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1. ALLOSTERIC MODULATION OF NMDAR REVERSES ANTI-NMDAR ENCEPHALITIS IN A MOUSE MODEL OF THIS DISEASE

Author: Estibaliz Maudes, Laura Marmolejo, Ana Serafim, Jesús Planagumá, Marija Radosevic, Mar Guasp, Jon Landa, Raquel Ruiz, Lidia Sabater, Marianna Spatola, Mike Lewis, and Josep Dalmau
Subjects: Neurosciences. Biological psychiatry. Neuropsychiatry, RC321-571
Published: 2023
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2. The diagnosis of anti-LGI1 encephalitis varies with the type of immunodetection assay and sample examined

Author: Guillermo Muñoz-Sánchez, Jesús Planagumà, Laura Naranjo, Rocío Couso, Lidia Sabater, Mar Guasp, Eugenia Martínez-Hernández, Francesc Graus, Josep Dalmau, and Raquel Ruiz-García
Subjects: neuronal antibodies, Lgi1, diagnostic test, autoimmune encephalitis (AE), immunofluorescent assay, Immunologic diseases. Allergy, RC581-607
Abstract: Detection of Leucine-rich glioma inactivated 1 (LGI1) antibodies in patients with suspected autoimmune encephalitis is important for diagnostic confirmation and prompt implementation of immunomodulatory treatment. However, the clinical laboratory diagnosis can be challenging. Previous reports have suggested that the type of test and patient’s sample (serum or CSF) have different clinical performances, however, there are no studies comparing different diagnostic tests on paired serum/CSF samples of patients with anti-LGI1 encephalitis. Here, we assessed the clinical performance of a commercial and an in house indirect immunofluorescent cell based assays (IIF-CBA) using paired serum/CSF of 70 patients with suspected anti-LGI1 encephalitis and positive rat brain indirect immunohistochemistry (IIHC). We found that all (100%) patients had CSF antibodies when the in house IIF-CBA was used, but only 88 (83%) were positive if the commercial test was used. In contrast, sera positivity rate was higher with the commercial test (94%) than with the in house assay (86%). If both serum and CSF were examined with the commercial IIFA-CBA, 69/70 (98.5%) patients were positive in at least one of the samples. These findings are clinically important for centers in which rat brain IIHC and in house IIFA-CBA are not available. Moreover, the observation that all patients with anti-LGI1 encephalitis have antibodies in CSF is in line with the concept that these antibodies are pathogenic.
Published: 2022
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3. CSF Biomarkers in COVID-19 Associated Encephalopathy and Encephalitis Predict Long-Term Outcome

Author: Mar Guasp, Guillermo Muñoz-Sánchez, Eugenia Martínez-Hernández, Daniel Santana, Álvaro Carbayo, Laura Naranjo, Uma Bolós, Mario Framil, Albert Saiz, Mircea Balasa, Raquel Ruiz-García, Raquel Sánchez-Valle, and The Barcelona Neuro-COVID Study Group
Subjects: COVID-19, encephalitis, neurofilaments, neuronal antibodies, SARS-CoV-2, neuro-COVID, Immunologic diseases. Allergy, RC581-607
Abstract: Patients with coronavirus disease 2019 (COVID-19) frequently develop acute encephalopathy and encephalitis, but whether these complications are the result from viral-induced cytokine storm syndrome or anti-neural autoimmunity is still unclear. In this study, we aimed to evaluate the diagnostic and prognostic role of CSF and serum biomarkers of inflammation (a wide array of cytokines, antibodies against neural antigens, and IgG oligoclonal bands), and neuroaxonal damage (14-3-3 protein and neurofilament light [NfL]) in patients with acute COVID-19 and associated neurologic manifestations (neuro-COVID). We prospectively included 60 hospitalized neuro-COVID patients, 25 (42%) of them with encephalopathy and 14 (23%) with encephalitis, and followed them for 18 months. We found that, compared to healthy controls (HC), neuro-COVID patients presented elevated levels of IL-18, IL-6, and IL-8 in both serum and CSF. MCP1 was elevated only in CSF, while IL-10, IL-1RA, IP-10, MIG and NfL were increased only in serum. Patients with COVID-associated encephalitis or encephalopathy had distinct serum and CSF cytokine profiles compared with HC, but no differences were found when both clinical groups were compared to each other. Antibodies against neural antigens were negative in both groups. While the levels of neuroaxonal damage markers, 14-3-3 and NfL, and the proinflammatory cytokines IL-18, IL-1RA and IL-8 significantly associated with acute COVID-19 severity, only the levels of 14-3-3 and NfL in CSF significantly correlated with the degree of neurologic disability in the daily activities at 18 months follow-up. Thus, the inflammatory process promoted by SARS-CoV-2 infection might include blood-brain barrier disruption in patients with neurological involvement. In conclusion, the fact that the levels of pro-inflammatory cytokines do not predict the long-term functional outcome suggests that the prognosis is more related to neuronal damage than to the acute neuroinflammatory process.
Published: 2022
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4. Limitations of a Commercial Assay as Diagnostic Test of Autoimmune Encephalitis

Author: Raquel Ruiz-García, Guillermo Muñoz-Sánchez, Laura Naranjo, Mar Guasp, Lidia Sabater, Albert Saiz, Josep Dalmau, Francesc Graus, and Eugenia Martinez-Hernandez
Subjects: neuronal antibodies, brain immunohistochemistry, diagnostic test, autoimmune encephalitis (AE), immunofluorescent assay, Immunologic diseases. Allergy, RC581-607
Abstract: Detection of neuronal surface antibodies (NSAb) is important for the diagnosis of autoimmune encephalitis (AE). Although most clinical laboratories use a commercial diagnostic kit (Euroimmun, Lübeck, Germany) based on indirect immunofluorescence on transfected cells (IIFA), clinical experience suggests diagnostic limitations. Here, we assessed the performance of the commercial IIFA in serum and CSF samples of patients with suspected AE previously examined by rat brain immunohistochemistry (Cohort A). Of 6213 samples, 404 (6.5%) showed brain immunostaining suggestive of NSAb: 163 (40%) were positive by commercial IIFA and 241 (60%) were negative. When these 241 samples were re-assessed with in-house IIFA, 42 (18%) were positive: 21 (9%) had NSAb against antigens not included in the commercial IIFA and the other 21 (9%) had NSAb against antigens included in the commercial kit (false negative results). False negative results occurred more frequently with CSF (29% vs 10% in serum) and predominantly affected GABABR (39%), LGI1 (17%) and AMPAR (11%) antibodies. Results were reproduced in a separate cohort (B) of 54 AE patients with LGI1, GABABR or AMPAR antibodies in CSF which were missed in 30% by commercial IIFA. Patients with discordant GABABR antibody results (positive in-house but negative commercial IIFA) were less likely to develop full-blown clinical syndrome; no significant clinical differences were noted for the other antibodies. Overall, NSAb testing by commercial IIFA led to false negative results in a substantial number of patients, mainly those affected by anti-LG1, GABABR or AMPAR encephalitis. If these disorders are suspected and commercial IIFA is negative, more comprehensive antibody studies are recommended.
Published: 2021
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5. Cycloid psychosis as a psychiatric expression of anti‐NMDAR encephalitis. A systematic review of case reports accomplished with the authors' cooperation

Author: Eloi Giné Servén, Ester Boix Quintana, Maria Martínez Ramírez, Nicolau Guanyabens Buscà, Desiree Muriana Batiste, Mar Guasp, Cristina Torres Rivas, Eva Davi Loscos, and Virginia Casado Ruiz
Subjects: acute psychosis, anti‐NMDAR encephalitis, atypical psychosis, autoimmune, first psychotic episode, Neurosciences. Biological psychiatry. Neuropsychiatry, RC321-571
Abstract: Abstract Objective We reviewed the psychotic symptoms of anti‐NMDA receptor encephalitis (NMDARE) to differentiate its presentation from those found in a primary psychiatric disorder. We hypothesized that the cycloid psychosis (CP) phenotype would be a frequent clinical presentation in the psychiatric phase of NMDARE. Method A systematic literature review in PubMed of all case reports published on NMDARE was performed from database inception to March 2020. We included all cases where psychotic symptoms were reported and whose diagnoses were confirmed by the presence of anti‐NMDAR antibodies in the cerebrospinal fluid (CSF). An email including a short test (CP phenotype, Perris and Brockington's criteria) was sent to all case report authors asking them to describe the psychotic symptoms. Results We identified 335 case reports fulfilling our criteria, and the authors of 200 replied. Our analyses were based exclusively on those answers and data extracted from the articles. Median patient age was 25 years (+‐11.4), 81% were female, and 39% had an ovarian teratoma. A complete CP phenotype was identified in 175 patients (87%). These were acute psychotic episodes with a sudden onset and a fluctuating clinical pattern mostly characterized by confusion (97%), delusions (75%), hallucinations (69%), motility disturbances (87%), and mood oscillations (80%). Conclusion The complete CP phenotype was frequently the expression of psychotic symptoms in NMDARE. We suggest that patients with a first psychotic episode who initially exhibit the CP phenotype should undergo CSF analysis to determine whether antibodies against neuronal cell surface or synaptic receptors are present to rule out a possible diagnosis of autoimmune encephalitis.
Published: 2021
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6. Evidence of neurophysiological improvement of early manifestations of small-fiber dysfunction after liver transplantation in a patient with familial amyloid neuropathy

Author: Mar Guasp, Alejandro A. Köhler, Michela Campolo, Jordi Casanova-Molla, and Josep Valls-Sole
Subjects: Neurosciences. Biological psychiatry. Neuropsychiatry, RC321-571
Abstract: Introduction: Small fiber polyneuropathy (SFP) is a common heralding clinical manifestation of damage to the nervous system in patients with familial amyloidosis. The diagnosis of SFP is a significant factor in the decision to treat a previously asymptomatic gene carrier, as treatment would prevent irreversible nerve damage. This requires detection of the earliest but unequivocal signs of peripheral nerve involvement. Case report: We present the case of a young female who was diagnosed of SFP, supported by data from quantitative sensory testing. She had preserved sensory nerve action potentials in the distalmost nerves of her feet and recordable nociceptive evoked potentials. She was successfully transplanted the liver from a previously healthy donor, and recovered fully of her symptoms and signs. Improvement was documented with repeated psychophysical and electrodiagnostic testing in the course of 4 years after transplantation. Significance: This case illustrates the utility of psychophysical testing to support the diagnosis of SFP. Keywords: Small fiber polyneuropathy, Familial amyloidosis, Quantitative sensory testing, Nociceptive evoked potentials, Psychophysical testing, Liver transplantation
Published: 2018
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7. Association of Time of Day With Outcomes Among Patients Triaged for a Suspected Severe Stroke in Nonurban Catalonia

