Differential diagnosis and comparison of diagnostic algorithms in children and adolescents with autoimmune encephalitis in Spain: a prospective cohort study and retrospective analysis
The usefulness of current diagnostic approaches in children with suspected autoimmune encephalitis is unknown. We aimed to assess the diagnosis of autoimmune encephalitis in clinical practice and to compare the performance of two international diagnostic algorithms (one intended for patients of any...
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          | Published in | Lancet neurology Vol. 24; no. 1; pp. 54 - 64 | 
|---|---|
| Main Authors | , , , , , , , , , , , , , , , , , , , , , , , , , , , , | 
| Format | Journal Article | 
| Language | English | 
| Published | 
        England
          Elsevier Ltd
    
        01.01.2025
     Elsevier Limited  | 
| Subjects | |
| Online Access | Get full text | 
| ISSN | 1474-4422 1474-4465 1474-4465  | 
| DOI | 10.1016/S1474-4422(24)00443-5 | 
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| Abstract | The usefulness of current diagnostic approaches in children with suspected autoimmune encephalitis is unknown. We aimed to assess the diagnosis of autoimmune encephalitis in clinical practice and to compare the performance of two international diagnostic algorithms (one intended for patients of any age [general], the other intended for paediatric patients), with particular emphasis on the evaluation of patients with probable antibody-negative autoimmune encephalitis because this diagnosis suggests that immunotherapy should be continued or escalated but is difficult to establish.
We did a prospective cohort study that included all patients (<18 years of age) with suspected autoimmune encephalitis recruited at 40 hospitals in Spain whose physicians provided clinical information every 6 months for 2 years or more. Neural antibody testing to confirm diagnosis of antibody-positive autoimmune encephalitis was done at Institut d’Investigacions Biomèdiques August Pi i Sunyer-Hospital Clínic, Barcelona. Patients were classified according to the most probable diagnosis at last follow-up into four prespecified categories. We used multivariable logistic analysis to assess a potential association between immunotherapy and outcome in individuals with probable antibody-negative autoimmune encephalitis. We also did a retrospective analysis of agreement, assessed with the kappa index, between diagnoses made according to the general and paediatric diagnostic algorithms.
Between June 1, 2013, and May 31, 2021, 729 children (mean age 7·1 years [SD 4·9]; 383 boys [53%], 346 girls [47%]) with suspected autoimmune encephalitis were recruited. After a median follow-up of 36 months (IQR 26–60), patients were classified according to their most probable diagnosis: definite autoimmune encephalitis or well defined inflammatory or autoimmune disorders (n=230 [32%]); CNS infections (n=112 [15%]); inflammatory CNS disorders of unknown cause (n=81 [11%], including three (4%) with a novel Klüver-Bucy-like syndrome; and non-inflammatory disorders (n=306 [42%]), which were predominantly epileptic or psychiatric disorders (177 [58%] of 306). Neural antibodies were detected in 150 (65%) of 230 patients who had definite autoimmune encephalitis; 127 (85%) of these 150 individuals had antibodies to the NMDA receptor or myelin oligodendrocyte glycoprotein (MOG). Agreement between algorithms was excellent (kappa index 0·99, 95% CI 0·97–1·00) for the diagnosis of children with antibody-positive autoimmune encephalitis, good (0·59, 0·54–0·65) for recommendations of empiric immunotherapy, and poor (0·29, 0·21–0·37) for the diagnosis of probable antibody-negative autoimmune encephalitis. Compared with the general algorithm, the paediatric algorithm included more patients in the probable antibody-negative autoimmune encephalitis category (173 vs 41). These patients included some of those who had a diagnosis of CNS inflammatory disorder of unknown cause at the last follow-up (80 of 81 with the paediatric algorithm vs 31 of 81 with the general algorithm), who might have benefitted from immunotherapy, and some of those diagnosed with a non-inflammatory disorder at the last follow-up (47 of 306 with the paediatric algorithm vs six of 306 with the general algorithm), who did not need immunotherapy.
