Analysis of EEG patterns and genotypes in patients with Angelman syndrome.
Publication/Presentation Date
3-1-2012
Abstract
We prospectively analyzed EEGs from participants in the ongoing NIH Rare Diseases Clinical Research Network Angelman Syndrome Natural History Study. Of the one-hundred-sixty enrolled patients (2006-2010), 115 had complete data (58 boys, median age 3.6 years). Distinct EEG findings were intermittent rhythmic delta waves (83.5%), interictal epileptiform discharges (74.2%), intermittent rhythmic theta waves (43.5%), and posterior rhythm slowing (43.5%). Centro-occipital and centro-temporal delta waves decreased with age (p=0.01, p=0.03). There were no specific correlations between EEG patterns and genotypes. A classification tree allowed the prediction of deletions class-1 (5.9 Mb) in patients with intermittent theta waves in50% theta and normal posterior rhythm; atypical deletions in patients with >50% theta but abnormal posterior rhythm. EEG patterns are important biomarkers in Angelman syndrome and may suggest the underlying genetic etiology.
Volume
23
Issue
3
First Page
261
Last Page
265
ISSN
1525-5069
Published In/Presented At
Vendrame, M., Loddenkemper, T., Zarowski, M., Gregas, M., Shuhaiber, H., Sarco, D. P., Morales, A., Nespeca, M., Sharpe, C., Haas, K., Barnes, G., Glaze, D., & Kothare, S. V. (2012). Analysis of EEG patterns and genotypes in patients with Angelman syndrome. Epilepsy & behavior : E&B, 23(3), 261–265. https://doi.org/10.1016/j.yebeh.2011.11.027
Disciplines
Medicine and Health Sciences
PubMedID
22341959
Department(s)
Department of Medicine
Document Type
Article