New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) of unknown aetiology: A comparison of the incomparable?

被引:4
作者
Nausch, Eiko [1 ]
Schaffeldt, Lisa [2 ]
Tautorat, Insa [2 ]
Margraf, Nils Gerd [2 ]
Haeusler, Martin [3 ]
Kluger, Gerhard [4 ,5 ]
Kellinghaus, Christoph [6 ]
Borzikowsky, Christoph [7 ]
Laufs, Helmut [2 ]
van Baalen, Andreas [1 ]
机构
[1] Kiel Univ CAU, Univ Med Ctr Schleswig Holstein, Dept Neuropediat, Arnold Heller St 3,House C, D-24105 Kiel, Germany
[2] Kiel Univ CAU, Univ Med Ctr Schleswig Holstein, Dept Neurol, Kiel, Germany
[3] Univ Hosp, Dept Paediat, Div Neuropediat & Social Pediat, RWTH Aachen, Aachen, Germany
[4] Schon Clin Vogtareuth, Epilepsy Ctr Children & Adolescents, Clin Neuropediat & Neurorehabil, Vogtareuth, Germany
[5] PMU Salzburg, Res Inst Rehabil Transit & Palliat, Salzburg, Austria
[6] Clin Osnabruck, Epilepsy Ctr Munster Osnabruck, Osnabruck, Germany
[7] Kiel Univ CAU, Univ Med Ctr Schleswig Holstein, Inst Med Informat & Stat, Kiel, Germany
来源
SEIZURE-EUROPEAN JOURNAL OF EPILEPSY | 2022年 / 96卷
关键词
Etiology; Definition; Epileptic encephalopathy; Fever;
D O I
10.1016/j.seizure.2022.01.006
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Purpose: The discussion is ongoing whether new-onset refractory status epilepticus (NORSE) in adults and febrile infection-related epilepsy syndrome (FIRES) in children are one syndrome if the aetiology is unknown. In this study we will compare an adult cohort with NORSE and a paediatric cohort with FIRES in order to determine if they are similar or different. Methods: We retrospectively compared 18 adults with NORSE versus 48 children with FIRES, both cohorts without identifiable cause despite extensive investigations. We analyzed demographic and clinical data using Mann-Whitney-U and chi(2)- tests. Results: NORSE affected more women (78% vs. 42%; P = 0.009) than in FIRES. Median acute hospital stay was longer in FIRES (35 days [interquartile range, IQR=36] vs. 20 days [IQR=19]; P<0.001). FIRES was treated more frequently with coma therapy (82% vs. 28%; P<0.001) and with a higher median number of antiseizure medicines (7 [IQR=5] vs. 4 [IQR=2]; P<0.001). Children with FIRES showed a higher cerebrospinal fluid (CSF) cell count (10 cells/mu l; P = 0.002) but a lower CSF protein level than adults with NORSE (48 mg/dl; P = 0.028). Immunotherapy was administered more frequently in FIRES (73% vs. 22%; P<0.001) than in NORSE. Group differences in number of antiseizure medicines after hospital stay (P = 0.229) and in overall mortality (P = 0.327) were not significant. Conclusion: In our explorative comparison, differences prevailed. NORSE and FIRES should be compared prospectively in age-matched cohorts.
引用
收藏
页码:18 / 21
页数:4
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