目的:迄今为止,Aicardi综合征(AIC)中脑电图(EEG)模式的描述涉及多达6例的小群群,并表明所有病例中的电活动严重紊乱。本研究旨在描述更大的AIC队列中的长期EEG演变,随访了23年,并确定临床和脑电图结果的可能早期预测因子。
方法:在一项回顾性研究中,两位经验丰富的临床神经生理学家系统地回顾了12例AIC病例的所有脑电图记录,从癫痫发作到现在。用标准化临床结果量表评估临床结果。
结果:数据分析揭示了两种不同的AIC表型。除了文献中已经描述的“经典严重表型”之外,我们发现了一种新的“轻度表型”。这两种表型在癫痫发作和演变过程中表现出完全不同的脑电图特征。对应于不同的临床结果。
结论:来自我们的长期脑电图和临床神经放射学研究的数据使我们能够描述AIC的两种不同表型,具有不同的成像严重程度,特别是,不同的脑电图,随着时间的推移往往保持不变。
结论:一起,这些发现可能有助于预测长期临床结局.
OBJECTIVE: Descriptions of electroencephalographic (EEG) patterns in Aicardi syndrome (AIC) have to date referred to small cohorts of up to six cases and indicated severe derangement of electrical activity in all cases. The present study was conducted to describe the long-term EEG evolution in a larger AIC cohort, followed for up to 23 years, and identify possible early predictors of the clinical and EEG outcomes.
METHODS: In a retrospective study, two experienced clinical neurophysiologists systematically reviewed all EEG traces recorded in 12 AIC cases throughout their follow-up, from epilepsy onset to the present. Clinical outcome was assessed with standardized clinical outcome scales.
RESULTS: Analysis of the data revealed two distinct AIC phenotypes. In addition to the \"classical severe phenotype\" already described in the literature, we identified a new \"mild phenotype\". The two phenotypes show completely different EEG features at onset of epilepsy and during its evolution, which correspond to different clinical outcomes.
CONCLUSIONS: Data from our long-term EEG and clinical-neuroradiological study allowed us to describe two different phenotypes of AIC, with different imaging severity and, in particular, different EEG at onset, which tend to remain constant over time.
CONCLUSIONS: Together, these findings might help to predict long-term clinical outcomes.