About a third of children with suspected autoimmune encephalitis eventually had confirmation of this diagnosis, or diagnosis of another well defined inflammatory disorder. Frequent mimics of autoimmune encephalitis were infectious, epileptic, and psychiatric disorders. Both algorithms performed well in the diagnosis of antibody-positive autoimmune encephalitis, but the paediatric algorithm under-recognised definite autoimmune encephalitis that can occur without autoantibodies and might have overdiagnosed patients with probable antibody-negative autoimmune encephalitis. By contrast, the general algorithm might have underdiagnosed patients with probable antibody-negative autoimmune encephalitis. Given that the diagnosis of probable antibody-negative autoimmune encephalitis has treatment implications, inaccuracies on this diagnostic category leads to overuse or underuse of immunotherapy.
Instituto de Salud Carlos III, Fundació Clínic per la Recerca Biomèdica, The Edmond J Safra Foundation, and la Caixa Foundation.
For the Spanish translation of the abstract see Supplementary Materials section. | 
    
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| AbstractList | The usefulness of current diagnostic approaches in children with suspected autoimmune encephalitis is unknown. We aimed to assess the diagnosis of autoimmune encephalitis in clinical practice and to compare the performance of two international diagnostic algorithms (one intended for patients of any age [general], the other intended for paediatric patients), with particular emphasis on the evaluation of patients with probable antibody-negative autoimmune encephalitis because this diagnosis suggests that immunotherapy should be continued or escalated but is difficult to establish.
We did a prospective cohort study that included all patients (<18 years of age) with suspected autoimmune encephalitis recruited at 40 hospitals in Spain whose physicians provided clinical information every 6 months for 2 years or more. Neural antibody testing to confirm diagnosis of antibody-positive autoimmune encephalitis was done at Institut d'Investigacions Biomèdiques August Pi i Sunyer-Hospital Clínic, Barcelona. Patients were classified according to the most probable diagnosis at last follow-up into four prespecified categories. We used multivariable logistic analysis to assess a potential association between immunotherapy and outcome in individuals with probable antibody-negative autoimmune encephalitis. We also did a retrospective analysis of agreement, assessed with the kappa index, between diagnoses made according to the general and paediatric diagnostic algorithms.
Between June 1, 2013, and May 31, 2021, 729 children (mean age 7·1 years [SD 4·9]; 383 boys [53%], 346 girls [47%]) with suspected autoimmune encephalitis were recruited. After a median follow-up of 36 months (IQR 26-60), patients were classified according to their most probable diagnosis: definite autoimmune encephalitis or well defined inflammatory or autoimmune disorders (n=230 [32%]); CNS infections (n=112 [15%]); inflammatory CNS disorders of unknown cause (n=81 [11%], including three (4%) with a novel Klüver-Bucy-like syndrome; and non-inflammatory disorders (n=306 [42%]), which were predominantly epileptic or psychiatric disorders (177 [58%] of 306). Neural antibodies were detected in 150 (65%) of 230 patients who had definite autoimmune encephalitis; 127 (85%) of these 150 individuals had antibodies to the NMDA receptor or myelin oligodendrocyte glycoprotein (MOG). Agreement between algorithms was excellent (kappa index 0·99, 95% CI 0·97-1·00) for the diagnosis of children with antibody-positive autoimmune encephalitis, good (0·59, 0·54-0·65) for recommendations of empiric immunotherapy, and poor (0·29, 0·21-0·37) for the diagnosis of probable antibody-negative autoimmune encephalitis. Compared with the general algorithm, the paediatric algorithm included more patients in the probable antibody-negative autoimmune encephalitis category (173 vs 41). These patients included some of those who had a diagnosis of CNS inflammatory disorder of unknown cause at the last follow-up (80 of 81 with the paediatric algorithm vs 31 of 81 with the general algorithm), who might have benefitted from immunotherapy, and some of those diagnosed with a non-inflammatory disorder at the last follow-up (47 of 306 with the paediatric algorithm vs six of 306 with the general algorithm), who did not need immunotherapy.
