Department. of Neurology, Comprehensive Epilepsy Center, Yale University School of Medicine, New Haven, Connecticut, USA.
Department of Neurology and Immunobiology, Yale University School of Medicine, New Haven, Connecticut, USA.
Epilepsia. 2023 Jun;64(6):e112-e117. doi: 10.1111/epi.17606. Epub 2023 Apr 13.
The etiology of new-onset refractory status epilepticus (NORSE), including its subtype with prior fever known as FIRES (febrile infection-related epilepsy syndrome), remains uncertain. Several arguments suggest that NORSE is a disorder of immunity, likely post-infectious. Consequently, seasonal occurrence might be anticipated. Herein we investigated if seasonality is a notable factor regarding NORSE presentation. We combined four different data sets with a total of 342 cases, all from the northern hemisphere, and 62% adults. The incidence of NORSE cases differed between seasons (p = .0068) and was highest in the summer (32.2%) (p = .0022) and lowest in the spring (19.0%, p = .010). Although both FIRES and non-FIRES cases occurred most commonly during the summer, there was a trend toward FIRES cases being more likely to occur in the winter than non-FIRES cases (OR 1.62, p = .071). The seasonality of NORSE cases differed according to the etiology (p = .024). NORSE cases eventually associated with autoimmune/paraneoplastic encephalitis occurred most frequently in the summer (p = .032) and least frequently in the winter (p = .047), whereas there was no seasonality for cryptogenic cases. This study suggests that NORSE overall and NORSE related to autoimmune/paraneoplastic encephalitis are more common in the summer, but that there is no definite seasonality in cryptogenic cases.
新发生的耐药性癫痫持续状态(NORSE)的病因,包括其先前有发热的亚型,即 FIRES(热性感染相关癫痫综合征),仍不确定。有几个论点表明,NORSE 是一种免疫紊乱,可能与感染后有关。因此,可以预期季节性发生。在此,我们研究了季节是否是 NORSE 发作的一个显著因素。我们合并了四个不同的数据组,共有 342 例病例,均来自北半球,62%为成年人。NORSE 病例的发病有季节性差异(p=0.0068),夏季最高(32.2%)(p=0.0022),春季最低(19.0%)(p=0.010)。尽管 FIRES 和非 FIRES 病例最常见于夏季,但 FIRES 病例在冬季发生的可能性较非 FIRES 病例高,有一定趋势(OR 1.62,p=0.071)。根据病因,NORSE 病例的季节性不同(p=0.024)。最终与自身免疫/副肿瘤性脑炎相关的 NORSE 病例最常发生在夏季(p=0.032),最不常发生在冬季(p=0.047),而原因不明的病例则无季节性。本研究表明,总体而言,NORSE 以及与自身免疫/副肿瘤性脑炎相关的 NORSE 在夏季更为常见,但原因不明的病例则无明确的季节性。