Descriptive analysis of the electroencephalogram in Angelman syndrome

Angelman syndrome is a neurodevelopmental disorder of genetic origin, with important clinical motor, behavioural, communicative and electroencephalographic manifestations, with particular relevance as regards the presence of epileptic seizures. To describe the electroencephalographic characteristics...

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Veröffentlicht in:Revista de neurologiá 2021-01, Vol.72 (2), p.51-60
Hauptverfasser: López-Pájaro, L F, Ruiz-Antorán, B, Marín-Serrano, E, Cazorla-Calleja, R, Iglesias-Escalera, G, Lara-Herguedas, J, García-Merino, J A, Avendaño-Solá, C, Sancho-López, A
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Zusammenfassung:Angelman syndrome is a neurodevelopmental disorder of genetic origin, with important clinical motor, behavioural, communicative and electroencephalographic manifestations, with particular relevance as regards the presence of epileptic seizures. To describe the electroencephalographic characteristics (qualitatively and quantitatively) of patients diagnosed with Angelman syndrome and to determine the electroencephalographic profile according to age and genetic alteration. A retrospective observational study in which the demographic, clinical and electroencephalographic characteristics of 51 patients with Angelman syndrome were analysed. A higher delta power was evident in all brain regions, with a maximum peak in the frontopolar and temporal regions, and a lower power in the alpha and beta frequency range in all regions, with a greater preponderance in younger patients, and a trend that decreases with age. The coherence showed a predominance of delta and theta in the frontopolar region, which was higher for all frequencies in the deletion group, where delta was predominant, especially in the frontopolar region. The electroencephalogram could be a useful biomarker as a qualitative and quantitative tool in the investigation of Angelman syndrome and in measuring the response to possible therapies under investigation.
ISSN:1576-6578
DOI:10.33588/rn.7202.2020548