About a third of children with suspected autoimmune encephalitis eventually had confirmation of this diagnosis, or diagnosis of another well defined inflammatory disorder. Frequent mimics of autoimmune encephalitis were infectious, epileptic, and psychiatric disorders. Both algorithms performed well in the diagnosis of antibody-positive autoimmune encephalitis, but the paediatric algorithm under-recognised definite autoimmune encephalitis that can occur without autoantibodies and might have overdiagnosed patients with probable antibody-negative autoimmune encephalitis. By contrast, the general algorithm might have underdiagnosed patients with probable antibody-negative autoimmune encephalitis. Given that the diagnosis of probable antibody-negative autoimmune encephalitis has treatment implications, inaccuracies on this diagnostic category leads to overuse or underuse of immunotherapy.
Instituto de Salud Carlos III, Fundació Clínic per la Recerca Biomèdica, The Edmond J Safra Foundation, and la Caixa Foundation.
For the Spanish translation of the abstract see Supplementary Materials section. Summary Background The usefulness of current diagnostic approaches in children with suspected autoimmune encephalitis is unknown. We aimed to assess the diagnosis of autoimmune encephalitis in clinical practice and to compare the performance of two international diagnostic algorithms (one intended for patients of any age [general], the other intended for paediatric patients), with particular emphasis on the evaluation of patients with probable antibody-negative autoimmune encephalitis because this diagnosis suggests that immunotherapy should be continued or escalated but is difficult to establish. Methods We did a prospective cohort study that included all patients (<18 years of age) with suspected autoimmune encephalitis recruited at 40 hospitals in Spain whose physicians provided clinical information every 6 months for 2 years or more. Neural antibody testing to confirm diagnosis of antibody-positive autoimmune encephalitis was done at Institut d’Investigacions Biomèdiques August Pi i Sunyer-Hospital Clínic, Barcelona. Patients were classified according to the most probable diagnosis at last follow-up into four prespecified categories. We used multivariable logistic analysis to assess a potential association between immunotherapy and outcome in individuals with probable antibody-negative autoimmune encephalitis. We also did a retrospective analysis of agreement, assessed with the kappa index, between diagnoses made according to the general and paediatric diagnostic algorithms. Findings Between June 1, 2013, and May 31, 2021, 729 children (mean age 7·1 years [SD 4·9]; 383 boys [53%], 346 girls [47%]) with suspected autoimmune encephalitis were recruited. After a median follow-up of 36 months (IQR 26–60), patients were classified according to their most probable diagnosis: definite autoimmune encephalitis or well defined inflammatory or autoimmune disorders (n=230 [32%]); CNS infections (n=112 [15%]); inflammatory CNS disorders of unknown cause (n=81 [11%], including three (4%) with a novel Klüver-Bucy-like syndrome; and non-inflammatory disorders (n=306 [42%]), which were predominantly epileptic or psychiatric disorders (177 [58%] of 306). Neural antibodies were detected in 150 (65%) of 230 patients who had definite autoimmune encephalitis; 127 (85%) of these 150 individuals had antibodies to the NMDA receptor or myelin oligodendrocyte glycoprotein (MOG). Agreement between algorithms was excellent (kappa index 0·99, 95% CI 0·97–1·00) for the diagnosis of children with antibody-positive autoimmune encephalitis, good (0·59, 0·54–0·65) for recommendations of empiric immunotherapy, and poor (0·29, 0·21–0·37) for the diagnosis of probable antibody-negative autoimmune encephalitis. Compared with the general algorithm, the paediatric algorithm included more patients in the probable antibody-negative autoimmune encephalitis category (173 vs 41). These