Abstract Details

Title Performance of c-NORSE Score in a Brazilian Cohort After Testing for Antineuronal Antibodies with Tissue-based and Cell-based Assays

Topic Epilepsy/Clinical Neurophysiology (EEG)

Presentation(s) P4 - Poster Session 4 (5:00 PM-6:00 PM)

Poster/Presentation
Number 9-003

Objective To describe a cohort of Brazilian NORSE patients and assess the sensitivity and specificity of a clinical score for early cryptogenic etiology prediction (c-NORSE score) after autoimmune encephalitis (AE) testing.

Background AE is a common cause of NORSE, but accurate estimates are lacking. Early recognition of NORSE etiology can impact treatment decisions.

Design/Methods Forty NORSE patients from the Brazilian Autoimmune Encephalitis Network (BrAIN) were investigated with brain MRI, CSF analysis, and TBA/CBAs for antineuronal antibodies (abs) in serum and CSF. Final diagnoses were compiled after chart review. Patients were divided into two groups by 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.

Results Of the 40 NORSE patients, 23 (57,5%) were children, 52% were female, 22.5% had AE, and 47.5% were c-NORSE. Eleven patients (27%) were in the HS group and 29 (73%) in the LS group. Bilateral and symmetric MRI findings were more frequent in HS group (HS 100% vs. LS 35%, p=0.003), while memory/behavioral symptoms were less common (HS 27% vs. LS 83%, p=0.002). All HS patients had c-NORSE, while 72% of the LS patients had other diagnoses, such as AE (n=9), HSV encephalitis (n=4), encephalomyelitis (n=1), ADEM (n=1), and other causes (n=5). The sensitivity of the c-NORSE score for predicting cryptogenic cases was 57.9% (95% CI: 36.3%-76.9%), and the specificity was 100% (95% CI: 84.5%-100%.

Conclusions In this cohort, most cases were cryptogenic and 22.5% had AE. C-NORSE score ≥5 was 100% specific for identifying cryptogenic cases, while a score <5 indicates further investigation is needed. Although the sensitivity of the c-NORSE was lower than previously reported, likely due to variations on complementary investigation performed, it remains a valuable bedside tool for NORSE evaluation in low-income countries with limited access to antineuronal abs testing.

