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Delta power robustly predicts cognitive function in Angelman syndrome
Annals of Clinical and Translational Neurology ( IF 4.4 ) Pub Date : 2021-05-28 , DOI: 10.1002/acn3.51385 Lauren M Ostrowski 1, 2 , Elizabeth R Spencer 1, 3 , Lynne M Bird 4 , Ronald Thibert 1, 5 , Robert W Komorowski 6 , Mark A Kramer 3 , Catherine J Chu 1, 5
Annals of Clinical and Translational Neurology ( IF 4.4 ) Pub Date : 2021-05-28 , DOI: 10.1002/acn3.51385 Lauren M Ostrowski 1, 2 , Elizabeth R Spencer 1, 3 , Lynne M Bird 4 , Ronald Thibert 1, 5 , Robert W Komorowski 6 , Mark A Kramer 3 , Catherine J Chu 1, 5
Affiliation
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.
中文翻译:
Delta 功率有力地预测了 Angelman 综合征的认知功能
Angelman 综合征 (AS) 是一种严重的神经发育障碍,由神经元中母系遗传的UBE3A基因功能丧失引起。恢复UBE3A表达的有希望的疾病改善治疗正在开发中,早期治疗反应的测量对于它们在临床试验中的部署至关重要。EEG 记录中增量能量的增加,反映了异常的神经元同步,发生在跨物种的 AS 中,并且与基因型相关。delta power 是否为临床症状提供了可靠的生物标志物仍然未知。
更新日期:2021-07-16
中文翻译:
Delta 功率有力地预测了 Angelman 综合征的认知功能
Angelman 综合征 (AS) 是一种严重的神经发育障碍,由神经元中母系遗传的UBE3A基因功能丧失引起。恢复UBE3A表达的有希望的疾病改善治疗正在开发中,早期治疗反应的测量对于它们在临床试验中的部署至关重要。EEG 记录中增量能量的增加,反映了异常的神经元同步,发生在跨物种的 AS 中,并且与基因型相关。delta power 是否为临床症状提供了可靠的生物标志物仍然未知。