patients included some of those who had a diagnosis of CNS inflammatory disorder of unknown cause at the last follow-up (80 of 81 with the paediatric algorithm vs 31 of 81 with the general algorithm), who might have benefitted from immunotherapy, and some of those diagnosed with a non-inflammatory disorder at the last follow-up (47 of 306 with the paediatric algorithm vs six of 306 with the general algorithm), who did not need immunotherapy. Interpretation About a third of children with suspected autoimmune encephalitis eventually had confirmation of this diagnosis, or diagnosis of another well defined inflammatory disorder. Frequent mimics of autoimmune encephalitis were infectious, epileptic, and psychiatric disorders. Both algorithms performed well in the diagnosis of antibody-positive autoimmune encephalitis, but the paediatric algorithm under-recognised definite autoimmune encephalitis that can occur without autoantibodies and might have overdiagnosed patients with probable antibody-negative autoimmune encephalitis. By contrast, the general algorithm might have underdiagnosed patients with probable antibody-negative autoimmune encephalitis. Given that the diagnosis of probable antibody-negative autoimmune encephalitis has treatment implications, inaccuracies on this diagnostic category leads to overuse or underuse of immunotherapy. Funding Instituto de Salud Carlos III, Fundació Clínic per la Recerca Biomèdica, The Edmond J Safra Foundation, and la Caixa Foundation. Translation For the Spanish translation of the abstract see Supplementary Materials section. The usefulness of current diagnostic approaches in children with suspected autoimmune encephalitis is unknown. We aimed to assess the diagnosis of autoimmune encephalitis in clinical practice and to compare the performance of two international diagnostic algorithms (one intended for patients of any age [general], the other intended for paediatric patients), with particular emphasis on the evaluation of patients with probable antibody-negative autoimmune encephalitis because this diagnosis suggests that immunotherapy should be continued or escalated but is difficult to establish.BACKGROUNDThe usefulness of current diagnostic approaches in children with suspected autoimmune encephalitis is unknown. We aimed to assess the diagnosis of autoimmune encephalitis in clinical practice and to compare the performance of two international diagnostic algorithms (one intended for patients of any age [general], the other intended for paediatric patients), with particular emphasis on the evaluation of patients with probable antibody-negative autoimmune encephalitis because this diagnosis suggests that immunotherapy should be continued or escalated but is difficult to establish.We did a prospective cohort study that included all patients (<18 years of age) with suspected autoimmune encephalitis recruited at 40 hospitals in Spain whose physicians provided clinical information every 6 months for 2 years or more. Neural antibody testing to confirm diagnosis of antibody-positive autoimmune encephalitis was done at Institut d'Investigacions Biomèdiques August Pi i Sunyer-Hospital Clínic, Barcelona. Patients were classified according to the most probable diagnosis at last follow-up into four prespecified categories. We used multivariable logistic analysis to assess a potential association between immunotherapy and outcome in individuals with probable antibody-negative autoimmune encephalitis. We also did a retrospective analysis of agreement, assessed with the kappa index, between diagnoses made according to the general and paediatric diagnostic algorithms.METHODSWe did a prospective cohort study that included all patients (<18 years of age) with suspected autoimmune encephalitis recruited at 40 hospitals in Spain whose physicians provided clinical information every 6 months for 2 years or more. Neural antibody testing to confirm diagnosis of antibody-positive autoimmune encephalitis was