| Autor: |
Lauren M. Ostrowski, Elizabeth R. Spencer, Lynne M. Bird, Ronald Thibert, Robert W. Komorowski, Mark A. Kramer, Catherine J. Chu |
| Jazyk: |
angličtina |
| Rok vydání: |
2021 |
| Předmět: |
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| Zdroj: |
Annals of Clinical and Translational Neurology, Vol 8, Iss 7, Pp 1433-1445 (2021) |
| Druh dokumentu: |
article |
| ISSN: |
2328-9503 |
| DOI: |
10.1002/acn3.51385 |
| Popis: |
Abstract Objective Angelman syndrome (AS) is a severe neurodevelopmental disorder caused by loss of function of the maternally inherited UBE3A gene in neurons. Promising disease‐modifying treatments to reinstate UBE3A expression are under development and an early measure of treatment response is critical to their deployment in clinical trials. Increased delta power in EEG recordings, reflecting abnormal neuronal synchrony, occurs in AS across species and correlates with genotype. Whether delta power provides a reliable biomarker for clinical symptoms remains unknown. Methods We analyzed combined EEG recordings and developmental assessments in a large cohort of individuals with AS (N = 82 subjects, 133 combined EEG and cognitive assessments, 1.08–28.16 years; 32F) and evaluated delta power as a biomarker for cognitive function, as measured by the Bayley Cognitive Score. We examined the robustness of this biomarker to varying states of consciousness, recording techniques and analysis procedures. Results Delta power predicted the Bayley Scale cognitive score (P 4) and predicted performance in expressive (P = 0.0209) and receptive communication (P |
| Databáze: |
Directory of Open Access Journals |
| Externí odkaz: |
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