Epileptic Electroencephalography Profile Associates with Attention Problems in Children with Fragile X Syndrome : Review and Case Series

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Cowley , B , Kirjanen , S , Partanen , J & Castrén , M 2016 , ' Epileptic Electroencephalography Profile Associates with Attention Problems in Children with Fragile X Syndrome : Review and Case Series ' , Frontiers in Human Neuroscience , vol. 10 , 353 . https://doi.org/10.3389/fnhum.2016.00353

Title: Epileptic Electroencephalography Profile Associates with Attention Problems in Children with Fragile X Syndrome : Review and Case Series
Author: Cowley, Benjamin; Kirjanen, Svetlana; Partanen, Juhani; Castrén, Maija
Contributor: University of Helsinki, Behavioural Sciences
University of Helsinki, Department of Neurosciences
University of Helsinki, Medicum
Date: 2016-07-12
Language: eng
Number of pages: 8
Belongs to series: Frontiers in Human Neuroscience
ISSN: 1662-5161
URI: http://hdl.handle.net/10138/165283
Abstract: Fragile X syndrome (FXS) is the most common cause of inherited intellectual disability and a variant of autism spectrum disorder (ASD). The FXS population is quite heterogeneous with respect to comorbidities, which implies the need for a personalized medicine approach, relying on biomarkers or endophenotypes to guide treatment. There is evidence that quantitative electroencephalography (EEG) endophenotype-guided treatments can support increased clinical benefit by considering the patient's neurophysiological profile. We describe a case series of 11 children diagnosed with FXS, aged one to 14 years, mean 4.6 years. Case data are based on longitudinal clinically-observed reports by attending physicians for comorbid symptoms including awake and asleep EEG profiles. We tabulate the comorbid EEG symptoms in this case series, and relate them to the literature on EEG endophenotypes and associated treatment options. The two most common endophenotypes in the data were diffuse slow oscillations and epileptiform EEG, which have been associated with attention and epilepsy respectively. This observation agrees with reported prevalence of comorbid behavioral symptoms for FXS. In this sample of FXS children, attention problems were found in 37% (4 of 11), and epileptic seizures in 45% (5 of 11). Attention problems were found to associate with the epilepsy endophenotype. From the synthesis of this case series and literature review, we argue that the evidence-based personalized treatment approach, exemplified by neurofeedback, could benefit FXS children by focusing on observable, specific characteristics of comorbid disease symptoms.
Subject: fragile X syndrome
electroencephalography
clinical case series
endophenotype
attention deficit disorder
neurofeedback
DEFICIT/HYPERACTIVITY DISORDER
QUANTITATIVE EEG
NEUROFEEDBACK
ADHD
AUTISM
MALES
GENE
HYPERACTIVITY
METAANALYSIS
SPECIFICITY
3111 Biomedicine
3112 Neurosciences
3124 Neurology and psychiatry
515 Psychology
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