done at Institut d'Investigacions Biomèdiques August Pi i Sunyer-Hospital Clínic, Barcelona. Patients were classified according to the most probable diagnosis at last follow-up into four prespecified categories. We used multivariable logistic analysis to assess a potential association between immunotherapy and outcome in individuals with probable antibody-negative autoimmune encephalitis. We also did a retrospective analysis of agreement, assessed with the kappa index, between diagnoses made according to the general and paediatric diagnostic algorithms.Between June 1, 2013, and May 31, 2021, 729 children (mean age 7·1 years [SD 4·9]; 383 boys [53%], 346 girls [47%]) with suspected autoimmune encephalitis were recruited. After a median follow-up of 36 months (IQR 26-60), patients were classified according to their most probable diagnosis: definite autoimmune encephalitis or well defined inflammatory or autoimmune disorders (n=230 [32%]); CNS infections (n=112 [15%]); inflammatory CNS disorders of unknown cause (n=81 [11%], including three (4%) with a novel Klüver-Bucy-like syndrome; and non-inflammatory disorders (n=306 [42%]), which were predominantly epileptic or psychiatric disorders (177 [58%] of 306). Neural antibodies were detected in 150 (65%) of 230 patients who had definite autoimmune encephalitis; 127 (85%) of these 150 individuals had antibodies to the NMDA receptor or myelin oligodendrocyte glycoprotein (MOG). Agreement between algorithms was excellent (kappa index 0·99, 95% CI 0·97-1·00) for the diagnosis of children with antibody-positive autoimmune encephalitis, good (0·59, 0·54-0·65) for recommendations of empiric immunotherapy, and poor (0·29, 0·21-0·37) for the diagnosis of probable antibody-negative autoimmune encephalitis. Compared with the general algorithm, the paediatric algorithm included more patients in the probable antibody-negative autoimmune encephalitis category (173 vs 41). These patients included some of those who had a diagnosis of CNS inflammatory disorder of unknown cause at the last follow-up (80 of 81 with the paediatric algorithm vs 31 of 81 with the general algorithm), who might have benefitted from immunotherapy, and some of those diagnosed with a non-inflammatory disorder at the last follow-up (47 of 306 with the paediatric algorithm vs six of 306 with the general algorithm), who did not need immunotherapy.FINDINGSBetween June 1, 2013, and May 31, 2021, 729 children (mean age 7·1 years [SD 4·9]; 383 boys [53%], 346 girls [47%]) with suspected autoimmune encephalitis were recruited. After a median follow-up of 36 months (IQR 26-60), patients were classified according to their most probable diagnosis: definite autoimmune encephalitis or well defined inflammatory or autoimmune disorders (n=230 [32%]); CNS infections (n=112 [15%]); inflammatory CNS disorders of unknown cause (n=81 [11%], including three (4%) with a novel Klüver-Bucy-like syndrome; and non-inflammatory disorders (n=306 [42%]), which were predominantly epileptic or psychiatric disorders (177 [58%] of 306). Neural antibodies were detected in 150 (65%) of 230 patients who had definite autoimmune encephalitis; 127 (85%) of these 150 individuals had antibodies to the NMDA receptor or myelin oligodendrocyte glycoprotein (MOG). Agreement between algorithms was excellent (kappa index 0·99, 95% CI 0·97-1·00) for the diagnosis of children with antibody-positive autoimmune encephalitis, good (0·59, 0·54-0·65) for recommendations of empiric immunotherapy, and poor (0·29, 0·21-0·37) for the diagnosis of probable antibody-negative autoimmune encephalitis. Compared with the general algorithm, the paediatric algorithm included more patients in the probable antibody-negative autoimmune encephalitis category (173 vs 41). These patients included some of those who had a diagnosis of CNS inflammatory disorder of unknown cause at the last follow-up (80 of 81 with the paediatric algorithm vs 31 of 81 with the general algorithm), who might have benefitted