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 organization: | Behavioural Sciences Department of Neurosciences Kliinisen neurofysiologian yksikkö Clinicum Medicum Department of Physiology NeuroDevDiseaseModelling |
Date: | 2016-07-12 |
Language: | eng |
Number of pages: | 8 |
Belongs to series: | Frontiers in Human Neuroscience |
ISSN: | 1662-5161 |
DOI: | https://doi.org/10.3389/fnhum.2016.00353 |
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 |
Peer reviewed: | Yes |
Rights: | cc_by |
Usage restriction: | openAccess |
Self-archived version: | publishedVersion |
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