Author: Álvaro García-Tornel, Alan Flores, Mikel Terceño, Pedro Cardona, Sergi Amaro, Meritxell Gomis, Josep Zaragoza, Jerzy Krupinski, Manuel Gómez-Choco, Natalia Mas, Dolores Cocho, Esther Catena, Francesc Purroy, Matias Deck, Marta Rubiera, Jorge Pagola, David Rodriguez-Luna, Jesús Juega, Noelia Rodríguez-Villatoro, Carlos A. Molina, Cristina Soro, Xavier Jimenez, Mercè Salvat-Plana, Antoni Dávalos, Tudor G Jovin, Sonia Abilleira, Natalia Pérez de la Ossa, Marc Ribó, Estela Sanjuan, Katherine Santana, Olga Maisterra, Estevo Santamarina, Marian Muchada, Sandra Boned, Antonio Palasi Franco, Marta Olivé Gadea, Matías Deck, Manuel Requena, Victoria Sala, Lucía Muñoz, Mónica Millán, Elena López-Cancio, Laura Dorado, María Hernández-Pérez, Jordi Ciurans, Daniela Samaniego, Tamara Canento, Lorena Martin, Anna Planas, Joaquin Broto, Agustín Sorrentino, Martí Paré, Nuole Zhu, Alicia Garrido, Laia Grau, Ane Miren Crespo, Silvia Presas, Miriam Almendrote, Alba Ramos, Giuseppe Lucente, Lourdes Ispierto, Manuel Lozano, Juan Luis Becerra, Marta Jiménez, Dolores Vilas Rolán, Nicolas Guanyabens, Josep Sanchez-Ojanguren, Alicia Martínez-Piñeiro, Sara Forcén, Mireia Gea, Marta Álvarez, Anna Ramos, Manuel Domínguez Lizarbe, Rubio Guerra, Irene Bragado, Andrea Arbex, Luis Rodríguez, Alejandro Bustamante, Pere Cardona Portela, Helena Quesada García, Blanca Lara Rodríguez, Nuria Cayuela, Julia Miró, Clara Marzal, Andrés Paipa, Sergio Campoy, Ana Núñez, Pablo Arroyo, Sarah Besora, Vanessa Adell, Jaume Campdelacreu, Montse Alemany Martí, Belén González, Laura Bau Vila, Marta Fiter Crespo, Anna Berbel, Ma. Cristina Villaescusa Urbaneja, Núria Guillen, Nuria Vidal, Patricia Valentina Vérez Santamaria, Diego Herrero Navarro, Marta Simó, Mercedes Falip, Elisabeth Matas, Neus Mongay Ochoa, Ariadna Gifreu, Albert Muñoz, Lucía Romero, Eduard Portell, Guillermo Hernández Perez, F. Ruiz Esteve, Silvia Teixidor, Augusto Salazar Talavera, Roser Gómez, Xabier Urra Nuin, Martha Vargas, Ángel Chamorro, Laura Llull, Arturo Renú, Salvatore Rudilosso, Raquel Sánchez del Valle, Helena Ariño, Nuria Solà, Delón la Puma, Francisco Gil, Juan Bernardo Gómez, Nuria Matos, Neus Falgàs, Sergi Borrego, Almudena Sánchez, Mircea Balasa, Carmen Montejo, Mar Guasp, David Reyes, Pablo Sánchez Cervilla, Jose Miguel Contador, Victor Augusto Vera Monge, Oscar Ramos, Alejandro Rodríguez, Ana Rodríguez Campello, Gemma Romeral Ballester, Mireia Llop Trujillano, Eva Giralt Steinhauer, Elisa Cuadrado Godia, Angel Javier Ois Santiago, Jordi Jimenez Conde, Joan Martí Fábregas, Daniel Guisado, Luís Prats, Pol Camps, Raquel Delgado, Alejandro Martínez Domeño, Rebeca Marín, David Cànovas, Jordi Estela, Marta Ros, Sonsoles Aranceta, Jordi Espinosa, Marta Rubio, Cristina Lafuente, Oriol Barrachina, Alicia Anguita, Anna Reverter, Carmen García, Gemma Sansa, Mariona Hervas, Monica Crosas, Tania Delgado, Dra Sonia Huertas Folch, Gemma Muñoz Gamito, Josep Trenado Alvarez, Teresa Subirana, Jessica Molina, Laura Comes Romero, Gemma Guardia Valls, Miriam Jover, Javier Joaquín Sotova, Sonia Mª García Sánchez, Sebastian Valenzuela, Juan José Mengual, M. Àngels Font, Mª Isabel Gómez Ruiz, Irati Zubizarreta, Susana Fernández González, Laura Gubieras, Carmen E. Cobos, Luis Mena Romo, Nuria Caballol, Luis Cano, Joaquín Serena Leal, Yolanda Silva Blas, Irene Bragado Trigo, Saima Bashir Viturro, Laura Pardo Albiñana, Montserrat Reina Garrido, Carla Marco Cazcarra, Karol Enrique Uscamaita, Fabian Márquez, Cristina Coll, Mar Irida Lloret Villlas, Berta Solano Vila, Berta Alemany Perna, Daniel López Domínguezl, Mercedes de Lera, Anna Cots Foraster, Urszula Bojaryn, Ikram Benabdelhak, Gerard Mauri Capdevila, Jordi Sanahuja Montesinos, Daniel Vázquez, José Vicente Hervás, Cristina González, Alejandro Quílez, Mikel Vicente Pascual, María Ruiz, Yolanda Riba, M. Pilar Gil Villar, Cristina García, Xavier Ustrell Roig, Mònica Baldrich Mora, Anna Pellisé Guinjoan, Judit Borras, Alba Mañé Martínez, Rafael Marés, Jaume Viñas i Gaya, Laia Seró, Paula Rodríguez, Gislaine Castilho, Angela Monterde Ortega, Silvia Reverté, Joan Josep Baiges, Gisela Martín Ozaeta, Sonia Escalante, Patricia Esteve Belloch, Iago Payo, Júlia Saura Salvado, Josep M Soler Insa, Ester Tio Vilamala, Josep Abos Navarro, Héctor Cruz Tabuenca, Tamara Canento Sánchez, Elisabet Roldán, Elsa Puiggrós Rubiol, Elisabet Franquet, Lidia Fuentes, Javier Donaire, Elena Martí, Laura Giménez, Junior Gil Vázquez, Eloisa Nieto Clara Gris Ambrós, Puri Rodríguez, José Félix Oletta, Pilar Pons Mellado, Bárbara Gómez, Vergilin Raileau, Oscar Pardina, Jordi Mercadal, María López-Diéguez, Pilar Pérez, Lourdes Gabarró, María Orriols, José Carlos Molina, Joan Josep Canet, Mireia Roca, Muriel Álvaro, Francesc Boneu, Georgina Giménez, Jaume Albà, Francesc Gibert, Jéssica Garcia, Patricia Barragan, Gustavo Jurado, Vanesa Pascual_Medicina Interna, Josep Sabaté Ortega, Joan Amaurys Martínez Solano, Víctor Fernández, Mónica Torres, Ana Belén Montero Alvaredo, Laura Redondo Parejo, Josep Maria Aragonés, Anna Bullón, Cora Loste, Paula González, Neker Bejarano, Francisco Sanchez, Gianni Lucchetti, Xavier Pla, Dr Javier Gimeno, Esteban Reynaga, Montse Barcons, Gabriel Celedón, Juan Ortiz, Dr Goran Anastasovski, Dr Oscar Mascaró, Dr Javier Diez de los Ríos, Meritxell Feliu, Anna Ribera, Cristhian Ruiz, Gerard Corominas, Dra Daniella Nunes, Claudia Roca, Nadina Latorre, Lizbeth Yataco, Maricelys Cruz, Nerea Blanco, Santiago Castejón, Claustre Pont Sunyer, Jordi Espinosa Garcia, Rodrigo Paz Martin, Albert de Luis Sanchez, Dolores Espinosa Vivas, Juan Valencia Molina, Planels Palome, Laia Tomas Chaume, Ares Villagrasa Vilella, Marco Bustamante, Anunciación Boltes, Fernando Rodríguez, Itziar Arrieta, Jordi Ciurans Molist, Barnés Andreu, Ernest Palomeras Soler, Nicolau Guanyabens Buscà, Manuel Daza López, Jordi Bigas Farreres, Virginia Casado Ruiz, Desiree Muriana Batiste, Mª Pilar Sanz Cartagena, Eulalia Cabot de Vega, Josep Bassa Real, Hector Pelaez Roman, César Socolich, Josep Mª Alonso Camp, Antonio Tomás Cano Orgaz, Mª Pilar Fossas Felip, Nicolás Morón, Sandra Bacca, Mauricio Molina, Francesc Casarramona, Lorena Elias, Muhammad Zidane Bukaei, Jose Antonio Martos Gutierrez, Judith Lopez Escuin, Cristina Olaizola, Yolexis López Vargas, Juan Jiménez Oyonarte, Rashida Soultana, Eduardo Sanjurjo Golpe, Esther Salvador, Guillem Vila, Marcos Serrano, Matilde Nuria López Claverol, Marian Lamolla, Miquel Amate, Adriana Rodriguez, Ruth Romero, Montgomery del Carpio, Ana Isabel Hernandez, Julián Martín, M Carmen Rosas, Antonio Nogueroles, Sorilandy Encarnación, Augusto Robles, Jose Antonio Herrera, Roger Gavilán, Toghrol Mameghani, Gastón Araujo, M.Angeles Garrido Morales, Enric Ramon Albert Segui, Eva Fernandez Climent, Francesc Paris Pujol, Mireia Judit Garrofé Seira, Lucía Gómez Pía, Fernando Salleres Nuñez, Cristina Aguar Peñalver, Cristina Vaz Lopes, Elisenda Ribera Tasa, Carmen Repullo Vilchez, Modesto Sánchez Zambrana, Beatriu Suescun Ribas, Inés Vilà Panés, Montserrat Vila Planavila, Alicia Vaqueiro Lorenzo, Meritxell Sabartés Guixes, Jorge Medina, David Sambrano, Javi Zamarreño, Carmen Pirela, Paola Vélez, Luis Cajamarca, Honey Pérez, Yarles Martínez, Jesus Alexander Gonçalves, Carles Regordosa, Claudia Mormeneo, Laura Griu, Maria Francia Colina, Enric Farik, Dolors Carrión Duch, Carles Badenas, Oscar Bernal, Núria Agramunt, Shyrlei Morales, Victoria Reynoso, Miguel Guerrero, Primitivo Romera Cid, Mònica Folqué, Claudia Pedroza, Adnan Hachem, Íñigo Soteras Martínez, Xavier Verdera García, Mercè López Amorós, Xavier Costa Subirós, Marta Cufí Benet, Cecile Van Eendenburg, Teresa Osuna, Dra Gina Santos, Dra Mireia Pallisera, Dr Lluís Gonzalo Oliva, Dra Gemma Sanchez, Dr Xavier Basurto, Dr Ludgi Vivoda, Dr Richard Van der Kleyn, Dra Laura Robles, Ana Cabanelas Barranco, Mª Cruz Almendros, Marc Pérez Oliveras, Amelia Fernández Álvarez, Maria Rybyeva, Antoni Viñas, Maria Barcons, Joaquim Danès Alberto Tavera, Pablo Burbano, Cintie López, David Cruz, Paula Bisbe, Nora Fernández, Juan Carlos Palacio, Eduardo Fraiz, Oriol Aguiló, Rollmy Amorodjo, John Velázquez, Elena Sánchez, Jaume Español, Judit Perez de Celis, Anna Coll, Glòria Díaz, Margarida Vergés i Sala, Mª Ángels Casas Capdevila, Yosmairy Yoselin Ferrini, Aitor Gorriz, Diego-Javier Cucurell Navarro, Dulce Velásquez, Jaume Pla Soler, Josep González, Julian David Higuera, Lina Cuellar, Liza Margarita Miniello, Lluis Pujol, Sorin Cracan, Mora Vives, M Angela, Lopez Lopez, Montse Gorchs Molist, Delofeu Anna, Silvia Solà Muñoz, Ferreres Yolanda, Carol Pujalte, Elisabeth Téllez Marín, Yolanda Font Casas, Sara Hernández Luque, Joaquim Mejias Sendra, Francesca Mellado Valero, Campos Escala, Galup De Lacanal, Lopez Diaz, Castillo Paramio, Català Estopà, Español Moreda, Calafell Majo, Carballo Almeida, Castro Naval, M Elena, Cregut Ruiz, Pere Lluis, Deulofeu Font, Fabregat Sanjuan, Joan Pere, Fontquerni Gorchs, Forés Bellés, Joan Antoni, Gomez Herrera, Juan Carlos, Hijazo Prades, Itzaina Torvisco, Marti Rovira, Obiols Gonzalez, Olive Cavero, Querol Gil, M Soledad, Rico Rodriguez, Rios Sambernardo, Ropero Molina, Jose Ramon, Sanchez Valero, Santos Arevalo, Maria Jose, Soto Garcia, Maria Angeles, Tebar Escribano, Torres Garcia, Pedro José, Verdes Carrion, M Isabel, Verge Lopez, Juan Jose, Lopez Canela, M Angeles, Lucas Guarque, Morales Alvarez, Jorge Arnulfo, Morell Fornieles, Subirats Gomez, Maria Teresa, Torello Masa, Miquel, Val Lopez, Maria Mercedes, Palmero Reyes, Juan Manuel, Rodriguez Forne, Ruana Turiel, Sanchez Gonzalez, Sebastia Gornals, Abrio Rico, Albert Gual Falip, Aznar Oliveros, Brugues Perotti, Calvet Molinero, Catells Franco, Corrales Medina, Daroca Miro, Duran Marquez, Feliu Pradas, Figueras Casanova, Garcia Tortajada, Grajera Gallego, Azahara del Mar, Las Arroyo, Dulcenombre de Jesus, Laserreta Sanz, Llambrich Vidal, Monllao Corral, Morales de la Cruz, Moreno Blanco, Orejuela Orgaz, Piñana Suazo, Pons Minguillon, Rodríguez Gómez, José Carlos, Rodriguez Pereira, Sedo Porcel, Anna, Sevil Villar, Sierra Lopez, Torres Romero, Trepat Calveres, Anna, Ventura Arnella, Acera Gil, M Teresa, Biesot Vico, Castro Galea, Rosa Maria, Escorcia Chafer, Juan Antonio, Gil Faure, Hernandez Luque, Jimenez Delgado, Mallafre Gay, M. Angel, Olivares Sanzo, Pubill Fondevila, Sabate Fort, Segovia Agámez, Antonio Carlos, Turata Longaretti, Valle Hernandez, Trayner Guixens, Aguilar Valor, Aguirre Alvarez, Alastrue Naval, Alferez Baquero, Alonso Marne, Aloy Orozco, Alvarez Colino, Alvarez Peñuela, Ameller Cirilo, Maria Vanessa, Andujar Guerrero, Estefania Remedios, Auladell Sillero, Azcarate Sorbet, Basany Genesca, Benavent Barduena, Benito Tudela, Blanch Pardo, Boluda Pla, Bonilla Gonzalez, Bru Serramalera, Burnat Andreu, Caballero Calvo-Rayo, Calvo Casellas, Camps Mombiela, Cano Bresme, Carcelen Fernandez, Caro Solà, Casasas Matavacas, Castanedo Bolado, Cerdeña Cortina, Abella Jane, Laia Collado Borrego, Colom Orri, Comellas Vilanova, Conillera Sole, Cots Torres, Cuchi Estepa, Marc de Sostoa Graell, Laura del Rio Lopez, Delgado Compte, Diaz Bueno, Diaz Maneiro, Andrea Lucia, Domenech Palau, M. Carme, Duran Carasso, Pablo Javier, El Abidi, Escoda Martin, Esteve Casanovas, Fernandez Perez, Ferre Quintero, Flores Escobar, Fontcuberta Abad, Fortes del Valle, Mª Luisa, Franco Quesada, Sara Maria, Franco Romero, Gallego Francisco, Gamiz Gala, Garcia Castañeda, Garcia Rodriguez, Casajuana Gemma, Gomez Arroyo, Gomez Girbau, Gonzalez Fernandez, Gonzalez Gonzalez, Gonzalez Lopez, Gual Obeso, Guerrero Blanco, Guillemas Roca, Guillermo Eudaldo, Sebastia Hernandez, Homar Covas, Hostench Alvarez, Huerta Royo, Ibañez Barcelo, Izquiero Cruz, Jaione Urdangarin Etxetxikia, Juárez Gimenez, Khlifi Alami, Laparra Gasco, Lara Moreales, Lasaosa Medina, Maria Lourdes, Lazaro Cava, Leon Berrar, López Maturana, Lopez Ramirez, López Romero, Lorente Marco, Lorenzo Martin, Lozano Casals, Lujan Nicola, Madrid Diaz, Jose Antonio, Maria Teresa Peris Morales, Marti Clemente, Martinez Castaño, David Natanael, Martinez Gamez, Martinez Gonzalez, Martinez Quesada, Maria de la Cabeza, Marzà Fusté, Melendo Lasheras, Miralles Mestre, Montserrat Puig Pastalle, Montserrate Vidal, Mora Mellado, Morales Ponce, Morera Cabre, Morera Vivet, Mundi Souza, Muñoz Quiles, Navea Rosa, Neira Iglesias, M Asuncion, Nuñez Manrique, Mª Pilar, Obiols Martinez, Pallares Reig, Partegas Torres, Pascual Berengueras, Perez Gamez, Perez Oset, Perez Restrepo, Maria Alejandra, Planas Yeste, Planells Mangado, Puertas Carbonell, Quinteiro Blanco, Rebollar Benavente, Recasens Fernandez, Reyes Gomez, Maria del Carmen, Roca Pou, Rodriguez Ferrer, Rodriguez Franco, Rodriguez Fuertes, Rodriguez Muñoz, Rodriguez Navarro, Roig Iniesta, Romero Gracia, Rovira Brunet, Rovira de Eugenio, Rubio Baena, Ruiz Llorens, Sala Llamazares, San Juan Alaez, Sanchez Segura, Santasusana Soldevilla, Sanz Salmeron, Segura Perez, Serra Balcells, Sorina Dumitras, Teixidor Montoya, Tena Vicente, Tierno Salinas, Ester Ines, Toledo Testa, Tomàs Figueras, Tomas Ruperez, Torrano Garcia-Penche, Torres Esparza, Vazquez Blanco, Vazquez De la Paz, Vazquez Gil, Vazquez Gonzalez, Vidal Meler, Vilalta Cots, Vilches Jimenez, Visser Fernandez, Viu Gavin, Vives Vives, Carmona Jimenez, Francisco Jose, Galobardes Vilches, Gonzalez Gomez, Jodar Manzanera, Marimon Cortes, Marrodan Orive, Martin Bosch, Martorell Lopez, Millares Roca, M Jesus, Miralles Guri, Muliterno Hernandez, Muñoz Oliva, Pons Pujol, Postius Conde, Prieto Arruñada, Quintana Mathé, Rosell Mata, Alvarin Alvarez, Arrufat Flores, Bañuelos Pago, Campo Vilar, Cardus Hidalgo, Castello Gil, Maria Gloria, Castrillo Montsesinos, Clave Garces, Comes Sanroma, Descalzo Sequi, Errando Ricol, Escudero Campillo, Maria del Mar, Fernandez Alvarez, Fernandez Gomez, Franch Espinosa, Freixes Graells, Garcia Plaza, Garcia-Marron Gallego, Hernandez Simancas, Jaraba Armas, Jimenez Ramos, Lopez Oganissian, Madrona Romero, M Rosa, Martinez Millan, Martínez Morón, Merchan Encinas, Moreno Jimenez, Muñoz Rico, Navarro Rodriguez, Nogales Ibañez, Rita Maria, Novillo Viera, Parella Torrabadella, Mari Alba, Pera Villar, Carla Montserrat, Rabella Miralles, Ramis Trilles, Ribera Ortiz, Sastre Perez, Soro Borrega, Tapia Fores, Tellez Bernad, Trenado Alvarez, Felix Martin, Herrero Perez, Lanau Fuster, Lopez Gomariz, Pardo Lozano, Quintana Altimiras, Ventosa Lopez, Almodovar Damian, Alvarez Monterroso, Arambudo Comas, Boullon Garzon, Burgos Capella, Campuzano Garcia, Maria Del Mar, Capdevila Olivas, Carre Marti, Clara Isabel, Carretero Bacaicoa, Cazorla Calderon, Chacon Osuna, Cortes Planas, Cortit Olio, Delhomme Estany, Fructuos Martinez, Garcia Lopez, Pascual Luis, Genis Amill Vallve, Gonzalez Muñoz, Hernaez Ventura, Lavernia Gimenez, Martin Rojo, Martínez Medina, Mayol Barrera, Mercader Pi, Merino Dalmau, Miguel Campodarbe, Moreno Sanchez, Francisco Javier, Penela Barrameda, Prieto Ajenjo, Rodriguez Pareja, Rodríguez Piñar, Rodriguez Tello, Romero Pereda, Sainz Saborido, Santacana Martin, Santos Martinez, Sierra Chavez, Turbau Torres, Vicente Domenech, Villena Esteo, Vives Pertegaz, Baena Gonzalez, Bagaria Pont, Beltran Sanchez, Bonet Alarcon, Borch Ballescà, Buhils Clanchet, Closa Garcia, Colomina Carril, Cristina Fernandez Sanchez, Edurne Judith Fernandez Toledo, Espases Rodriguez, Espert Resa, F Xavier, Fernández Sol, Gonzalez Lugo, Gracia Llesera, Herrada Lendinez, Juliench Liesa, Sandra Alicia, Lopez Ortega, Lopez Yuste, Lozano Villanueva, Lucia Sola Soto, Madrid Castellano, Megias Cano, Daniel Jesus, Montasell Ponce, Montesinos Sospedra, Moya Martinez, Muñoz Herrera, Ocaña Fernandez, Palau Bernal, Perez Sole, Ramon Carbonell, Ribas Lop, Rubio López, Rueda Lopez, Sabat Castaño, Sabata Lara, Salmerón Ramírez, Serra Creus, Serrano Dalmau, Sumasi Mainero, Gonzalo Gabriel, Torello Masa, Valencia Primo, Ana Maria Viñuales Muñoz, Josep Roig, and Verònica Hidalgo
Subjects: Advanced and Specialized Nursing, Neurology (clinical), Cardiology and Cardiovascular Medicine
Abstract: Background: We aim to assess whether time of day modified the treatment effect in the RACECAT trial (Direct Transfer to an Endovascular Center Compared to Transfer to the Closest Stroke Center in Acute Stroke Patients With Suspected Large Vessel Occlusion Trial), a cluster-randomized trial that did not demonstrate the benefit of direct transportation to a thrombectomy-capable center versus nearest local stroke center for patients with a suspected large vessel stroke triaged in nonurban Catalonia between March 2017 and June 2020. Methods: We performed a post hoc analysis of RACECAT to evaluate if the association between initial transport routing and functional outcome differed according to trial enrollment time: daytime (8:00 am –8:59 pm ) and nighttime (9:00 pm –7:59 am ). Primary outcome was disability at 90 days, as assessed by the shift analysis on the modified Rankin Scale score, in patients with ischemic stroke. Subgroup analyses according to stroke subtype were evaluated. Results: We included 949 patients with an ischemic stroke, of whom 258 patients(27%) were enrolled during nighttime. Among patients enrolled during nighttime, direct transport to a thrombectomy-capable center was associated with lower degrees of disability at 90 days (adjusted common odds ratio [acOR], 1.620 [95% CI, 1.020–2.551]); no significant difference between trial groups was present during daytime (acOR, 0.890 [95% CI, 0.680–1.163]; P interaction =0.014). Influence of nighttime on the treatment effect was only evident in patients with large vessel occlusion(daytime, acOR 0.766 [95% CI, 0.548–1.072]; nighttime, acOR, 1.785 [95% CI, 1.024–3.112] ; P interaction P interaction >0.1 for all comparisons). We observed longer delays in alteplase administration, interhospital transfers, and mechanical thrombectomy initiation during nighttime in patients allocated to local stroke centers. Conclusions: Among patients evaluated during nighttime for a suspected acute severe stroke in non-urban areas of Catalonia, direct transport to a thrombectomy-capable center was associated with lower degrees of disability at 90 days. This association was only evident in patients with confirmed large vessel occlusion on vascular imaging. Time delays in alteplase administration and interhospital transfers might mediate the observed differences in clinical outcome. Registration: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT02795962.
Published: 2023
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8. Workflows and Outcomes in Patients With Suspected Large Vessel Occlusion Stroke Triaged in Urban and Nonurban Areas