from immunotherapy, and some of those diagnosed with a non-inflammatory disorder at the last follow-up (47 of 306 with the paediatric algorithm vs six of 306 with the general algorithm), who did not need immunotherapy.About a third of children with suspected autoimmune encephalitis eventually had confirmation of this diagnosis, or diagnosis of another well defined inflammatory disorder. Frequent mimics of autoimmune encephalitis were infectious, epileptic, and psychiatric disorders. Both algorithms performed well in the diagnosis of antibody-positive autoimmune encephalitis, but the paediatric algorithm under-recognised definite autoimmune encephalitis that can occur without autoantibodies and might have overdiagnosed patients with probable antibody-negative autoimmune encephalitis. By contrast, the general algorithm might have underdiagnosed patients with probable antibody-negative autoimmune encephalitis. Given that the diagnosis of probable antibody-negative autoimmune encephalitis has treatment implications, inaccuracies on this diagnostic category leads to overuse or underuse of immunotherapy.INTERPRETATIONAbout a third of children with suspected autoimmune encephalitis eventually had confirmation of this diagnosis, or diagnosis of another well defined inflammatory disorder. Frequent mimics of autoimmune encephalitis were infectious, epileptic, and psychiatric disorders. Both algorithms performed well in the diagnosis of antibody-positive autoimmune encephalitis, but the paediatric algorithm under-recognised definite autoimmune encephalitis that can occur without autoantibodies and might have overdiagnosed patients with probable antibody-negative autoimmune encephalitis. By contrast, the general algorithm might have underdiagnosed patients with probable antibody-negative autoimmune encephalitis. Given that the diagnosis of probable antibody-negative autoimmune encephalitis has treatment implications, inaccuracies on this diagnostic category leads to overuse or underuse of immunotherapy.Instituto de Salud Carlos III, Fundació Clínic per la Recerca Biomèdica, The Edmond J Safra Foundation, and la Caixa Foundation.FUNDINGInstituto de Salud Carlos III, Fundació Clínic per la Recerca Biomèdica, The Edmond J Safra Foundation, and la Caixa Foundation.For the Spanish translation of the abstract see Supplementary Materials section.TRANSLATIONFor the Spanish translation of the abstract see Supplementary Materials section.  | 
    
| Author | Felipe, Ana Jiménez-Legido, María Martínez-Hernández, Eugenia Martínez-González, Maria J González-Álvarez, Veronica Arrabal Fernandez, Luisa Fetta, Anna Armangué, Thaís Olivé-Cirera, Gemma Beseler, Beatriz Spatola, Marianna Saiz, Albert Chen, Li-Wen Guasp, Mar Vázquez-López, Maria Blanco, Yolanda Graus, Francesc Cantarín-Extremera, Verónica Nuñez-Enamorado, Noemí Fernández-Ramos, Joaquín Delgadillo, Verónica Muñoz-Cabello, Beatriz Camacho, Ana Fonseca, Elianet Turón-Viñas, Eulàlia Dalmau, Josep Sabater, Lídia Monge-Galindo, Lorena Alvarez-Molinero, Mireia  | 
    
| Author_xml | – sequence: 1 givenname: Gemma surname: Olivé-Cirera fullname: Olivé-Cirera, Gemma organization: Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer/CaixaResearch Institute, Hospital Clínic de Barcelona, Barcelona, Spain – sequence: 2 givenname: Elianet surname: Fonseca fullname: Fonseca, Elianet organization: Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer/CaixaResearch Institute, Hospital Clínic de Barcelona, Barcelona, Spain – sequence: 3 givenname: Li-Wen surname: Chen fullname: Chen, Li-Wen organization: Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer/CaixaResearch Institute, Hospital Clínic de Barcelona, Barcelona, Spain – sequence: 4 givenname: Anna surname: Fetta fullname: Fetta, Anna organization: Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer/CaixaResearch Institute, Hospital Clínic de Barcelona, Barcelona, Spain – sequence: 5 givenname: Eugenia surname: Martínez-Hernández fullname: Martínez-Hernández, Eugenia organization: Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer/CaixaResearch Institute, Hospital Clínic de Barcelona, Barcelona, Spain – sequence: 6 givenname: Mar surname: Guasp fullname: Guasp, Mar organization: Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer/CaixaResearch Institute, Hospital Clínic de Barcelona, Barcelona, Spain – sequence: 7 givenname: Veronica surname: González-Álvarez fullname: González-Álvarez, Veronica organization: Pediatric Neuroimmunology Unit, Neurology Department, Sant Joan de Déu Children's Hospital, Barcelona, Spain – sequence: 8 givenname: Verónica surname: Delgadillo fullname: Delgadillo, Verónica organization: Pediatric Neuroimmunology Unit, Neurology Department, Sant Joan de Déu Children's Hospital, Barcelona, Spain – sequence: 9 givenname: Verónica surname: Cantarín-Extremera fullname: Cantarín-Extremera, Verónica organization: Pediatric Neurology Unit, Hospital Infantil Universitario Niño Jesús, Madrid, Spain – sequence: 10 givenname: María surname: Jiménez-Legido fullname: Jiménez-Legido, María organization: Pediatric Neurology Unit, Hospital Infantil Universitario Niño Jesús, Madrid, Spain – sequence: 11 givenname: Lorena surname: Monge-Galindo fullname: Monge-Galindo, Lorena organization: Pediatric Neurology Unit, Hospital Miguel Servet, Zaragoza, Spain – sequence: 12 givenname: Ana surname: Felipe fullname: Felipe, Ana organization: Pediatric Neurology Unit, Hospital Vall d’Hebron, Barcelona, Spain – sequence: 13 givenname: Beatriz surname: Beseler fullname: Beseler, Beatriz organization: Pediatric Neurology Unit, Hospital de la Fe, Valencia, Spain – sequence: 14 givenname: Eulàlia surname: Turón-Viñas fullname: Turón-Viñas, Eulàlia organization: Pediatric Neurology Unit, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain – sequence: 15 givenname: Joaquín surname: Fernández-Ramos fullname: Fernández-Ramos, Joaquín organization: Pediatric Neurology Unit, Hospital Universitario Reina Sofía, Córdoba, Spain – sequence: 16 givenname: Maria J surname: Martínez-González fullname: Martínez-González, Maria J organization: Pediatric Neurology Unit, Hospital Universitario de Cruces, Barakaldo, Bizkaia, Spain – sequence: 17 givenname: Maria surname: Vázquez-López fullname: Vázquez-López, Maria organization: Pediatric Neurology Unit, Hospital Gregorio Marañon, Madrid, Spain – sequence: 18 givenname: Luisa surname: Arrabal Fernandez fullname: Arrabal Fernandez, Luisa organization: Pediatric Neurology Unit, Hospital Virgen de las Nieves de Granada; Granada, Spain – sequence: 19 givenname: Mireia surname: Alvarez-Molinero fullname: Alvarez-Molinero, Mireia organization: Pediatric Neurology Unit, Hospital Joan XIII, Tarragona, Spain – sequence: 20 givenname: Beatriz surname: Muñoz-Cabello fullname: Muñoz-Cabello, Beatriz organization: Pediatric Neurology Unit, Hospital Virgen del Rocío, Sevilla, Spain – sequence: 21 givenname: Ana surname: Camacho fullname: Camacho, Ana organization: Pediatric Neurology Unit, Hospital 12 de Octubre, Madrid, Spain – sequence: 22 givenname: Noemí surname: Nuñez-Enamorado fullname: Nuñez-Enamorado, Noemí organization: Pediatric Neurology Unit, Hospital 12 de Octubre, Madrid, Spain – sequence: 23 givenname: Marianna surname: Spatola fullname: Spatola, Marianna organization: Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer/CaixaResearch Institute, Hospital Clínic de Barcelona, Barcelona, Spain – sequence: 24 givenname: Lídia surname: Sabater fullname: Sabater, Lídia organization: Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer/CaixaResearch Institute, Hospital Clínic de Barcelona, Barcelona, Spain – sequence: 25 givenname: Yolanda surname: Blanco fullname: Blanco, Yolanda organization: Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer/CaixaResearch Institute, Hospital Clínic de Barcelona, Barcelona, Spain – sequence: 26 givenname: Albert surname: Saiz fullname: Saiz, Albert