Randò Teresa, Bancale Adina, Baranello Giovanni, Bini Margherita, De Belvis Anton Giulio, Epifanio Roberta, Frisone Maria Flavia, Guzzetta Andrea, La Torre Giuseppe, Ricci Daniela, Signorini Sabrina, Tinelli Francesca, Biagioni Enrico, Veggiotti Pierangelo, Mercuri Eugenio, Fazzi Elisa, Cioni Giovanni, Guzzetta Francesco
Division of Child Neurology and Psychiatry, Catholic University, Rome, Italy.
Epilepsia. 2004 Jul;45(7):781-6. doi: 10.1111/j.0013-9580.2004.41403.x.
Several studies have reported behavioral and electrophysiological evidence of visual impairment during the active stage of West syndrome. The underlying mechanisms are, however, poorly understood, and little has been reported about the correlation between visual impairment, EEG patterns, and brain lesions. The aim of the study was to assess visual function at the onset of spasm and 2 months thereafter and relate visual findings to brain lesions and EEG features.
Twenty-five infants with West syndrome were enrolled and studied with (a) a full clinical assessment including a battery of tests specifically designed to assess visual function, (b) a video-polygraphic study, and (c) brain magnetic resonance imaging (MRI). Besides brain neuroimaging and EEG comparison with visual function, an intra-EEG analysis was performed to investigate the possible relation of EEG patterns to fluctuating visual behavior (fixation and following).
Twenty-two children had at least one abnormal result on one or more of the tests assessing visual function at T0. Visual impairment at the spasm onset was related to the sleep disorganization rather than to the hypsarrhythmic pattern in awake EEG. After 2 months, both EEG features become significantly linked to visual function. Visual function improved in several cases after 2 months, in parallel with the seizure regression. No relation was found between EEG patterns and fluctuating visual behavior.
The study supplies new evidence of the involvement of visual function in West syndrome. The presence of abnormal visual findings in infants without lesions on brain MRI suggests that visual abnormalities are due not only to brain injury but also to epileptic disorder per se. New insight is also provided into the possible mechanisms underlying clinical and EEG abnormalities.
多项研究报告了韦斯特综合征活动期视觉障碍的行为和电生理证据。然而，其潜在机制尚不清楚，关于视觉障碍、脑电图模式和脑损伤之间的相关性报道甚少。本研究的目的是评估痉挛发作时及之后2个月的视觉功能，并将视觉结果与脑损伤和脑电图特征相关联。
招募了25名韦斯特综合征婴儿，并进行了以下研究：（a）全面的临床评估，包括一系列专门设计用于评估视觉功能的测试；（b）视频多导记录研究；（c）脑磁共振成像（MRI）。除了将脑影像学和脑电图与视觉功能进行比较外，还进行了脑电图内部分析，以研究脑电图模式与波动的视觉行为（注视和追随）之间的可能关系。
22名儿童在T0时进行的一项或多项评估视觉功能的测试中至少有一项异常结果。痉挛发作时的视觉障碍与睡眠紊乱有关，而非与清醒脑电图中的高度节律失调模式有关。2个月后，两种脑电图特征均与视觉功能显著相关。2个月后，部分患儿的视觉功能有所改善，同时癫痫发作也有所缓解。未发现脑电图模式与波动的视觉行为之间存在关联。
该研究提供了视觉功能参与韦斯特综合征的新证据。在脑MRI无病变的婴儿中存在异常视觉结果表明，视觉异常不仅归因于脑损伤，还归因于癫痫疾病本身。该研究还为临床和脑电图异常的潜在机制提供了新的见解。