Author: Alvaro Garcia-Tornel, Monica Millan, Marta Rubiera, Alejandro Bustamante, Manuel Requena, Laura Dorado, Marta Olivé-Gadea, Xavier Jiménez, Angels Soto, Marisol Querol, Maria Hernández-Pérez, Meritxell Gomis, Pere Cardona, Xabier Urra, Francesc Purroy, Yolanda Silva, Xavier Ustrell, Patricia Esteve, Mercè Salvat-Plana, Miquel Gallofré, Carlos Molina, Antoni Dávalos, Tudor Jovin, Sonia Abilleira, Marc Ribo, Natalia Pérez de la Ossa, Marc Ribó Jacobi, Estela Sanjuan, Katherine Santana, Noelia Rodríguez, Jorge Pagola, David Rodriguez-Luna, Olga Maisterra, Estevo Santamarina, Marian Muchada, Jesús Juega, Sandra Boned, Antonio Palasi Franco, Álvaro García -Tornel, Matías Deck, Victoria Sala, Lucía Muñoz, Mónica Millán, Elena López-Cancio, María Hernández-Pérez, Jordi Ciurans, Daniela Samaniego, Tamara Canento, Lorena Martin, Anna Planas, Joaquin Broto, Agustín Sorrentino, Martí Paré, Nuole Zhu, Alicia Garrido, Laia Grau, Ane Miren Crespo, Silvia Presas, Miriam Almendrote, Alba Ramos, Giuseppe Lucente, Lourdes Ispierto, Manuel Lozano, Juan Luis Becerra, Marta Jiménez, Dolores Vilas Rolán, Nicolas Guanyabens, Josep Sanchez-Ojanguren, Alicia Martínez-Piñeiro, Sara Forcén, Mireia Gea, Marta Álvarez, Anna Ramos, Manuel Domínguez Lizarbe, Sara, Rubio Guerra, Irene Bragado, Andrea Arbex, Luis Rodríguez, Pere Cardona Portela, Helena Quesada García, Blanca Lara Rodríguez, Nuria Cayuela, Julia Miró, Clara Marzal, Andrés Paipa, Sergio Campoy, Ana Núñez, Pablo Arroyo, Sarah Besora, Vanessa Adell, Jaume Campdelacreu, Montse Alemany Martí, Belén González, Laura Bau Vila, Marta Fiter Crespo, Anna Berbel, Ma. Cristina Villaescusa Urbaneja, Núria Guillen, Nuria Vidal, Patricia Valentina Vérez Santamaria, Diego Herrero Navarro, Marta Simó, Mercedes Falip, Elisabeth Matas, Neus Mongay Ochoa, Ariadna Gifreu, Albert Muñoz, Lucía Romero, Eduard Portell, Guillermo Hernández Perez, F. Ruiz Esteve, Silvia Teixidor, Augusto Salazar Talavera, Roser Gómez, Xabier Urra Nuin, Martha Vargas, Ángel Chamorro, Sergio Amaro, Laura Llull, Arturo Renú, Salvatore Rudilosso, Raquel Sánchez del Valle, Helena Ariño, Nuria Solà, Delón la Puma, Francisco Gil, Juan Bernardo Gómez, Nuria Matos, Neus Falgàs, Sergi Borrego, Almudena Sánchez, Mircea Balasa, Carmen Montejo, Mar Guasp, David Reyes, Pablo Sánchez Cervilla, Jose Miguel Contador, Victor Augusto Vera Monge, Oscar Ramos, Alejandro Rodríguez, Ana Rodríguez Campello, Gemma Romeral Ballester, Mireia Llop Trujillano, Eva Giralt Steinhauer, Elisa Cuadrado Godia, Angel Javier Ois Santiago, Jordi Jimenez Conde, Joan Martí Fábregas, Daniel Guisado, Luís Prats, Pol Camps, Raquel Delgado, Alejandro Martínez Domeño, Rebeca Marín, David Cànovas, Jordi Estela, Marta Ros, Sonsoles Aranceta, Jordi Espinosa, Marta Rubio, Cristina Lafuente, Oriol Barrachina, Alicia Anguita, Anna Reverter, Carmen García, Gemma Sansa, Mariona Hervas, Monica Crosas, Tania Delgado, Jerzy Krupinski, Sonia Huertas Folch, Gemma Muñoz Gamito, Josep Trenado Alvarez, Teresa Subirana, Jessica Molina, Laura Comes Romero, Gemma Guardia Valls, Miriam Jover, Javier Joaquín Sotova, Sonia Mª García Sánchez, Sebastian Valenzuela (intensivista), Manuel Gómez-Choco, Juan José Mengual, M. Àngels Font, Mª Isabel Gómez Ruiz, Irati Zubizarreta, Susana Fernández González, Laura Gubieras, Carmen E. Cobos, Luis Mena Romo, Nuria Caballol, Luis Cano, Joaquín Serena Leal, Mikel Terceño Izarra, Irene Bragado Trigo, Saima Bashir Viturro, Laura Pardo Albiñana, Montserrat Reina Garrido, Carla Marco Cazcarra, Karol Enrique Uscamaita, Fabian Márquez, Cristina Coll, Mar Irida Lloret Villlas, Berta Solano Vila, Berta Alemany Perna, Daniel López Domínguezl, Mercedes de Lera, Anna Cots Foraster, Urszula Bojaryn, Ikram Benabdelhak, Gerard Mauri Capdevila, Jordi Sanahuja Montesinos, Daniel Vázquez, José Vicente Hervás, Cristina González, Alejandro Quílez, Mikel Vicente Pascual, María Ruiz, Yolanda Riba, M. Pilar Gil Villar, Cristina García, Xavier Ustrell Roig, Mònica Baldrich Mora, Anna Pellisé Guinjoan, Judit Borras, Alba Mañé Martínez, Rafael Marés, Jaume Viñas i Gaya, Laia Seró, Alan Flores, Paula Rodríguez, Gislaine Castilho, Angela Monterde Ortega, Silvia Reverté, Josep Zaragoza, Joan Josep Baiges, Gisela Martín Ozaeta, Sonia Escalante, Iago Payo, Júlia Saura Salvado, Natalia Mas Sala, Josep M Soler Insa, Ester Tio Vilamala, Josep Abos Navarro, Héctor Cruz Tabuenca, Tamara Canento Sánchez, Elisabet Roldán, Elsa Puiggrós Rubiol, Elisabet Franquet, Lidia Fuentes, Javier Donaire, Elena Martí, Laura Giménez, Junior Gil Vázquez, Eloisa Nieto Clara Gris Ambrós, Puri Rodríguez, José Félix Oletta, Pilar Pons Mellado, Catena, Bárbara Gómez, Vergilin Raileau, Esther Catena Ruíz, Oscar Pardina, Jordi Mercadal, María López-Diéguez, Pilar Pérez, Lourdes Gabarró, María Orriols, Joan Josep Canet, Mireia Roca, Muriel Álvaro, Francesc Boneu, Georgina Giménez, Jaume Albà, Francesc Gibert, Jéssica Garcia, Patricia Barragan, Gustavo Jurado, Josep Sabaté Ortega, Joan Amaurys Martínez Solano, Víctor Fernández, Mónica Torres, Ana Belén Montero Alvaredo, Laura Redondo Parejo, Josep Maria Aragonés, Anna Bullón, Cora Loste, Paula González, Neker Bejarano, Francisco Sanchez, Gianni Lucchetti, Xavier Pla, Javier Gimeno, Esteban Reynaga, Montse Barcons, Gabriel Celedón, Juan Ortiz, Goran Anastasovski, Oscar Mascaró, Javier Diez de los Ríos, Meritxell Feliu, Anna Ribera, Cristhian Ruiz, Gerard Corominas, Daniella Nunes, Claudia Roca, Nadina Latorre, Lizbeth Yataco, Maricelys Cruz, Nerea Blanco, Santiago Castejón, Dolores Cocho Calderón, Claustre Pont Sunyer, Jordi Espinosa Garcia, Rodrigo Paz Martin, Albert de Luis Sanchez, Dolores Espinosa Vivas, Juan Valencia Molina, Gemma, Planels Palome, Laia Tomas Chaume, Ares Villagrasa Vilella, Marco Bustamante, Anunciación Boltes, Fernando Rodríguez, Itziar Arrieta, Jordi Ciurans Molist, Barnés Andreu, Ernest Palomeras Soler, Nicolau Guanyabens Buscà, Manuel Daza López, Jordi Bigas Farreres, Virginia Casado Ruiz, Desiree Muriana Batiste, Mª Pilar Sanz Cartagena, Eulalia Cabot de Vega, Josep Bassa Real, Hector Pelaez Roman, César Socolich, Josep Mª Alonso Camp, Antonio Tomás Cano Orgaz, Mª Pilar Fossas Felip, Nicolás Morón, Sandra Bacca, Mauricio Molina, Francesc Casarramona, Lorena Elias, Muhammad Zidane Bukaei, Jose Antonio Martos Gutierrez, Judith Lopez Escuin, Cristina Olaizola, Yolexis López Vargas, Juan Jiménez Oyonarte, Rashida Soultana, Eduardo Sanjurjo Golpe, Esther Salvador, Guillem Vila, Marcos Serrano, Matilde Nuria López Claverol, Marian Lamolla, Miquel Amate, Adriana Rodriguez, Ruth Romero, Montgomery del Carpio, Ana Isabel Hernandez, Julián Martín, M Carmen Rosas, Antonio Nogueroles, Sorilandy Encarnación, Augusto Robles, Jose Antonio Herrera, Roger Gavilán, Toghrol Mameghani, Gastón Araujo, M.Angeles Garrido Morales, Enric Ramon Albert Segui, Eva Fernandez Climent, Francesc Paris Pujol, Mireia Judit Garrofé Seira, Lucía Gómez Pía, Fernando Salleres Nuñez, Cristina Aguar Peñalver, Cristina Vaz Lopes, Elisenda Ribera Tasa, Carmen Repullo Vilchez, Modesto Sánchez Zambrana, Beatriu Suescun Ribas, Inés Vilà Panés, Montserrat Vila Planavila, Alicia Vaqueiro Lorenzo, Meritxell Sabartés Guixes, Jorge Medina, David Sambrano, Javi Zamarreño, Carmen Pirela, Paola Vélez, Luis Cajamarca, Honey Pérez, Yarles Martínez, Jesus Alexander Gonçalves, Carles Regordosa, Claudia Mormeneo, Laura Griu, Maria Francia Colina, Enric Farik, Dolors Carrión Duch, Carles Badenas, Oscar Bernal, Núria Agramunt, Shyrlei Morales, Victoria Reynoso, Miguel Guerrero, Primitivo Romera Cid, Mònica Folqué, Claudia Pedroza, Adnan Hachem, Íñigo Soteras Martínez, Xavier Verdera García, Mercè López Amorós, Xavier Costa Subirós, Marta Cufí Benet, Cecile Van Eendenburg, Teresa Osuna, Dra Gina Santos, Mireia Pallisera, Lluís Gonzalo Oliva, Gemma Sanchez, Xavier Basurto, Ludgi Vivoda, Richard Van der Kleyn, Laura Robles, Ana Cabanelas Barranco, Mª Cruz Almendros, Marc Pérez Oliveras, Amelia Fernández Álvarez, Maria Rybyeva, Antoni Viñas, Maria Barcons, Joaquim Danès Alberto Tavera, Pablo Burbano, Cintie López, David Cruz, Paula Bisbe, Nora Fernández, Juan Carlos Palacio, Eduardo Fraiz, Oriol Aguiló, Rollmy Amorodjo, John Velázquez, Elena Sánchez, Jaume Español, Judit Perez de Celis, Anna Coll, Glòria Díaz, Margarida Vergés i Sala, Mª Ángels Casas Capdevila, Yosmairy Yoselin Ferrini, Aitor Gorriz, Diego-Javier Cucurell Navarro, Dulce Velásquez, Jaume Pla Soler, Josep González, Julian David Higuera, Lina Cuellar, Liza Margarita Miniello, Lluis Pujol, Sorin Cracan, Mora Vives, M Angela, Lopez Lopez, null Anabel, Montse Gorchs Molist, Delofeu Anna, Silvia Solà Muñoz, Ferreres Yolanda, Carol Pujalte, Elisabeth Téllez Marín, Yolanda Font Casas, Sara Hernández Luque, Joaquim Mejias Sendra, Francesca Mellado Valero, Campos Escala, Galup De Lacanal, Lopez Diaz, Castillo Paramio, Català Estopà, Español Moreda, Calafell Majo, Carballo Almeida, Castro Naval, M Elena, Cregut Ruiz, Pere Lluis, Deulofeu Font, Fabregat Sanjuan, Joan Pere, Fontquerni Gorchs, Forés Bellés, Joan Antoni, Gomez Herrera, Juan Carlos, Hijazo Prades, Itzaina Torvisco, Marti Rovira, Obiols Gonzalez, Olive Cavero, Querol Gil, M Soledad, Rico Rodriguez, Rios Sambernardo, Ropero Molina, Jose Ramon, Sanchez Valero, Santos Arevalo, Maria Jose, Soto Garcia, Maria Angeles, Tebar Escribano, Torres Garcia, Pedro José, Verdes Carrion, M Isabel, Verge Lopez, Juan Jose, Lopez Canela, M Angeles, Lucas Guarque, Morales Alvarez, Jorge Arnulfo, Morell Fornieles, Subirats Gomez, Maria