organization: Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer/CaixaResearch Institute, Hospital Clínic de Barcelona, Barcelona, Spain – sequence: 27 givenname: Francesc surname: Graus fullname: Graus, Francesc organization: Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer/CaixaResearch Institute, Hospital Clínic de Barcelona, Barcelona, Spain – sequence: 28 givenname: Josep surname: Dalmau fullname: Dalmau, Josep email: jdalmau@clinic.cat organization: Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer/CaixaResearch Institute, Hospital Clínic de Barcelona, Barcelona, Spain – sequence: 29 givenname: Thaís surname: Armangué fullname: Armangué, Thaís email: thais.armangue@sjd.es organization: Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer/CaixaResearch Institute, Hospital Clínic de Barcelona, Barcelona, Spain  | 
    
| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/39706634$$D View this record in MEDLINE/PubMed | 
    
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| CorporateAuthor | Spanish Pediatric Autoimmune Encephalitis study group | 
    
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priorities in encephalitis: consensus statement of the international encephalitis consortium publication-title: Clin Infect Dis doi: 10.1093/cid/cit458 – volume: 10 year: 2023 ident: 10.1016/S1474-4422(24)00443-5_bib8 article-title: Mimics of autoimmune encephalitis: validation of the 2016 Clinical Autoimmune Encephalitis Criteria publication-title: Neurol Neuroimmunol Neuroinflamm doi: 10.1212/NXI.0000000000200148 – volume: 22 start-page: 529 year: 2023 ident: 10.1016/S1474-4422(24)00443-5_bib4 article-title: Diagnostic criteria for autoimmune encephalitis: utility and pitfalls for antibody-negative disease publication-title: Lancet Neurol doi: 10.1016/S1474-4422(23)00083-2 – volume: 92 start-page: 279 year: 2022 ident: 10.1016/S1474-4422(24)00443-5_bib24 article-title: ZSCAN1 autoantibodies are associated with pediatric paraneoplastic ROHHAD publication-title: Ann Neurol doi: 10.1002/ana.26380 – volume: 128 start-page: 1764 year: 2005 ident: 10.1016/S1474-4422(24)00443-5_bib17 article-title: Treatment-responsive limbic encephalitis identified by neuropil antibodies: MRI and PET correlates publication-title: Brain doi: 10.1093/brain/awh526  | 
    
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| Snippet | The usefulness of current diagnostic approaches in children with suspected autoimmune encephalitis is unknown. We aimed to assess the diagnosis of autoimmune... Summary Background The usefulness of current diagnostic approaches in children with suspected autoimmune encephalitis is unknown. We aimed to assess the...  | 
    
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| SubjectTerms | Adolescent Age Algorithms Antibodies Autoantibodies Autoantibodies - blood Autoimmune diseases Autoimmune Diseases of the Nervous System - diagnosis Autoimmune Diseases of the Nervous System - immunology Autoimmune Diseases of the Nervous System - therapy Child Child, Preschool Children Cohort analysis Cohort Studies Convulsions & seizures Diagnosis, Differential Differential diagnosis Encephalitis Encephalitis - diagnosis Encephalitis - immunology Encephalitis - therapy Encephalomyelitis Epilepsy Female Glutamic acid receptors (ionotropic) Hashimoto Disease - diagnosis Hashimoto Disease - immunology Hashimoto Disease - therapy Humans Immunotherapy Infant Inflammatory diseases Investigations Male Medical diagnosis Mental disorders Myelin N-Methyl-D-aspartic acid receptors Oligodendrocyte-myelin glycoprotein Patients Pediatrics Physicians Prospective Studies Retrospective Studies Spain Teenagers Translation  | 
    
| Title | Differential diagnosis and comparison of diagnostic algorithms in children and adolescents with autoimmune encephalitis in Spain: a prospective cohort study and retrospective analysis | 
    
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| Volume | 24 | 
    
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