Losito Emma, Battaglia Domenica, Chieffo Daniela, Raponi Matteo, Ranalli Domiziana, Contaldo Ilaria, Giansanti Cristina, De Clemente Valentina, Quintiliani Michela, Antichi Eleonora, Verdolotti Tommaso, de Waure Chiara, Tartaglione Tommaso, Mercuri Eugenio, Guzzetta Francesco
Child Neurology and Psychiatry, Catholic University, Rome, Italy.
Institute of Public Health, Catholic University, Rome, Italy.
Epilepsy Res. 2015 Jan;109:90-9. doi: 10.1016/j.eplepsyres.2014.10.015. Epub 2014 Oct 28.
The study aims at a better definition of continuous spike-waves during sleep (CSWS) with an early thalamic lesion, focusing on various grades of sleep-potentiated epileptiform activity (SPEA). Their possible relationship with different clinical features was studied to try to define prognostic factors of the epileptic disorder, especially relating to behavior/cognitive outcome, in order to improve prevention and treatment strategies.
Sixty patients with early thalamic injury were followed since the first registration of SPEA with serial neurological, long term EEG monitoring and neuropsychological examinations, as well as neuroimaging and a detailed clinical history. They were classified in three different groups according to the sleep spike-waves (SW) quantification: electrical status epilepticus during sleep (ESES), more than 85% of slow sleep; overactivation between 50% and 85% and simple activation between 10 and 50%). Results were then examined also with a statistical analysis.
In our series of CSWS occurring in early brain injured children with unilateral thalamic involvement there is a common neuropathologic origin but with various grades of SPEA severity. Statistical analysis showed that patients evolving toward ESES presented more commonly the involvement of the mediodorsal part of thalamus nuclei and a bilateral cortico-subcortical brain injury, epilepsy was more severe with a delayed onset; moreover, in the acute stage .ESES patients presented the worst behavior/cognitive performances. As to cognitive and behavior outcome, longer SPEA duration as well as bilateral brain injury and cognitive/behavior impairment in acute phase appear linked to a poor outcome; some particular neuropathology (ischemic stroke and haemorrhagic infarction) as well as hydrocephalus shunting are associated with behavior disorders.
Discrete features seem to support different underlying mechanisms in ESES patients in comparison with less severe SPEA; they represent negative prognostic factors. Longer SPEA duration as well as bilateral brain injury and cognitive/behavior impairment in acute phase seem predictive of a worse cognitive/behavior outcome.
本研究旨在更好地界定早期丘脑病变患者睡眠期持续棘慢波（CSWS），重点关注不同程度的睡眠增强型癫痫样放电（SPEA）。研究其与不同临床特征之间的可能关系，以试图确定癫痫性疾病的预后因素，尤其是与行为/认知结局相关的因素，从而改进预防和治疗策略。
自首次记录到SPEA起，对60例早期丘脑损伤患者进行随访，包括系列神经学检查、长期脑电图监测、神经心理学检查、神经影像学检查以及详细的临床病史采集。根据睡眠期棘慢波（SW）量化情况将患者分为三个不同组：睡眠期癫痫性电持续状态（ESES），慢波睡眠期超过85%；激活超过50%至85%以及激活在10%至50%之间）。然后对结果进行统计分析。
在我们这组发生于早期脑损伤且单侧丘脑受累儿童的CSWS系列中，存在共同的神经病理起源，但SPEA严重程度不同。统计分析表明，发展为ESES的患者丘脑核团内侧背侧部分受累及双侧皮质 - 皮质下脑损伤更为常见，癫痫更严重且起病延迟；此外，在急性期，ESES患者的行为/认知表现最差。关于认知和行为结局，较长的SPEA持续时间以及急性期的双侧脑损伤和认知/行为损害似乎与不良结局相关；某些特定的神经病理学情况（缺血性卒中及出血性梗死）以及脑积水分流与行为障碍有关。
与不太严重的SPEA相比，离散特征似乎支持ESES患者存在不同的潜在机制；它们代表不良预后因素。较长的SPEA持续时间以及急性期的双侧脑损伤和认知/行为损害似乎预示着更差的认知/行为结局。
