Hadi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: International Scholarly Research Network 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3324893/
https://www.ncbi.nlm.nih.gov/pubmed/22550595
http://dx.doi.org/10.5402/2012/976206
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author Karimzadeh, Parvaneh
Alaee, Mohammad Reza
Zarafshan, Hadi
author_facet Karimzadeh, Parvaneh
Alaee, Mohammad Reza
Zarafshan, Hadi
author_sort Karimzadeh, Parvaneh
collection PubMed
description Background. Brain defect leading to developmental delay is one of the clinical manifestations of phenylketonuria. The aim of this study was to evaluate the association between EEG abnormality and developmental delay/behavioral disorders in phenylketonuria. Patients and Methods. 105 phenylketonuria patients, who were diagnosed through newborn screening tests or during follow-up evaluation, were enrolled. Patients who were seizure-free for at least six months before the study were included. The developmental score were evaluated by the ASQ questionnaire (age-stage questionnaire) and the test of child symptom inventory-4 (CSI-4), respectively. Results. 55 patients had a history of seizure more than 6 months before the study. Seventy had abnormal EEG (cases) and 35 had normal EEG (controls). There was no significant difference between mean phenylalanine levels in the abnormal and normal EEG groups at the time of diagnosis, after six months and at our evaluation. Distribution of DQ level in the abnormal and normal EEG groups revealed a significant difference. An abnormal EEG was associated with a higher percentage of low DQ levels. Conclusion. Paroxysmal epileptic discharges in PKU patients are important. Treatment of these EEG abnormalities may affect developmental scores or may lead to correction of some behavioral disorders in patients.
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spelling pubmed-33248932012-05-01 The Association between EEG Abnormality and Behavioral Disorder: Developmental Delay in Phenylketonuria Karimzadeh, Parvaneh Alaee, Mohammad Reza Zarafshan, Hadi ISRN Pediatr Research Article Background. Brain defect leading to developmental delay is one of the clinical manifestations of phenylketonuria. The aim of this study was to evaluate the association between EEG abnormality and developmental delay/behavioral disorders in phenylketonuria. Patients and Methods. 105 phenylketonuria patients, who were diagnosed through newborn screening tests or during follow-up evaluation, were enrolled. Patients who were seizure-free for at least six months before the study were included. The developmental score were evaluated by the ASQ questionnaire (age-stage questionnaire) and the test of child symptom inventory-4 (CSI-4), respectively. Results. 55 patients had a history of seizure more than 6 months before the study. Seventy had abnormal EEG (cases) and 35 had normal EEG (controls). There was no significant difference between mean phenylalanine levels in the abnormal and normal EEG groups at the time of diagnosis, after six months and at our evaluation. Distribution of DQ level in the abnormal and normal EEG groups revealed a significant difference. An abnormal EEG was associated with a higher percentage of low DQ levels. Conclusion. Paroxysmal epileptic discharges in PKU patients are important. Treatment of these EEG abnormalities may affect developmental scores or may lead to correction of some behavioral disorders in patients. International Scholarly Research Network 2012-03-29 /pmc/articles/PMC3324893/ /pubmed/22550595 http://dx.doi.org/10.5402/2012/976206 Text en Copyright © 2012 Parvaneh Karimzadeh et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Research Article
Karimzadeh, Parvaneh
Alaee, Mohammad Reza
Zarafshan, Hadi
The Association between EEG Abnormality and Behavioral Disorder: Developmental Delay in Phenylketonuria
title The Association between EEG Abnormality and Behavioral Disorder: Developmental Delay in Phenylketonuria
title_full The Association between EEG Abnormality and Behavioral Disorder: Developmental Delay in Phenylketonuria
title_fullStr The Association between EEG Abnormality and Behavioral Disorder: Developmental Delay in Phenylketonuria
title_full_unstemmed The Association between EEG Abnormality and Behavioral Disorder: Developmental Delay in Phenylketonuria
title_short The Association between EEG Abnormality and Behavioral Disorder: Developmental Delay in Phenylketonuria
title_sort association between eeg abnormality and behavioral disorder: developmental delay in phenylketonuria
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3324893/
https://www.ncbi.nlm.nih.gov/pubmed/22550595
http://dx.doi.org/10.5402/2012/976206
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