Teresa, Torello Masa, Val Lopez, Maria Mercedes, Palmero Reyes, Juan Manuel, Rodriguez Forne, Ruana Turiel, Sanchez Gonzalez, Sebastia Gornals, Abrio Rico, Albert Gual Falip, Aznar Oliveros, Brugues Perotti, Calvet Molinero, Catells Franco, Corrales Medina, Daroca Miro, Duran Marquez, Feliu Pradas, Figueras Casanova, Garcia Tortajada, Grajera Gallego, Azahara del Mar, Las Arroyo, Dulcenombre de Jesus, Laserreta Sanz, Llambrich Vidal, Monllao Corral, Morales de la Cruz, Moreno Blanco, Orejuela Orgaz, Piñana Suazo, Pons Minguillon, Rodríguez Gómez, José Carlos, Rodriguez Pereira, Sedo Porcel, Sevil Villar, Sierra Lopez, Torres Romero, Trepat Calveres, Ventura Arnella, Acera Gil, M Teresa, Biesot Vico, Castro Galea, Rosa Maria, Escorcia Chafer, Juan Antonio, Gil Faure, Hernandez Luque, Jimenez Delgado, Mallafre Gay, M. Angel, Olivares Sanzo, Pubill Fondevila, Sabate Fort, Segovia Agámez, Antonio Carlos, Turata Longaretti, Valle Hernandez, Trayner Guixens, Aguilar Valor, Aguirre Alvarez, Alastrue Naval, Alferez Baquero, Alonso Marne, Cristina, Aloy Orozco, Alvarez Colino, Alvarez Peñuela, Ameller Cirilo, Maria Vanessa, Andujar Guerrero, Estefania Remedios, Auladell Sillero, Azcarate Sorbet, Basany Genesca, Benavent Barduena, Benito Tudela, Blanch Pardo, Boluda Pla, Bonilla Gonzalez, Bru Serramalera, Burnat Andreu, Caballero Calvo-Rayo, Calvo Casellas, Camps Mombiela, Cano Bresme, Carcelen Fernandez, Caro Solà, Casasas Matavacas, Castanedo Bolado, Cerdeña Cortina, Abella Jane, Laia Collado Borrego, Colom Orri, Comellas Vilanova, Conillera Sole, Cots Torres, Cuchi Estepa, de Sostoa Graell, del Rio Lopez, Delgado Compte, Diaz Bueno, Diaz Maneiro, Andrea Lucia, Domenech Palau, M. Carme, Duran Carasso, Pablo Javier, El Abidi, Escoda Martin, Esteve Casanovas, Fernandez Perez, Ferre Quintero, Flores Escobar, Fontcuberta Abad, Fortes del Valle, Mª Luisa, Franco Quesada, Sara Maria, Franco Romero, Gallego Francisco, Gamiz Gala, Alicia, Garcia Castañeda, Garcia Rodriguez, Casajuana Gemma, Gomez Arroyo, Gomez Girbau, Gonzalez Fernandez, Gonzalez Gonzalez, Gonzalez Lopez, Gual Obeso, Guerrero Blanco, Guillemas Roca, Guillermo Eudaldo, Hernandez Sebastia, Homar Covas, Hostench Alvarez, Huerta Royo, Ibañez Barcelo, Izquiero Cruz, Jaione Urdangarin Etxetxikia, Juárez Gimenez, Khlifi Alami, Laparra Gasco, Lara Moreales, Lasaosa Medina, Maria Lourdes, Lazaro Cava, Leon Berrar, López Maturana, Lopez Ramirez, López Romero, Lorente Marco, Lorenzo Martin, Lozano Casals, Lujan Nicola, Madrid Diaz, Jose Antonio, Maria Teresa Peris Morales, Marti Clemente, Martinez Castaño, David Natanael, Martinez Gamez, Martinez Gonzalez, Martinez Quesada, Maria de la Cabeza, Marzà Fusté, Mireia, M Conillera, Melendo Lasheras, Miralles Mestre, Montserrat Puig Pastalle, Montserrate Vidal, Mora Mellado, Morales Ponce, Morera Cabre, Morera Vivet, Mundi Souza, Muñoz Quiles, Navea Rosa, Neira Iglesias, M Asuncion, Nuñez Manrique, Mª Pilar, Obiols Martinez, Pallares Reig, Partegas Torres, Pascual Berengueras, Perez Gamez, Perez Oset, Perez Restrepo, Maria Alejandra, Planas Yeste, Planells Mangado, Puertas Carbonell, Quinteiro Blanco, Rebollar Benavente, Recasens Fernandez, Reyes Gomez, Maria del Carmen, Roca Pou, Rodriguez Ferrer, Rodriguez Franco, Rodriguez Fuertes, Rodriguez Muñoz, Rodriguez Navarro, Roig Iniesta, Romero Gracia, Rovira Brunet, Rovira de Eugenio, Rubio Baena, Ruiz Llorens, Sala Llamazares, San Juan Alaez, Sanchez Segura, Santasusana Soldevilla, Sanz Salmeron, Segura Perez, Serra Balcells, Sorina Dumitras, Teixidor Montoya, Tena Vicente, Tierno Salinas, Ester Ines, Toledo Testa, Tomàs Figueras, Tomas Ruperez, Torrano Garcia-Penche, Torres Esparza, Florenc Uku, Vazquez Blanco, Vazquez De la Paz, Vazquez Gil, Vazquez Gonzalez, Vidal Meler, Vilalta Cots, Vilches Jimenez, Visser Fernandez, Viu Gavin, Vives Vives, Carmona Jimenez, Francisco Jose, Galobardes Vilches, Gonzalez Gomez, Jodar Manzanera, Marimon Cortes, Marrodan Orive, Martin Bosch, Martorell Lopez, Millares Roca, M Jesus, Miralles Guri, Muliterno Hernandez, Muñoz Oliva, Pons Pujol, Postius Conde, Prieto Arruñada, Quintana Mathé, Rosell Mata, Alvarin Alvarez, Arrufat Flores, Bañuelos Pago, Campo Vilar, Cardus Hidalgo, Castello Gil, Maria Gloria, Castrillo Montsesinos, Clave Garces, Comes Sanroma, Descalzo Sequi, Errando Ricol, Escudero Campillo, Maria del Mar, Fernandez Alvarez, Fernandez Gomez, Franch Espinosa, Freixes Graells, Garcia Plaza, Garcia-Marron Gallego, Hernandez Simancas, Jaraba Armas, Jimenez Ramos, Lopez Oganissian, Madrona Romero, M Rosa, Martinez Millan, Martínez Morón, Merchan Encinas, Moreno Jimenez, Muñoz Rico, Navarro Rodriguez, Nogales Ibañez, Rita Maria, Novillo Viera, Parella Torrabadella, Mari Alba, Pera Villar, Carla Montserrat, Rabella Miralles, Ramis Trilles, Ribera Ortiz, Sastre Perez, Soro Borrega, Tapia Fores, Tellez Bernad, Trenado Alvarez, Felix Martin, Herrero Perez, Lanau Fuster, Lopez Gomariz, Pardo Lozano, Quintana Altimiras, Ventosa Lopez, Almodovar Damian, Alvarez Monterroso, Arambudo Comas, Boullon Garzon, Burgos Capella, Campuzano Garcia, Maria Del Mar, Capdevila Olivas, Carre Marti, Clara Isabel, Carretero Bacaicoa, Cazorla Calderon, Chacon Osuna, Cortes Planas, Cortit Olio, Delhomme Estany, Fructuos Martinez, Garcia Lopez, Pascual Luis, Genis Amill Vallve, Gonzalez Muñoz, Hernaez Ventura, Lavernia Gimenez, Martin Rojo, Martínez Medina, Mayol Barrera, Mercader Pi, Merino Dalmau, Miguel Campodarbe, Moreno Sanchez, Francisco Javier, Alba Mari, Penela Barrameda, Prieto Ajenjo, Rodriguez Pareja, Rodríguez Piñar, Javier Francisco, Rodriguez Tello, Romero Pereda, Sainz Saborido, Santacana Martin, Santos Martinez, Sierra Chavez, Turbau Torres, Vicente Domenech, Villena Esteo, Vives Pertegaz, Baena Gonzalez, Bagaria Pont, Beltran Sanchez, Bonet Alarcon, Borch Ballescà, Buhils Clanchet, Closa Garcia, Colomina Carril, Cristina Fernandez Sanchez, Fernandez Toledo Edurne Judith, Espases Rodriguez, Espert Resa, F Xavier, Fernández Sol, Gonzalez Lugo, Gracia Llesera, Herrada Lendinez, Juliench Liesa, Sandra Alicia, Lopez Ortega, Lopez Yuste, Lozano Villanueva, Lucia Sola Soto, Madrid Castellano, Megias Cano, Daniel Jesus, Montasell Ponce, Montesinos Sospedra, Moya Martinez, Muñoz Herrera, Ocaña Fernandez, Palau Bernal, Perez Sole, Ramon Carbonell, Ribas Lop, Rubio López, Rueda Lopez, Sabat Castaño, Sabata Lara, Salmerón Ramírez, Serra Creus, Serrano Dalmau, Sumasi Mainero, Gonzalo Gabriel, Valencia Primo, Ana Maria Viñuales Muñoz, Josep Roig, and Verònica Hidalgo.
Subjects: Stroke, Advanced and Specialized Nursing, Treatment Outcome, Endovascular Procedures, Humans, Neurology (clinical), Cardiology and Cardiovascular Medicine, Brain Ischemia, Ischemic Stroke, Thrombectomy, Workflow
Abstract: Background: We aim to compare the outcome of patients from urban areas, where the referral center is able to perform thrombectomy, with patients from nonurban areas enrolled in the RACECAT trial (Direct Transfer to an Endovascular Center Compared to Transfer to the Closest Stroke Center in Acute Stroke Patients With Suspected Large Vessel Occlusion). Methods: Patients with suspected large vessel occlusion stroke, as evaluated by a Rapid Arterial Occlusion Evaluation score of ≥5, from urban catchment areas of thrombectomy-capable centers during RACECAT trial enrollment period were included in the Stroke Code Registry of Catalonia. Primary outcome was disability at 90 days, as assessed by the shift analysis on the modified Rankin Scale score, in patients with an ischemic stroke. Secondary outcomes included mortality at 90 days, rate of thrombolysis and thrombectomy, time from onset to thrombolysis, and thrombectomy initiation. Propensity score matching was used to assemble a cohort of patients with similar characteristics. Results: The analysis included 1369 patients from nonurban areas and 2502 patients from urban areas. We matched 920 patients with an ischemic stroke from urban areas and nonurban areas based on their propensity scores. Patients with ischemic stroke from nonurban areas had higher degrees of disability at 90 days (median [interquartle range] modified Rankin Scale score, 3 [2–5] versus 3 [1–5], common odds ratio, 1.25 [95% CI, 1.06–1.48]); the observed average effect was only significant in patients with large vessel stroke (common odds ratio, 1.36 [95% CI, 1.08–1.65]). Mortality rate was similar between groups(odds ratio, 1.02 [95% CI, 0.81–1.28]). Patients from nonurban areas had higher odds of receiving thrombolysis (odds ratio, 1.36 [95% CI, 1.16–1.67]), lower odds of receiving thrombectomy(odds ratio, 0.61 [95% CI, 0.51–0.75]), and longer time from stroke onset to thrombolysis (mean difference 38 minutes [95% CI, 25–52]) and thrombectomy(mean difference 66 minutes [95% CI, 37–95]). Conclusions: In Catalonia, Spain, patients with large vessel occlusion stroke triaged in nonurban areas had worse neurological outcomes than patients from urban areas, where the referral center was able to perform thrombectomy. Interventions aimed at improving organizational practices and the development of thrombectomy capabilities in centers located in remote areas should be pursued. Registration: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT02795962.
Published: 2022
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9. Clinical characterisation of patients in the post-acute stage of anti-NMDA receptor encephalitis: a prospective cohort study and comparison with patients with schizophrenia spectrum disorders