Authors/Disclosures
João Henrique F. Fregadolli Ferreira, MD PRESENTER	Mr. Ferreira has nothing to disclose.
Anna M. Gomes, MD	Dr. Gomes has nothing to disclose.
Ana C. Zetehaku, MD	Mrs. Zetehaku has nothing to disclose.
Taissa Ferrari Marinho, PhD	Dr. Ferrari Marinho has nothing to disclose.
Fabio F. Toso, MD (Universidade Federal de São Paulo, São Paulo, Brazil)	Dr. Toso has nothing to disclose.
Caio Disserol, MD	Dr. Disserol has nothing to disclose.
Mariana B. Krueger, PhD	An immediate family member of Dr. Krueger has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Libbs.
Mara Lucia Schmitz Santos, MD	Prof. Santos has nothing to disclose.
Aline B. Juliano, MD	Mrs. Juliano has nothing to disclose.
Tércio L. Barbosa, Sr., Esp	Dr. Barbosa has nothing to disclose.
Letícia J. Rocha, PhD	Prof. Rocha has nothing to disclose.
Fernando Gamaleira	Fernando Gamaleira has nothing to disclose.
Renata Paolilo (University of São Paulo)	Renata Paolilo has nothing to disclose.
Vanessa Daccach Marques	Vanessa Daccach Marques has nothing to disclose.
Joao Pedro I. Gomes, MD (Hospital de Base)	Dr. Gomes has nothing to disclose.
Katia Lin, MD, PhD	Prof. Lin has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Aché. Prof. Lin has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Eurofarma. Prof. Lin has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Prati-Donaduzzi. Prof. Lin has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Abbott. Prof. Lin has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Adium. Prof. Lin has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for UCB. Prof. Lin has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Torrent. Prof. Lin has received research support from CNPq. Prof. Lin has received research support from FAPESC. Prof. Lin has received research support from CAPES. Prof. Lin has received publishing royalties from a publication relating to health care. Prof. Lin has received publishing royalties from a publication relating to health care. Prof. Lin has received publishing royalties from a publication relating to health care. Prof. Lin has received publishing royalties from a publication relating to health care. Prof. Lin has received publishing royalties from a publication relating to health care. Prof. Lin has a non-compensated relationship as a President with Brazilian Epilepsy League that is relevant to AAN interests or activities. Prof. Lin has a non-compensated relationship as a Chair Prioritization Task Force with International League Against Epilepsy that is relevant to AAN interests or activities.
Adaucto Nóbrega Junior, MD	Dr. Nóbrega Junior has nothing to disclose.
Emily L. Marmentini, Student	Miss Marmentini has nothing to disclose.
Anderson De Cuffa, MD (Unimed Grande Florianopolis)	Dr. De Cuffa has nothing to disclose.
GEORGIA L. TAVARES, Sr., PhD	Miss TAVARES has nothing to disclose.
Ronaldo Dias, MD (Hospital de Base)	Dr. Dias has nothing to disclose.
Sabrina S. Diniz, MD	Mrs. Diniz has nothing to disclose.
Fabio Siquineli, MD (Hospital Santa Isabel)	Dr. Siquineli has nothing to disclose.
Pedro Braga-Neto, MD, PhD (Universidade Federal Do Ceara)	Dr. Braga-Neto has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Dr. Braga-Neto has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biogen.
Paulo R. Nobrega, MD (Hospital Universitario Walter Cantidio)	Dr. Nobrega has nothing to disclose.
Lorena Sanders, MD	Dr. Sanders has nothing to disclose.
Fernanda M. Maia, MD, PhD (Hospital General De Fortaleza)	Dr. Maia has nothing to disclose.
Renata B. Pereira II, MD	Mrs. Pereira has nothing to disclose.
Eduardo S. Melo, PhD	Dr. Melo has nothing to disclose.
ADÉLIA MARIA DE MIRANDA d. HENRIQUES-SOUZA, MD, PhD	Dr. HENRIQUES-SOUZA has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for ADIUM. Dr. HENRIQUES-SOUZA has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. Dr. HENRIQUES-SOUZA has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for APSEN. Dr. HENRIQUES-SOUZA has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for ADIUM.
Clecio O. Godeiro, Jr., MD, PhD (UFRN)	Dr. Godeiro has nothing to disclose.
ADALIMARA S. BARBOSA, MD	Ms. BARBOSA has nothing to disclose.
Filipe Nolasco de Souza e Silva, MD	Mr. Nolasco de Souza e Silva has nothing to disclose.
Karla Couto, MD	Dr. Couto has nothing to disclose.
Dayane Danieli, MD (Instituto Crianca)	Ms. Danieli has nothing to disclose.
Katia W. Seitz	Mrs. Seitz has nothing to disclose.
Alessandra D. Dellavance, PhD	Mrs. Dellavance has nothing to disclose.
Luis Eduardo C. Andrade, MD, PhD	Prof. Andrade has nothing to disclose.
LUIS O. CABOCLO, MD, PhD (Hospital Israelita Albert Einstein)	Dr. CABOCLO has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB. Dr. CABOCLO has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Libbs Phamaceutical. Dr. CABOCLO has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for UCB. Dr. CABOCLO has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Adium Pharmaceutical. Dr. CABOCLO has received personal compensation in the range of $0-$499 for serving as an officer or member of the Board of Directors for Endogen Pharmaceutical (Brazil). Dr. CABOCLO has stock in Endogen Pharmaceutical (Brazil).
Livia A. Dutra, MD (Hospital Israelita Albert Einstein)	Dr. Dutra has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kyverna. The institution of Dr. Dutra has received research support from Hospital Israelita Albert Einstein. The institution of Dr. Dutra has received research support from Laboratório Fleury. Dr. Dutra has received personal compensation in the range of $0-$499 for serving as a Speaker with Roche.

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