Pavone Piero, Pappalardo Xena Giada, Parano Enrico, Falsaperla Raffaele, Marino Simona Domenica, Fink John Kane, Ruggieri Martino
Unit of Clinical Pediatrics, AOU "Policlinico", PO "G. Rodolico", University of Catania, Catania, Italy.
Unit of Catania, National Council of Research, Institute for Research and Biomedical Innovation (IRIB), Catania, Italy.
Front Pediatr. 2022 Apr 21;10:858945. doi: 10.3389/fped.2022.858945. eCollection 2022.
In addition to central nervous system infections, seizures and fever may occur together in several neurological disorders. Formerly, based on the clinical features and prognostic evolution, the co-association of seizure and fever included classical febrile seizures (FS) divided into simple, complex, and prolonged FS (also called febrile status epilepticus). Later, this group of disorders has been progressively indicated, with a more inclusive term, as "fever-associated seizures or epilepsy" (FASE) that encompasses: (a) FS divided into simple, complex, and prolonged FS; (b) FS plus; (c) severe myoclonic epilepsy in infancy (Dravet syndrome); (d) genetic epilepsy with FS plus; and (e) febrile infection-related epilepsy syndrome (FIRES). Among the FASE disorders, simple FS, the most common and benign condition, is rarely associated with subsequent epileptic seizures. The correlation of FS with epilepsy and other neurological disorders is highly variable. The pathogenesis of FASE is unclear but immunological and genetic factors play a relevant role and the disorders belonging to the FASE group show to have an underlying common clinical, immunological, and genetic pathway. In this study, we have reviewed and analyzed the clinical data of each of the heterogeneous group of disorders belonging to FASE.
除中枢神经系统感染外，癫痫发作和发热还可能同时出现在多种神经系统疾病中。以前，根据临床特征和预后演变，癫痫发作和发热的联合包括经典的热性惊厥（FS），分为简单型、复杂型和延长型FS（也称为热性癫痫持续状态）。后来，这组疾病逐渐被用一个更具包容性的术语“发热相关性癫痫发作或癫痫”（FASE）来表示，它包括：（a）分为简单型、复杂型和延长型FS的热性惊厥；（b）热性惊厥附加症；（c）婴儿严重肌阵挛性癫痫（Dravet综合征）；（d）伴有热性惊厥附加症的遗传性癫痫；以及（e）发热感染相关性癫痫综合征（FIRES）。在FASE疾病中，简单型FS是最常见且良性的情况，很少与随后的癫痫发作相关。热性惊厥与癫痫和其他神经系统疾病的相关性差异很大。FASE的发病机制尚不清楚，但免疫和遗传因素起着相关作用，属于FASE组的疾病显示出有潜在的共同临床、免疫和遗传途径。在本研究中，我们回顾并分析了属于FASE的每组异质性疾病的临床数据。