Author: Mar Guasp, Mireia Rosa-Justicia, Amaia Muñoz-Lopetegi, Eugenia Martínez-Hernández, Thais Armangué, Gisela Sugranyes, Heike Stein, Roger Borràs, Laia Prades, Helena Ariño, Jesús Planagumà, Elena De-La-Serna, Domingo Escudero, Sara Llufriu, Raquel Sánchez-Valle, Joan Santamaria, Albert Compte, Josefina Castro-Fornieles, Josep Dalmau, Dolores Páramo, Vicente Medrano, Virginia Casado, Nicolau Guanyabens, Eloi Giné-Servén, María Ángeles del Real, Javier Pardo, Leticia Martin-Gil, Francisco Javier Barrero-Hernández, Nuria García-Barragán, Mercè Falip, Marta Simó, Eloy Rodríguez, Juan José Ruiz Ezquerro, Luis Bataller, Gemma Safont, José Vicente-Hervàs, Luis Brieva, Ignacio Casado, Juan Carlos Portilla, Sònia Escalante, Juan Francisco Arenillas, Elena Erro, Ivonne Jericó-Pascual, Alejandro Fuerte-Hortigón, Alba Morató, Albert Saiz, Yolanda Blanco, Maria Sepúlveda, Raquel Ruiz, Laura Naranjo, Maria Rodés, Esther Aguilar, Mercè Alba, and Eva Caballero
Subjects: Anti-N-Methyl-D-Aspartate Receptor Encephalitis, Schizophrenia, Humans, Prospective Studies, Neurology (clinical), Nijmegen Breakage Syndrome, Biomarkers
Abstract: Anti-NMDA receptor (NMDAR) encephalitis is associated with a post-acute stage that is not well known. We aimed to describe the clinical features of this stage, similarities with schizophrenia spectrum disorders, and the factors that predict cognitive-psychiatric outcomes and could serve as prognostic biomarkers.In this prospective cohort study, participants (aged 12-60 years) with anti-NMDAR encephalitis during the post-acute stage visited Hospital Clínic de Barcelona (Barcelona, Spain) on three occasions (at study entry [V1], at 6 months [V2], and at 12 months [V3]) and underwent comprehensive neuropsychiatric evaluations. Similar evaluations were done in a group of age-matched participants with schizophrenia spectrum disorders and a group of age-matched and sex-matched healthy participants also recruited from Hospital Clínic de Barcelona. We analysed differences between and within groups in the longitudinal follow-up using multilevel linear mixed-effect models, adjusting for group, age, sex, and socioeconomic status to control for possible confounding.Between Jan 1, 2017, and Sept 30, 2020, 82 participants were recruited, 28 (34%) with anti-NMDAR encephalitis, 27 (33%) with schizophrenia spectrum disorders, and 27 (33%) healthy participants. Although, by V1 (median 4 months [IQR 3-7] from disease onset), many acute-stage symptoms in participants with anti-NMDAR encephalitis had resolved (acute stage median modified Rankin Scale [mRS] score 5 [IQR 4-5] vs V1 mRS score 2 [1-2]; p0·0001), 25 (89%) participants showed deficits in at least one cognitive domain. In this group, 15 (68%) of 22 cognitive domain variables were impaired at V1, whereas only eight (36%) were altered at V3 (p=0·016). In participants with schizophrenia spectrum disorders, 11 (50%) of 22 variables (all shared with participants with anti-NMDAR encephalitis) were impaired at V1, without changes at V3. Two acute-stage features of anti-NMDAR encephalitis (ie, decreased consciousness and no improvement within the first 4 weeks of treatment) predicted cognitive domain outcomes, and a visuospatial task (ie, serial biases) at V1 showed potential in predicting learning and memory outcomes. At V1, all psychiatric symptom clusters were similarly altered in participants with anti-NMDAR encephalitis and in those with schizophrenia spectrum disorders, but only those in individuals with anti-NMDAR encephalitis subsequently improved (p=0·031). The greatest cognitive-psychiatric improvement in participants with anti-NMDAR encephalitis occurred between V1 and V2. During this interval, four (14%) participants with anti-NMDAR encephalitis would have met the diagnostic criteria of schizophrenia if CSF antibody findings had not been investigated.The cognitive-psychiatric symptoms of anti-NMDAR encephalitis in the post-acute stage resembled those of stabilised schizophrenia, but only those in participants with anti-NMDAR encephalitis progressively improved, predominantly during V1-V2. These findings are important for clinical trials on anti-NMDAR encephalitis and suggest that prompt cognitive-psychosocial rehabilitation might be a valuable intervention.Instituto Salud Carlos III, NEURON Network of European Funding for Neuroscience Research, National Alliance for Research in Schizophrenia and Affective Disorders, and la Caixa Health-Research Foundation.
Published: 2022
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10. Predicting Functional Outcome and Response to Therapy of Anti-NMDAR Encephalitis, Already at Diagnosis: The NEOS2 Model. (P5-5.009)

