Ferreira João Henrique Fregadolli, Gomes Anna Maria, Zetehaku Ana Carolina, Marinho Taissa Ferrari, Toso Fabio Fieni, Disserol Caio César Diniz, Krueger Mariana Braatz, Santos Mara Lúcia Schmitz Ferreira, Juliano Aline Freire Borges, Barbosa Tércio Luz, de Almeida Rocha Letícia Januzi, Gameleira Fernando Tenório, Paolilo Renata Barbosa, Daccach Vanessa, Gomes João Pedro Izidoro, Lin Katia, da Nóbrega Adaucto Wanderley, Marmentini Emily Lima, Fusão Eduardo Ferracioli, de Cuffa Anderson, Tavares Georgia Lelis Aranha, Dias Ronaldo Maciel, Diniz Sabrina Stephanie Lana, Siquineli Fábio, Braga-Neto Pedro, Nobrega Paulo Ribeiro, Sanders Lorena Pitombeira, Carvalho Fernanda Martins Maia, Pereira Renata Brasileiro Reis, de Melo Eduardo Sousa, de Miranda Henriques-Souza Adélia Maria, de Oliveira Godeiro Clecio, Arambula Omar Gurrola, de Souza E Silva Filipe Nolasco, Couto Karla Oliveira, Danieli Dayane, Seitz Katia Werneck, Dellavance Alessandra, Endmayr Verena, Höftberger Romana, Andrade Luis Eduardo Coelho, Caboclo Luis Otavio, Dutra Livia Almeida
Instituto do Cérebro, Hospital Israelita Albert Einstein, São Paulo, Brazil.
Hospital de Clínicas da Universidade Federal do Paraná, Curitiba, Brazil.
Epilepsia. 2025 May 2. doi: 10.1111/epi.18374.
This study was undertaken to describe a case series of Brazilian new onset refractory status epilepticus (NORSE) patients and evaluate the sensitivity and specificity of a clinical score to predict cryptogenic etiology (c-NORSE score) after autoimmune encephalitis (AE) testing.
Thirty-seven patients with NORSE from the Brazilian Autoimmune Encephalitis Network were investigated with brain magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) analysis, and complementary testing with tissue-based assays and cell-based assays for antineuronal and antiglial antibodies (abs) in serum/CSF. Final diagnoses were compiled after chart review. Patients were allocated into two groups according to c-NORSE score: (≥5) high score (HS) or (<5) low score (LS), and clinical variables were compared. c-NORSE score sensitivity and specificity were calculated.
We found that 23 (62%) of the NORSE patients were children, 49% were female, 22% had AE, and 51% were classified as c-NORSE. Eleven patients (30%) were allocated to the HS group and 26 (70%) to LS. Bilateral and symmetric MRI findings were more frequent in the HS group (HS 100% vs. LS 43%, p = .018), whereas memory or behavioral symptoms were less common (HS 27% vs. LS 89%, p = .001). All HS patients had c-NORSE, whereas 69% of the LS patients had other diagnoses, such as AE (n = 8), herpes simplex virus encephalitis (n = 4), paraneoplastic encephalomyelitis (n = 1), acute disseminated encephalomyelitis (n = 1), and other causes (n = 4). The sensitivity of the c-NORSE score for predicting cryptogenic cases was 57.9% (95% confidence interval [CI] = 36%-80%), and the specificity was 100% (95% CI = 84%-100%).
In this cohort, AE was the most identified cause of NORSE, and 51% were cryptogenic. c-NORSE score ≥ 5 had a specificity of 100% (95% CI = 84%-100%) for identifying cryptogenic cases, whereas a score < 5 indicates additional investigation should be ordered. Although the sensitivity of the c-NORSE score was lower than previously reported, suggesting it may vary depending on complementary investigation, it is a useful tool for bedside NORSE evaluation in low-income countries, where access to antineuronal abs is limited.
本研究旨在描述一组巴西新发难治性癫痫持续状态（NORSE）患者的病例系列，并评估一种临床评分在自身免疫性脑炎（AE）检测后预测隐源性病因（c-NORSE评分）的敏感性和特异性。
对来自巴西自身免疫性脑炎网络的37例NORSE患者进行了脑磁共振成像（MRI）、脑脊液（CSF）分析，并通过基于组织的检测和基于细胞的检测对血清/CSF中的抗神经元和抗神经胶质抗体（abs）进行补充检测。经病历审查后汇总最终诊断结果。根据c-NORSE评分将患者分为两组：（≥5）高分（HS）组或（<5）低分（LS）组，并比较临床变量。计算c-NORSE评分的敏感性和特异性。
我们发现，23例（62%）NORSE患者为儿童，49%为女性，22%患有AE，51%被归类为c-NORSE。11例患者（30%）被分配到HS组，26例（70%）被分配到LS组。双侧和对称的MRI表现在HS组中更为常见（HS组为100%，LS组为43%，p = 0.018），而记忆或行为症状则较少见（HS组为27%，LS组为89%，p = 0.001）。所有HS患者均为c-NORSE，而69%的LS患者有其他诊断，如AE（n = 8）、单纯疱疹病毒性脑炎（n = 4）、副肿瘤性脑脊髓炎（n = 1）、急性播散性脑脊髓炎（n = 1）和其他病因（n = 4）。c-NORSE评分预测隐源性病例的敏感性为57.9%（95%置信区间[CI]=36%-80%），特异性为100%（95%CI = 84%-100%）。
在该队列中，AE是NORSE最常见的病因，51%为隐源性。c-NORSE评分≥5对识别隐源性病例的特异性为100%（95%CI = 84%-100%），而评分<5表明应进行进一步检查。尽管c-NORSE评分的敏感性低于先前报道，提示其可能因补充检查而异，但在获得抗神经元abs受限的低收入国家，它是床边NORSE评估的有用工具。
