Electroencephalographic characteristics of Dravet syndrome
Electroencephalographic characteristics of Dravet syndrome
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DOI：
10.1111/j.1528-1167.2011.02996.x
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发表时间：
2011-04-01
期刊：
影响因子：
5.6
通讯作者：
Dalla Bernardina, Bernardo
中科院分区：
文献类型：
--
作者：
Bureau, Michelle;Dalla Bernardina, Bernardo
在Dravet综合征中，发作间期和发作期脑电图（EEG）记录可能仍然具有误导性，并且没有特别的改变.此外，临床和EEG发作类型具有很大的多态性。有些可以在其他癫痫综合征中观察到，但其他的更特异，特别是特殊的单侧癫痫发作，假性全身性癫痫发作，可能伴有局灶性发作，以及不稳定性癫痫发作。在某些情况下，发作表现的特征是惊厥性癫痫发作的持续性主要复发，通常由体温升高引起，最终与部分复杂性癫痫发作相关。肌阵挛事件、肌阵挛成分缺失、迟钝状态和光敏性和/或模式敏感性缺失或出现相对较晚，短暂复发。在这些病例中，发作间期EEG的特征是持续缺乏阵发性放电。在其他情况下，在惊厥发作和体温敏感性的背景下，人们可能会发现（1）不同类型的肌阵挛发作，（2）对光和图形刺激的强烈敏感性，与早期发作和持续时间的可变关联。在这些情况下，发作间期发作和自发和诱导（间歇性光刺激，模式和眼睛闭合）刺激往往出现早期，并在演变过程中频繁和持续。根据这些电临床模式，有可能将人群分为两个亚群，两者共享共同的遗传机制，但具有不同的临床结果。
P>In Dravet syndrome, interictal and ictal electroencephalography (EEG) recording may remain misleading, and are not specifically altered. Moreover, there is a great polymorphism of clinical and EEG seizure types. Some can be observed in other epileptic syndromes, but others are more specific-particularly the peculiar unilateral seizures, the falsely generalized seizures, probably with a focal onset, and the unstable seizures. In some cases, the ictal manifestations are characterized by the persistent predominant recurrence of convulsive seizures, often induced by body temperature increase, eventually associated with partial complex seizures. The myoclonic events, absences with myoclonic component, obtundation status, and photosensitivity and/or pattern sensitivity are absent or appear relatively late and recur transiently for short periods. In these cases interictal EEG is characterized by the persistent paucity of paroxysmal discharges. In other cases, on a background of convulsive seizures and body temperature sensitivity, one may find a variable association of (1) myoclonic seizures of different types, (2) a strong sensitivity to light and pattern stimulations, with early onset and persistent in time. In these cases, interictal paroxysms and spontaneous and induced (intermittent photic stimulation, patterns, and eye closure) stimulation tend to appear early and to be frequent and persistent during the evolution. According to these electroclinical patterns it is possible to divide the population into two subsets, both sharing common genetic mechanisms but with a different clinical outcome.