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Retrospective Pediatric Cohort Study Validates NEOS Score and Demonstrates Applicability in Children With Anti-NMDAR Encephalitis

BACKGROUND AND OBJECTIVES: Anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) is the most common form of autoimmune encephalitis in children and adults. Although our understanding of the disease mechanisms has progressed, little is known about estimating patient outcomes. Therefore, the NEOS (...

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Autores principales: Nikolaus, Marc, Rausch, Philipp, Rostásy, Kevin, Bertolini, Annikki, Wickström, Ronny, Johannsen, Jessika, Denecke, Jonas, Breu, Markus, Schimmel, Mareike, Diepold, Katharina, Haeusler, Martin, Quade, Annegret, Berger, Andrea, Rosewich, Hendrik, Steen, Claudia, von Au, Katja, Dreesmann, Mona, Finke, Carsten, Bartels, Frederik, Kaindl, Angela M., Schuelke, Markus, Knierim, Ellen
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10032577/
https://www.ncbi.nlm.nih.gov/pubmed/36948591
http://dx.doi.org/10.1212/NXI.0000000000200102
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author Nikolaus, Marc
Rausch, Philipp
Rostásy, Kevin
Bertolini, Annikki
Wickström, Ronny
Johannsen, Jessika
Denecke, Jonas
Breu, Markus
Schimmel, Mareike
Diepold, Katharina
Haeusler, Martin
Quade, Annegret
Berger, Andrea
Rosewich, Hendrik
Steen, Claudia
von Au, Katja
Dreesmann, Mona
Finke, Carsten
Bartels, Frederik
Kaindl, Angela M.
Schuelke, Markus
Knierim, Ellen
author_facet Nikolaus, Marc
Rausch, Philipp
Rostásy, Kevin
Bertolini, Annikki
Wickström, Ronny
Johannsen, Jessika
Denecke, Jonas
Breu, Markus
Schimmel, Mareike
Diepold, Katharina
Haeusler, Martin
Quade, Annegret
Berger, Andrea
Rosewich, Hendrik
Steen, Claudia
von Au, Katja
Dreesmann, Mona
Finke, Carsten
Bartels, Frederik
Kaindl, Angela M.
Schuelke, Markus
Knierim, Ellen
author_sort Nikolaus, Marc
collection PubMed
description BACKGROUND AND OBJECTIVES: Anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) is the most common form of autoimmune encephalitis in children and adults. Although our understanding of the disease mechanisms has progressed, little is known about estimating patient outcomes. Therefore, the NEOS (anti-NMDAR Encephalitis One-Year Functional Status) score was introduced as a tool to predict disease progression in NMDARE. Developed in a mixed-age cohort, it currently remains unclear whether NEOS can be optimized for pediatric NMDARE. METHODS: This retrospective observational study aimed to validate NEOS in a large pediatric-only cohort of 59 patients (median age of 8 years). We reconstructed the original score, adapted it, evaluated additional variables, and assessed its predictive power (median follow-up of 20 months). Generalized linear regression models were used to examine predictability of binary outcomes based on the modified Rankin Scale (mRS). In addition, neuropsychological test results were investigated as alternative cognitive outcome. RESULTS: The NEOS score reliably predicted poor clinical outcome (mRS ≥3) in children in the first year after diagnosis (p = 0.0014) and beyond (p = 0.036, 16 months after diagnosis). A score adapted to the pediatric cohort by adjusting the cutoffs of the 5 NEOS components did not improve predictive power. In addition to these 5 variables, further patient characteristics such as the “Herpes simplex virus encephalitis (HSE) status” and “age at disease onset” influenced predictability and could potentially be useful to define risk groups. NEOS also predicted cognitive outcome with higher scores associated with deficits of executive function (p = 0.048) and memory (p = 0.043). DISCUSSION: Our data support the applicability of the NEOS score in children with NMDARE. Although not yet validated in prospective studies, NEOS also predicted cognitive impairment in our cohort. Consequently, the score could help identify patients at risk of poor overall clinical outcome and poor cognitive outcome and thus aid in selecting not only optimized initial therapies for these patients but also cognitive rehabilitation to improve long-term outcomes.