Author: Juliette Brenner, Anna Bastiaansen, Marienke de Bruijn, Frank Leypoldt, Jerome Honnorat, Josep Dalmau, Takahiro Iizuka, Mareike Jansen, Ina Schroder, Sergio Muniz-Castrillo, Yvette Crijnen, Juna de Vries, Marco Schreurs, Amaia Munoz, Mar Guasp, Peter Sillevis Smitt, and Maarten Titulaer
Published: 2023
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11. Clinical characterisation of patients in the post-acute stage of anti-NMDA receptor encephalitis: a prospective cohort study and comparison with patients with schizophrenia spectrum disorders (S22.006)

Author: Mar Guasp, Mireia Rosa-Justicia, Amaia Muñoz-Lopetegi, Eugenia Martinez-Hernandez, Thais Armangue, Gisela Sugranyes, Heike Stein, Laia Prades, Helena Ariño, Jesús Planagumà, Elena De La Serna, Domingo Escudero, Sara Llufriu, Raquel Sánchez-Valle, Joan Santamaria, Albert Compte, Josefina Castro-Fornieles, and Josep Dalmau
Published: 2023
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12. Clinical, Neuroimmunologic, and CSF Investigations in First Episode Psychosis

Author: Mireia Rosa-Justicia, Ester Boix-Quintana, Albert Saiz, Eloi Giné-Servén, Estibaliz Maudes, Josefina Castro-Fornieles, Yasmina Módena-Ouarzi, Eugenia Martinez-Hernandez, Eva Davi-Loscos, Gisela Sugranyes, Francesc Graus, V. Casado, Josep Dalmau, Eduard Parellada, Isabella Pacchiarotti, Desiree Muriana, Miquel Bioque, Cristina Torres-Rivas, Lidia Sabater, Nicolau Guanyabens, José Ríos, and Mar Guasp
Subjects: Psychosis, medicine.medical_specialty, medicine.diagnostic_test, biology, business.industry, Electroencephalography, medicine.disease, Gastroenterology, Immunoglobulin G, 030227 psychiatry, 03 medical and health sciences, 0302 clinical medicine, CSF pleocytosis, First episode psychosis, Internal medicine, medicine, biology.protein, Immunohistochemistry, Neurology (clinical), Antibody, business, 030217 neurology & neurosurgery, Encephalitis
Abstract: ObjectivesTo report the neuropsychiatric features and frequency of NMDA receptor (NMDAR) and other neuronal immunoglobulin G antibodies in patients with first episode psychosis (FEP) and to assess the performance of reported warning signs and criteria for autoimmune psychosis (AP).MethodsThis was a prospective observational study of patients with FEP assessed for neuropsychiatric symptoms, serum and CSF neuronal antibodies (brain immunohistochemistry, cell-based assays, live neurons), and warning signs and criteria of AP. Previous autoimmune FEP series were reviewed.ResultsOne hundred five patients were included; their median age was 30 (range 14–75) years, and 44 (42%) were female. None had neuronal antibodies. Two of 105 (2%) had CSF pleocytosis, 4 of 100 (4%) had brain MRI abnormalities, and 3 of 73 (4%) EEG alterations. Thirty-four (32%) and 39 (37%) patients fulfilled 2 sets of warning signs of AP, and 21 (20%) fulfilled criteria of possible or probable AP, yet none developed AP. The cause of FEP was psychiatric in 101 (96%) and nonpsychiatric in 4 (4%). During this study, 3 patients with psychosis caused by anti-NMDAR encephalitis were transferred to our center; 2 did not meet criteria for possible AP. Of 1,159 reported patients with FEP, only 7 (1%) had CSF studies; 36 (3%) had serum NMDAR antibodies (without definite diagnosis of AP), and 4 had CSF NMDAR antibodies (3 classic anti-NMDAR encephalitis and 1 with isolated psychiatric features).ConclusionsNMDAR antibodies were not found in patients with FEP unless they had anti-NMDAR encephalitis. Warning signs and criteria for AP have limited utility when neurologic symptoms are absent or paraclinical tests are normal. A diagnostic algorithm for autoimmune FEP is provided.
Published: 2021
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13. Association of PSP phenotypes with survival: A brain-bank study

Author: Esteban Muñoz, Oriol Grau, Nuria Caballol, Alexandra Pérez-Soriano, Celia Painous, Ellen Gelpi, Mar Guasp, Francesc Valldeoriola, Alicia Garrido, Ana Cámara, Guenter H. Höglinger, Gesine Respondek, Almudena Sánchez-Gómez, María José Martí, Yaroslau Compta, and Laura Molina-Porcel
Subjects: Male, 0301 basic medicine, Oncology, classification [Supranuclear Palsy, Progressive], physiopathology [Supranuclear Palsy, Progressive], Richardson's syndrome, Survival, PSP-PGF, Disease, diagnosis [Supranuclear Palsy, Progressive], Cohort Studies, 0302 clinical medicine, diagnosis [Parkinsonian Disorders], Corticobasal degeneration, classification [Parkinsonian Disorders], PSP, Middle Aged, Prognosis, Phenotype, mortality [Parkinsonian Disorders], Neurology, physiopathology [Parkinsonian Disorders], Practice Guidelines as Topic, Cohort, Female, Supranuclear Palsy, Progressive, PSP-P, medicine.medical_specialty, Tissue Banks, mortality [Supranuclear Palsy, Progressive], Sensitivity and Specificity, 03 medical and health sciences, Atrophy, Parkinsonian Disorders, Internal medicine, medicine, Humans, ddc:610, Pathological, Aged, Retrospective Studies, business.industry, medicine.disease, Survival Analysis, eye diseases, 030104 developmental biology, Clinical diagnosis, Brain bank, Neurology (clinical), Geriatrics and Gerontology, business, 030217 neurology & neurosurgery
Abstract: Introduction The MDS-PSP criteria expand the phenotypic spectrum of PSP by adding to Richardson's syndrome (PSP-RS) other presentations such as PSP-parkinsonism (PSP–P), PSP-pure-gait-freezing (PSP-PGF), PSP-speech-language (PSP-SL), PSP-frontal (PSP–F), PSP-postural-instability (PSP-PI) and PSP-corticobasal-syndrome (PSP-CBS). Evidence about the prognostic differences between PSP phenotypes is scarce and focused on PSP-RS vs. non-PSP-RS. Using a brain-bank cohort we assessed PSP survival not only in PSP-RS vs. non-PSP-RS, but also in PSP-RS + cortical vs. subcortical phenotypes. Besides, we assessed sensitivity and specificity of the MDS-PSP criteria in of PSP and other degenerative parkinsonisms. Methods We retrospectively applied the MDS-PSP diagnostic criteria to 32 definite PSP cases and 30 cases with other degenerative parkinsonian syndromes (Parkinson's disease [PD; n = 11], multiple system atrophy [MSA; n = 11], corticobasal degeneration [CBD; n = 8]). We conducted survival statistics in neuropathologically confirmed PSP cases considering PSP-RS vs. non-PSP-RS and PSP-RS + PSP-cortical (PSP–F + PSP-SL + PSP-CBS) vs. PSP-subcortical (PSP–P + PSP-PGF) phenotypes. We also adjusted survival analyses for PSP tau scores. Results Diagnostic sensitivity was 100% and specificity ranged from 47% to 87% when excluding cases that met the “suggestive of PSP” definition early in their disease course but with other clinical features better matching with a non-PSP pathological diagnosis. Survival was significantly shorter in PSP-RS vs. non-PSP-RS cases, but it was more markedly shorter in PSP-RS + PSP-cortical vs. PSP-subcortical, independently of PSP tau scores, which were not associated with survival. Conclusions PSP-subcortical phenotypes appear to have longer survival than PSP-RS and cortical phenotypes. This might be of prognostic relevance when informing patients upon clinical diagnosis.
Published: 2021
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14. Author Response: Clinical, Neuroimmunologic, and CSF Investigations in First Episode Psychosis

Author: Mar Guasp and Josep Dalmau
Subjects: Psychotic Disorders, Neuroimmunomodulation, Humans, Neurology (clinical)
Published: 2022

15. Neurofilament Light Chain Levels in Anti-NMDAR Encephalitis and Primary Psychiatric Psychosis

Author: Mar Guasp, Lorena Martín-Aguilar, Lidia Sabater, Miquel Bioque, Thaís Armangué, Eugenia Martínez-Hernández, Jon Landa, Estibaliz Maudes, Roger Borràs, Amaia Muñoz-Lopetegi, Albert Saiz, Josefina Castro-Fornieles, Francesc Graus, Eduard Parellada, Luis Querol, and Josep Dalmau
Subjects: Anti-N-Methyl-D-Aspartate Receptor Encephalitis, Psychotic Disorders, Neurofilament Proteins, Intermediate Filaments, Humans, Neurology (clinical), Encephalitis, Herpes Simplex, Biomarkers
Abstract: Background and ObjectivesAn important challenge in diagnosing anti–NMDA receptor (NMDAR) encephalitis (NMDARe) is differentiating it from a first episode of psychosis (FEP) caused by a psychiatric disease (pFEP). CSF antibody testing distinguishes these diseases, but spinal taps are difficult to obtain in psychiatric facilities. A separate problem is the lack of biomarkers of NMDARe severity and outcome. Here we assessed the performance of neurofilament light chain (NfL) testing in these settings.MethodsIn this observational study, NfL levels were determined with single-molecule array in patients with NMDARe, pFEP, herpes simplex encephalitis (HSE), and healthy participants (HC), with the last 2 groups used as controls. Receiver operating characteristic (ROC) analyses were performed to assess the prediction accuracy of serum NfL (sNfL) levels for NMDARe and pFEP and to obtain clinically useful cutoffs.ResultsOne hundred eighteen patients with NMDARe (33 with isolated psychosis at presentation), 45 with pFEP, 36 with HSE, and 36 HC were studied. Patients with NMDARe with seizures/status epilepticus, intensive care unit admission, and CSF pleocytosis (>20 white blood cells/µL) and without early immunotherapy were more likely to have higher NfL (mainly in CSF) than individuals with NMDARe without these features. NfL levels at diagnosis of NMDARe did not correlate with outcome at 1-year follow-up assessed with the modified Rankin Scale. Patients with NMDARe had significantly higher sNfL than individuals with pFEP and HC and lower sNfL than patients with HSE. ROC analysis of sNfL between NMDARe with isolated psychosis and pFEP provided an area under the curve of 0.93 (95% CI 0.87–0.99) and an sNfL cutoff ≥15 pg/mL to distinguish these disorders (sensitivity 85%, specificity 96%, positive likelihood ratio 19.3). Forty-three of 45 (96%) patients with pFEP had sNfLp < 0.001). None of the patients with HSE and 35 of 36 (97%) HC had sNfLDiscussionNfL measured at diagnosis of NMDARe associated with features of disease severity but not with long-term outcome. Young patients with FEP and sNfL ≥15 pg/mL had a 120 times higher chance of having NMDARe than those with pFEP. This cutoff correctly classified 96% of patients with pFEP and 85% of patients with NMDARe with isolated psychosis. Patients with FEP of unclear etiology and sNfL ≥15 pg/mL should undergo CSF NMDAR antibody testing.
Published: 2022

16. Thyrotoxic Psychosis Associated With Amiodarone

Author: Alex Mesa, Marta Gómez-Ramiro, Gerard Anmella, Mar Guasp, Eduard Parellada, and Nuria Baldaquí
Subjects: Psychiatry and Mental health, Psychosis, Pediatrics, medicine.medical_specialty, Text mining, business.industry, medicine, MEDLINE, Pharmacology (medical), business, medicine.disease, Amiodarone, medicine.drug
Published: 2021
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17. Clinical significance of anti-NMDAR concurrent with glial or neuronal surface antibodies

Author: Josep Dalmau, Tamir Ben-Hur, Albert Saiz, Mar Guasp, Anna García-Serra, Takahiro Iizuka, Thaís Armangue, Estibaliz Maudes, Maria Sepúlveda, Ana P. Ramos, Helena Ariño, Francesc Graus, and Eugenia Martinez-Hernandez
Subjects: 0301 basic medicine, Pathology, medicine.medical_specialty, biology, Glial fibrillary acidic protein, business.industry, medicine.disease, Myelin oligodendrocyte glycoprotein, 03 medical and health sciences, 030104 developmental biology, 0302 clinical medicine, nervous system, Neuroblastoma, medicine, biology.protein, Immunohistochemistry, Optic neuritis, Neurology (clinical), Antibody, business, 030217 neurology & neurosurgery, Immunostaining, Encephalitis
Abstract: ObjectiveTo determine the frequency and significance of concurrent glial (glial-Ab) or neuronal-surface (NS-Ab) antibodies in patients with anti–NMDA receptor (NMDAR) encephalitis.MethodsPatients were identified during initial routine screening of a cohort (C1) of 646 patients consecutively diagnosed with anti-NMDAR encephalitis and another cohort (C2) of 200 patients systematically rescreened. Antibodies were determined with rat brain immunostaining and cell-based assays.ResultsConcurrent antibodies were identified in 42 patients (4% from C1 and 7.5% from C2): 30 (71%) with glial-Ab and 12 (29%) with NS-Ab. Glial-Ab included myelin oligodendrocyte glycoprotein (MOG) (57%), glial fibrillary acidic protein (GFAP) (33%), and aquaporin 4 (AQP4) (10%). NS-Ab included AMPA receptor (AMPAR) (50%), GABAa receptor (GABAaR) (42%), and GABAb receptor (8%). In 39 (95%) of 41 patients, concurrent antibodies were detected in CSF, and in 17 (41%), concurrent antibodies were undetectable in serum. On routine clinical-immunologic studies, the presence of MOG-Ab and AQP4-Ab was suggested by previous episodes of encephalitis or demyelinating disorders (8, 27%), current clinical-radiologic features (e.g., optic neuritis, white matter changes), or standard rat brain immunohistochemistry (e.g., AQP4 reactivity). GFAP-Ab did not associate with distinct clinical-radiologic features. NS-Ab were suggested by MRI findings (e.g., medial temporal lobe changes [AMPAR-Ab], or multifocal cortico-subcortical abnormalities [GABAaR-Ab]), uncommon comorbid conditions (e.g., recent herpesvirus encephalitis), atypical tumors (e.g., breast cancer, neuroblastoma), or rat brain immunostaining. Patients with NS-Ab were less likely to have substantial recovery than those with glial-Ab (5 of 10 [50%] vs 17 of 19 [89%], p = 0.03).ConclusionsBetween 4% and 7.5% of patients with anti-NMDAR encephalitis have concurrent glial-Ab or NS-Ab. Some of these antibodies (MOG-Ab, AQP4-Ab, NS-Ab) confer additional clinical-radiologic features and may influence prognosis.
Published: 2020
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18. Author Response: Neurofilament Light Chain Levels in Anti-NMDAR Encephalitis and Primary Psychiatric Psychosis

