Ronzano Nadia, Valvo Giulia, Ferrari Anna Rita, Guerrini Renzo, Sicca Federico
Epilepsy and Clinical Neurophysiology Laboratory, Department of Clinical Neuroscience, IRCCS Stella Maris Foundation, Calambrone, Pisa, Italy.
Epilepsy and Clinical Neurophysiology Laboratory, Department of Clinical Neuroscience, IRCCS Stella Maris Foundation, Calambrone, Pisa, Italy Pediatric Neurology Unit, A. Meyer Pediatric Hospital, University of Florence, Florence, Italy.
J Child Neurol. 2015 Feb;30(2):153-9. doi: 10.1177/0883073814532547. Epub 2014 Jun 6.
To evaluate the diverse presentation and course of late-onset epileptic spasms in relation to etiology, we analyzed the clinical, electroencephalographic (EEG), and prognostic features in 34 patients. We divided the patient sample into cryptogenic or symptomatic based on etiology. An association emerged between symmetric spasms at onset and focal interictal EEG abnormalities in cryptogenic patients, and onset with focal or generalized seizures before displaying asymmetric spasms, and multifocal or diffuse EEG abnormalities, in the symptomatic group. Despite an overall poor prognosis, symptomatic patients starting with generalized seizures seem to have a relatively more favorable outcome. The high occurrence of intellectual disability, and sometimes psychomotor regression, confirmed this rare and poorly understood heterogeneous clinical condition as a severe form of epileptic encephalopathy that deserves further study.
为了评估晚发性癫痫性痉挛在病因学方面的多样表现及病程，我们分析了34例患者的临床、脑电图（EEG）及预后特征。我们根据病因将患者样本分为隐源性或症状性。隐源性患者中，发作时对称痉挛与发作间期局灶性脑电图异常之间存在关联；而在症状性组中，在出现不对称痉挛之前先有局灶性或全身性发作，以及多灶性或弥漫性脑电图异常。尽管总体预后较差，但以全身性发作起病的症状性患者似乎预后相对较好。智力残疾的高发生率，以及有时出现的精神运动发育倒退，证实了这种罕见且了解甚少的异质性临床状况是一种严重形式的癫痫性脑病，值得进一步研究。