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spelling pubmed-100325772023-03-23 Retrospective Pediatric Cohort Study Validates NEOS Score and Demonstrates Applicability in Children With Anti-NMDAR Encephalitis Nikolaus, Marc Rausch, Philipp Rostásy, Kevin Bertolini, Annikki Wickström, Ronny Johannsen, Jessika Denecke, Jonas Breu, Markus Schimmel, Mareike Diepold, Katharina Haeusler, Martin Quade, Annegret Berger, Andrea Rosewich, Hendrik Steen, Claudia von Au, Katja Dreesmann, Mona Finke, Carsten Bartels, Frederik Kaindl, Angela M. Schuelke, Markus Knierim, Ellen Neurol Neuroimmunol Neuroinflamm Research Article BACKGROUND AND OBJECTIVES: Anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) is the most common form of autoimmune encephalitis in children and adults. Although our understanding of the disease mechanisms has progressed, little is known about estimating patient outcomes. Therefore, the NEOS (anti-NMDAR Encephalitis One-Year Functional Status) score was introduced as a tool to predict disease progression in NMDARE. Developed in a mixed-age cohort, it currently remains unclear whether NEOS can be optimized for pediatric NMDARE. METHODS: This retrospective observational study aimed to validate NEOS in a large pediatric-only cohort of 59 patients (median age of 8 years). We reconstructed the original score, adapted it, evaluated additional variables, and assessed its predictive power (median follow-up of 20 months). Generalized linear regression models were used to examine predictability of binary outcomes based on the modified Rankin Scale (mRS). In addition, neuropsychological test results were investigated as alternative cognitive outcome. RESULTS: The NEOS score reliably predicted poor clinical outcome (mRS ≥3) in children in the first year after diagnosis (p = 0.0014) and beyond (p = 0.036, 16 months after diagnosis). A score adapted to the pediatric cohort by adjusting the cutoffs of the 5 NEOS components did not improve predictive power. In addition to these 5 variables, further patient characteristics such as the “Herpes simplex virus encephalitis (HSE) status” and “age at disease onset” influenced predictability and could potentially be useful to define risk groups. NEOS also predicted cognitive outcome with higher scores associated with deficits of executive function (p = 0.048) and memory (p = 0.043). DISCUSSION: Our data support the applicability of the NEOS score in children with NMDARE. Although not yet validated in prospective studies, NEOS also predicted cognitive impairment in our cohort. Consequently, the score could help identify patients at risk of poor overall clinical outcome and poor cognitive outcome and thus aid in selecting not only optimized initial therapies for these patients but also cognitive rehabilitation to improve long-term outcomes. Lippincott Williams & Wilkins 2023-03-22 /pmc/articles/PMC10032577/ /pubmed/36948591 http://dx.doi.org/10.1212/NXI.0000000000200102 Text en Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND) (https://creativecommons.org/licenses/by-nc-nd/4.0/) , which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.
spellingShingle Research Article
Nikolaus, Marc
Rausch, Philipp
Rostásy, Kevin
Bertolini, Annikki
Wickström, Ronny
Johannsen, Jessika
Denecke, Jonas
Breu, Markus
Schimmel, Mareike
Diepold, Katharina
Haeusler, Martin
Quade, Annegret
Berger, Andrea
Rosewich, Hendrik
Steen, Claudia
von Au, Katja
Dreesmann, Mona
Finke, Carsten
Bartels, Frederik
Kaindl, Angela M.
Schuelke, Markus
Knierim, Ellen
Retrospective Pediatric Cohort Study Validates NEOS Score and Demonstrates Applicability in Children With Anti-NMDAR Encephalitis
title Retrospective Pediatric Cohort Study Validates NEOS Score and Demonstrates Applicability in Children With Anti-NMDAR Encephalitis
title_full Retrospective Pediatric Cohort Study Validates NEOS Score and Demonstrates Applicability in Children With Anti-NMDAR Encephalitis
title_fullStr Retrospective Pediatric Cohort Study Validates NEOS Score and Demonstrates Applicability in Children With Anti-NMDAR Encephalitis
title_full_unstemmed Retrospective Pediatric Cohort Study Validates NEOS Score and Demonstrates Applicability in Children With Anti-NMDAR Encephalitis
title_short Retrospective Pediatric Cohort Study Validates NEOS Score and Demonstrates Applicability in Children With Anti-NMDAR Encephalitis
title_sort retrospective pediatric cohort study validates neos score and demonstrates applicability in children with anti-nmdar encephalitis
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10032577/
https://www.ncbi.nlm.nih.gov/pubmed/36948591
http://dx.doi.org/10.1212/NXI.0000000000200102
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