Author: Mar Guasp and Josep Dalmau
Subjects: Neurology (clinical)
Published: 2022
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19. Thymoma and Autoimmune Encephalitis: Clinical Manifestations and Antibodies

Author: Solange Kapetanovic, Takahiro Iizuka, Raquel Ruiz-García, Jon Landa, Jesús Planagumà, Luis Bataller, Albert Saiz, Lidia Sabater, Francesc Graus, Pedro Sánchez, Masataka Nakamura, Mar Guasp, Makoto Kinoshita, Mateus Mistieri Simabukuro, Eugenia Martinez-Hernandez, Kenichi Kaida, Jordi Bruna, Amaia Muñoz-Lopetegi, Josep Dalmau, Masanori Kurihara, and Laura Naranjo
Subjects: 0301 basic medicine, Encephalomyelitis, 0302 clinical medicine, hemic and lymphatic diseases, Immunologia, Aged, 80 and over, biology, Limbic encephalitis, Brain, Middle Aged, surgical procedures, operative, Estudi de casos, Neurology, DISEASES, Encephalitis, medicine.symptom, Antibody, Adult, Thymoma, Immunology, chemical and pharmacologic phenomena, CASE SERIES, PERM, Antibodies, Article, 03 medical and health sciences, Young Adult, Autoimmune Diseases of the Nervous System, Antigen, medicine, Humans, AMPA RECEPTOR ENCEPHALITIS, LIMBIC ENCEPHALITIS, neoplasms, Aged, Retrospective Studies, Autoimmune encephalitis, SPECTRUM, business.industry, Encefalitis, PROFILES, medicine.disease, 030104 developmental biology, biology.protein, AUTOANTIBODIES, Case studies, Neurology (clinical), business, Myoclonus, 030217 neurology & neurosurgery
Abstract: ObjectiveTo report the clinical, neuroimaging, and antibody associations in patients with autoimmune encephalitis (AE) and thymoma.MethodsA retrospective cohort study of 43 patients was conducted. Antibody determination and immunoprecipitation to characterize novel antigens were performed using reported techniques.ResultsPatients' median age was 52 years (range: 23–88 years). Forty (93%) had neuronal surface antibodies: gamma-aminobutyric acid receptor A (GABAAR) (15), amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) (13), contactin-associated protein-like 2 (CASPR2) (4), leucine-rich, glioma inactivated 1 (LGI1) (3), glycine receptor (GlyR) (3), and unknown antigens (2). Concurrent antibodies against intracellular antigens occurred in 13 (30%; 9 anti–collapsin response mediator protein 5 [CRMP5]) and were more frequent in anti-AMPAR encephalitis (54% vs 20%; p = 0.037). The most common clinical presentation was encephalitis with multiple T2/fluid-attenuated inversion recovery hyperintense lesions in 23 (53%) patients (15 GABAAR, 5 AMPAR, and 1 unknown neuropil antibody), followed by encephalitis with peripheral nerve hyperexcitability in 7 (16%; 4 CASPR2, 2 LGI1, and 1 unknown antibody), limbic encephalitis in 6 (14%; 4 AMPAR, 1 LGI1, and 1 antibody negative), progressive encephalomyelitis with rigidity and myoclonus in 4 (9%; 3 GlyR and 1 AMPAR antibodies), and encephalitis with normal MRI in 3 (7%; AMPAR antibodies). Anti-GABAAR encephalitis was more prevalent in Japanese patients compared with Caucasians and other ethnicities (61% vs 16%; p = 0.003). In anti-AMPAR encephalitis, 3/4 patients with poor and 0/6 with good outcome had concurrent CRMP5 antibodies (p = 0.033). Immunoprecipitation studies identified metabotropic glutamate receptor 3 antibodies that were additionally found in 5 patients (3 with and 2 without encephalitis).ConclusionsAE in patients with thymoma include several clinical-radiologic syndromes that vary according to the associated antibodies. Anti-GABAAR encephalitis was the most frequent AE and occurred more frequently in Japanese patients.
Published: 2021

20. Incidence and Impact of COVID-19 in MS A Survey From a Barcelona MS Unit

Author: Albert Saiz, Irene Pulido-Valdeolivas, Montse Artola, Ana Hernando, Laura Llansó, Domingo Escudero, Francesc Graus, Eugenia Martinez-Hernandez, Maria Sepúlveda, Marta Aldea, Elisabeth Solana, Eloy Martinez-Heras, Martí Català, Carmen Montejo, Yolanda Blanco, Sara Llufriu, Mar Guasp, Clara Prats, Universitat Politècnica de Catalunya. Doctorat en Física Computacional i Aplicada, Universitat Politècnica de Catalunya. Departament de Física, and Universitat Politècnica de Catalunya. BIOCOM-SC - Grup de Biologia Computacional i Sistemes Complexos
Subjects: Male, Cross-sectional study, Comorbidity, COVID-19 (Malaltia), Cohort Studies, COVID-19 (Disease), 0302 clinical medicine, Modelització en etapes múltiples, Surveys and Questionnaires, Electronic Health Records, Multiscale modeling, Cumulative incidence, 030212 general & internal medicine, Epidemies, Aged, 80 and over, education.field_of_study, Matemàtiques i estadística::Anàlisi numèrica::Modelització matemàtica [Àrees temàtiques de la UPC], Incidence, Incidence (epidemiology), Medical record, Middle Aged, Hospitalization, Treatment Outcome, Neurology, Antirheumatic Agents, Cohort, Female, Cohort study, Adult, medicine.medical_specialty, Multiple Sclerosis, Expanded disability severity scale, Population, Article, Young Adult, 03 medical and health sciences, Sex Factors, Internal medicine, medicine, Disease-modifying therapy, Humans, Epidemics, education, Aged, SARS-CoV-2, business.industry, COVID-19, medicine.disease, Cross-Sectional Studies, Spain, Neurology (clinical), business, 030217 neurology & neurosurgery
Abstract: ObjectiveTo investigate the incidence of coronavirus disease 2019 (COVID-19) in a single-center cohort of patients with MS and to explore the contribution of their comorbidities and therapies to the outcome.MethodsA cross-sectional mixed-method study was conducted involving an email-based, self-administered questionnaire sent on May 21, 2020, to 586 patients with MS followed at the MS Unit of Hospital Clinic, University of Barcelona, along with telephone interview, and review of electronic medical records until June 18, 2020. The cumulative incidence of confirmed COVID-19 (positive PCR or antibody test) and all COVID-19 cases (confirmed and suspected) from the start of the pandemic was compared with the population estimates for Barcelona.ResultsA total of 407 patients (69.5%) completed the survey. Most of the responders (67%) were female. The responders had a median age of 48 years (range 19–86), relapsing-remitting disease (84%), at least 1 comorbidity (45%), and were on disease-modifying therapy (DMT; 74.7%). COVID-19 was confirmed in 5 patients (1.2%) and suspected in 46 (11.3%). The cumulative incidence of confirmed COVID-19 cases was similar to that of the general population but was almost 2-fold higher when all cases were considered (p < 0.001). Six patients (11.7%) were hospitalized, of which 5 had good recovery and 1 died. Hospitalized patients were more frequently male, had diabetes and had progressive forms of MS (p < 0.05). DMT was not associated with the risk of infection or the outcome.ConclusionsIn the studied MS cohort, the incidence of COVID-19 was higher than that of the general population; however, most patients did not require hospitalization and had a good outcome despite the frequent presence of comorbidities and treatment with DMT.
Published: 2021

21. Seizure-related 6 homolog like 2 autoimmunity Neurologic syndrome and antibody effects

Author: Lorena García-Fernández, Mar Petit-Pedrol, Jan J.G.M. Verschuuren, Lidia Sabater, Albert Saiz, Josep Dalmau, Liliana Ramirez-Gomez, Rachel Saunders-Pullman, Mar Guasp, Jon Landa, Jesús Planagumà, Michael D. Geschwind, Eugenia Martinez-Hernandez, Francesc Graus, and Raquel Ruiz-García
Subjects: Male, 0301 basic medicine, Pathology, medicine.medical_specialty, Cerebellar Ataxia, Neuroimmunology, Autoimmunity, Autoanticossos, Antígens, Clinical immunology, Article, Epitope, Immunoglobulin G, 03 medical and health sciences, 0302 clinical medicine, Antigen, Neuropil, medicine, Immunologia clínica, Animals, Humans, Antigens, Aged, Autoantibodies, Cerebellar ataxia, biology, business.industry, Middle Aged, Neuroimmunologia, Rats, 030104 developmental biology, medicine.anatomical_structure, Neurology, Gait Ataxia, biology.protein, Immunohistochemistry, Female, Neurology (clinical), medicine.symptom, Antibody, business, 030217 neurology & neurosurgery
Abstract: ObjectiveTo describe the clinical syndrome of 4 new patients with seizure-related 6 homolog like 2 antibodies (SEZ6L2-abs), study the antibody characteristics, and evaluate their effects on neuronal cultures.MethodsSEZ6L2-abs were initially identified in serum and CSF of a patient with cerebellar ataxia by immunohistochemistry on rat brain sections and immunoprecipitation from rat cerebellar neurons. We used a cell-based assay (CBA) of HEK293 cells transfected with SEZ6L2 to test the serum of 95 patients with unclassified neuropil antibodies, 331 with different neurologic disorders, and 10 healthy subjects. Additional studies included characterization of immunoglobulin G (IgG) subclasses and the effects of SEZ6L2-abs on cultures of rat hippocampal neurons.ResultsIn addition to the index patient, SEZ6L2-abs were identified by CBA in 3/95 patients with unclassified neuropil antibodies but in none of the 341 controls. The median age of the 4 patients was 62 years (range: 54–69 years), and 2 were female. Patients presented with subacute gait ataxia, dysarthria, and mild extrapyramidal symptoms. Initial brain MRI was normal, and CSF pleocytosis was found in only 1 patient. None improved with immunotherapy. SEZ6L2-abs recognized conformational epitopes. IgG4 SEZ6L2-abs were found in all 4 patients, and it was the predominant subclass in 2. SEZ6L2-abs did not alter the number of total or synaptic SEZ6L2 or the AMPA glutamate receptor 1 (GluA1) clusters on the surface of hippocampal neurons.ConclusionsSEZ6L2-abs associate with a subacute cerebellar syndrome with frequent extrapyramidal symptoms. The potential pathogenic effect of the antibodies is not mediated by internalization of the antigen.
Published: 2021

22. Evolution of clinical‐pathological correlation of early‐onset Alzheimer's disease: 1994–2009 vs 2010–2017

Author: Albert Lladó, Teresa Ximelis, Laura Molina, Raquel Sánchez-Valle, Jordi Sarto, Gerard Mayà, Mar Guasp, Sergi Borrego, Mircea Balasa, José Contador, Ivan Aldecoa, Ellen Gelpi, and Iban Archilla
Subjects: Pathology, medicine.medical_specialty, Epidemiology, business.industry, Health Policy, Neuropathology, medicine.disease, Psychiatry and Mental health, Cellular and Molecular Neuroscience, Developmental Neuroscience, medicine, Early-onset Alzheimer's disease, Neurology (clinical), Geriatrics and Gerontology, business, Pathological correlation
Published: 2020
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23. Absence of GluD2 Antibodies in Patients With Opsoclonus-Myoclonus Syndrome

Author: Andres Morales La Madrid, Mar Petit-Pedrol, Albert Saiz, Mar Guasp, Cinzia Lavarino, Thaís Armangue, Francesc Graus, and Josep Dalmau
Subjects: Adult, Male, Pathology, medicine.medical_specialty, Null Hypothesis, Cell, Purkinje cell, 03 medical and health sciences, 0302 clinical medicine, Glutamate Dehydrogenase, Neuroblastoma, Opsoclonus myoclonus syndrome, medicine, Humans, 030212 general & internal medicine, Child, Autoantibodies, Opsoclonus-Myoclonus Syndrome, biology, business.industry, Glutamate receptor, Infant, Middle Aged, medicine.disease, Primary and secondary antibodies, medicine.anatomical_structure, Child, Preschool, biology.protein, Immunohistochemistry, Female, Neurology (clinical), Antibody, business, 030217 neurology & neurosurgery
Abstract: Objective:A recent study showed glutamate receptor delta 2 antibodies (GluD2-ab) in sera of patients with opsoclonus-myoclonus syndrome (OMS). Inconsistencies between cerebellar immunoreactivity and expression of GluD2 led us to hypothesize that these antibodies are not biomarkers of OMS.Methods:Serum of 45 children with OMS (10 [22%] with neuroblastoma), 158 adults with OMS (53 [33%] with tumors), and 172 controls including 152 patients with several types of encephalitis, 18 with neuroblastoma without OMS, and 20 healthy participants were investigated. Antibodies were determined with 3 different techniques: (1) rat brain immunohistochemistry, (2) a live cell-based assay using a standard secondary antibody (two-step CBA), and (3) a similar CBA with a secondary and tertiary antibodies (three-step CBA). Two plasmids were used in the CBA studies. Three commercial GluD2-ab and two human sera with GluD2-ab served as controls for expression of GluD2.Results:The three commercial GluD2-ab showed predominant reactivity with the molecular and Purkinje cell layers (where GluD2 is highly enriched), and were also positive with the indicated CBAs. Substantially milder reactivity with brain tissue and CBA was obtained with the two control human sera containing GluD2-ab. None of the 203 patients with OMS and 172 controls showed immunoreactivities consistent with GluD2-abs. Compared with a standard two-step CBA, the three-step assay did not improve antibody detection and showed more frequent non-specific reactivity that was not immunoabsorbed with GluD2.Conclusion:We did not find GluD2-ab in a large cohort of patients with OMS. GluD2-ab should not be considered diagnostic biomarkers of OMS.
Published: 2020

24. Encephalitis with Autoantibodies against the Glutamate Kainate Receptors GluK2

Author: GluK2 encephalitis study group, Jon Landa, Mar Guasp, Federico Míguez-Cabello, Joana Guimarães, Takayasu Mishima, Fumiko Oda, Frauke Zipp, Vladimir Krajinovic, Peter Fuhr, Jerome Honnorat, M.J. (Maarten) Titulaer, Mateus Simabukuro, Jesus Planagumà, Eugenia Martínez-Hernández, Thais Armangué, Albert Saiz, Xavier Gasull, David Soto, Francesc Graus, Lidia Sabater, Josep Dalmau, GluK2 encephalitis study group, Jon Landa, Mar Guasp, Federico Míguez-Cabello, Joana Guimarães, Takayasu Mishima, Fumiko Oda, Frauke Zipp, Vladimir Krajinovic, Peter Fuhr, Jerome Honnorat, M.J. (Maarten) Titulaer, Mateus Simabukuro, Jesus Planagumà, Eugenia Martínez-Hernández, Thais Armangué, Albert Saiz, Xavier Gasull, David Soto, Francesc Graus, Lidia Sabater, and Josep Dalmau
Abstract: Objective: The objective of this study was to report the identification of antibodies against the glutamate kainate receptor subunit 2 (GluK2-abs) in patients with autoimmune encephalitis, and describe the clinical-immunological features and antibody effects. Methods: Two sera from 8 patients with similar rat brain immunostaining were used to precipitate the antigen from neuronal cultures. A cell-based assay (CBA) with GluK2-expressing HEK293 cells was used to assess 596 patients with different neurological disorders, and 23 healthy controls. GluK2-ab effects were determined by confocal microscopy in cultured neurons and electrophysiology in GluK2-expressing HEK293 cells. Results: Patients’ antibodies precipitated GluK2. GluK2 antibody-specificity was confirmed by CBA, immunoprecipitation, GluK2-immunoabsorption, and GluK2 knockout brain immunohistochemistry. In 2 of 8 samples, antibodies reacted with additional GluK2 epitopes present in GluK1 or GluK3; in both, the reactivity was abrogated after GluK2 immuno-absorption. Six of 8 patients developed acute encephalitis and clinical or magnetic resonance imaging (MRI) features of predominant cerebellar involvement (4 presenting as cerebellitis, which in 2 patients caused obstructive hydrocephalus), and 2 patients had other syndromes (1 with cerebellar symptoms). One of the samples showed mild reactivity with non-kainate receptors (alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptors [AMPAR] and N-methyl-D-aspartate receptors [NMDAR]) leading to identify 6 additional cases with GluK2-abs among patients with anti-AMPAR (5/71) or anti-NMDAR encephalitis (1/73). GluK2-abs internalized GluK2 in HEK293 cells
Published: 2021
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25. Encefalitis por anticuerpos contra el receptor de NMDA

Author: Josep Dalmau and Mar Guasp
Subjects: 0301 basic medicine, 03 medical and health sciences, 030104 developmental biology, 0302 clinical medicine, business.industry, Medicine, General Medicine, business, Humanities, 030217 neurology & neurosurgery
Abstract: Resumen La encefalitis asociada a anticuerpos contra el receptor N-metil-D-aspartato (NMDAR) se caracteriza por la presencia de anticuerpos contra la subunidad GluN1 del NMDAR, resultando en sintomas parecidos a los observados en modelos de alteracion genetica o antagonistas farmacologicos del receptor. Los pacientes suelen ser adultos jovenes, predominantemente mujeres, y ninos/as que presentan de manera rapida y secuencial: psicosis, movimientos anormales, disfuncion autonomica y coma. Las crisis epilepticas son variables y pueden ocurrir en cualquier momento de la evolucion. La enfermedad suele confundirse con encefalitis virales, procesos psiquiatricos primarios, ingesta de drogas y sindrome neuroleptico maligno. El 50% de las mujeres jovenes tienen un teratoma de ovario; en ninas y varones, la presencia de un tumor es infrecuente. En algunos pacientes la enfermedad es iniciada por una encefalitis herpetica. El reconocimiento de la encefalitis anti-NMDAR es importante porque, a pesar de su gravedad, la mayoria de los pacientes responden a la inmunoterapia.
Published: 2018
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26. Psychiatric and Cognitive Profile in the Post-Acute Stage of Anti-NMDA Receptor Encephalitis: A Comparative Analysis with Patients with Schizophrenia. (1806)

Author: Rosa-Justicia, Mireia, primary, Mar, Guasp, additional, Muñoz-Lopetegi, Amaia, additional, Martinez-Hernandez, Eugenia, additional, Prades, Laia, additional, Sugranyes, Gisela, additional, Compte, Albert, additional, Dalmau, Josep, additional, and Castro-Fornieles, Josefina, additional
Published: 2021
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27. Evidence of neurophysiological improvement of early manifestations of small-fiber dysfunction after liver transplantation in a patient with familial amyloid neuropathy

Author: Michela Campolo, Jordi Casanova-Molla, Alejandro Kohler, Josep Valls-Solé, and Mar Guasp
Subjects: Nervous system, Pathology, medicine.medical_specialty, medicine.medical_treatment, Quantitative sensory testing, 030204 cardiovascular system & hematology, Liver transplantation, Asymptomatic, Small fiber polyneuropathy, lcsh:RC321-571, 03 medical and health sciences, 0302 clinical medicine, Physiology (medical), medicine, lcsh:Neurosciences. Biological psychiatry. Neuropsychiatry, Psychophysical testing, business.industry, Familial amyloid neuropathy, medicine.disease, Transplantation, medicine.anatomical_structure, Nociception, Neurology, Clinical and Research Article, Nociceptive evoked potentials, Neurology (clinical), medicine.symptom, business, Polyneuropathy, Familial amyloidosis, 030217 neurology & neurosurgery, Sensory nerve
Abstract: Highlights • Detecting signs of neuropathy helps therapeutic decisions in familial amyloidosis. • Psychophysical thermal testing may be the only test showing damage in small fibers. • Quantitative signs of improvement may remain a few years after liver transplantation., Introduction Small fiber polyneuropathy (SFP) is a common heralding clinical manifestation of damage to the nervous system in patients with familial amyloidosis. The diagnosis of SFP is a significant factor in the decision to treat a previously asymptomatic gene carrier, as treatment would prevent irreversible nerve damage. This requires detection of the earliest but unequivocal signs of peripheral nerve involvement. Case report We present the case of a young female who was diagnosed of SFP, supported by data from quantitative sensory testing. She had preserved sensory nerve action potentials in the distalmost nerves of her feet and recordable nociceptive evoked potentials. She was successfully transplanted the liver from a previously healthy donor, and recovered fully of her symptoms and signs. Improvement was documented with repeated psychophysical and electrodiagnostic testing in the course of 4 years after transplantation. Significance This case illustrates the utility of psychophysical testing to support the diagnosis of SFP.
Published: 2018

28. Author Response: Clinical, Neuroimmunologic, and CSF Investigations in First Episode Psychosis

Author: Mar Guasp, Francesc Graus, and Josep Dalmau
Subjects: First episode, Psychosis, Echolalia, Pediatrics, medicine.medical_specialty, medicine.diagnostic_test, Neuroimmunomodulation, Catatonia, business.industry, Cognition, Electroencephalography, medicine.disease, Level of consciousness, Psychotic Disorders, medicine, Humans, Neurology (clinical), medicine.symptom, business, Encephalitis
Abstract: We appreciate the interest in our research.1 According to Pollak and colleagues,2 criteria of possible autoimmune psychosis (AP) are fulfilled if a patient has abrupt onset psychotic symptoms with at least one of the following: the presence of a tumor, movement disorder (dyskinesias, catatonia), adverse response to antipsychotics, “severe or disproportionate” cognitive dysfunction, decreased level of consciousness, unexplained seizures, and significant autonomic dysfunction.2 Fulfilment of these criteria should lead to additional tests such as EEG, MRI, and serum or CSF investigations. In our series of 105 patients with first episode of psychosis (FEP), 20% fulfilled these criteria but never developed AP.1 We confirm that 2 of 3 patients with anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis presenting with FEP did not fulfill any of these criteria, including catatonia, which is a complex syndrome with its own set of 12 criteria that include echolalia.3 Thus, catatonia and echolalia should not be used as interchangeable terms.
Published: 2021
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29. Higher Solar Irradiance Is Associated With a Lower Incidence of Coronavirus Disease 2019

Author: Carlos Laredo, Xabier Urra, and Mar Guasp
Subjects: Microbiology (medical), 2019-20 coronavirus outbreak, Veterinary medicine, Coronavirus disease 2019 (COVID-19), Ultraviolet Rays, Climate, 010501 environmental sciences, Solar irradiance, Global Health, 01 natural sciences, Population density, 03 medical and health sciences, 0302 clinical medicine, Medicine, Humans, 030212 general & internal medicine, 0105 earth and related environmental sciences, Sunlight, Population Density, business.industry, SARS-CoV-2, climatological factors, Incidence (epidemiology), Incidence, Brief Report, Outbreak, COVID-19, solar irradiance, Lower incidence, Infectious Diseases, AcademicSubjects/MED00290, business
Abstract: We studied the relationship between the incidence of coronavirus disease 2019 (COVID-19), demographical, and climatological measurements in different regions across the world. Lower solar irradiance and higher population density were independent predictors of greater COVID-19 outbreaks. Further studies on the potential protective effect of sunlight over COVID-19 are warranted.
Published: 2020

30. Clinical features of seronegative, but CSF antibody-positive, anti-NMDA receptor encephalitis

Author: Yasmina Módena, Mar Guasp, Thaís Armangue, Francesc Graus, Josep Dalmau, and Receptors neurals
Subjects: Adult, Male, Adolescent, Article, Young Adult, Interquartile range, Neoplasms, Medicine, Animals, Humans, Receptor, Autoantibodies, Retrospective Studies, Anti-NMDA receptor encephalitis, Anti-N-Methyl-D-Aspartate Receptor Encephalitis, biology, Neural receptors, business.industry, Age Factors, Encefalitis, Middle Aged, Rat brain, medicine.disease, Rats, Neurology, Immunology, biology.protein, NMDA receptor, Encephalitis, Female, Neurology (clinical), Antibody, business, Neural receptor, Immunostaining
Abstract: ObjectiveTo determine the frequency of anti-NMDA receptor encephalitis without detectable serum NMDAR antibodies and to compare the clinical features of these patients with those with NMDAR antibodies in serum and CSF.MethodsThis is a retrospective assessment of serum antibody status and clinical features of 489 patients with anti-NMDAR encephalitis, defined by the presence of NMDAR antibodies in the CSF, and available paired serum/CSF samples examined at Hospital Clínic-Institut d’Investigacions Biomèdiques August Pi I Sunyer, Barcelona, between January 2007 and December 2017. NMDAR antibodies were determined with rat brain immunostaining, in-house cell-based assay (CBA), and a commercial CBA. Patients were considered seronegative if NMDAR antibodies were undetectable with the 3 indicated techniques.ResultsSerum NMDAR antibodies were not detected in 75 of 489 (15%) patients. Compared with the 414 seropositive patients, the seronegative were older (23.5 years [interquartile range (IQR): 17–43] vs 20.5 [IQR: 14–31]; p < 0.0001) and less frequently women (39 [52%] vs 313 [76%]; p < 0.001) and had less tumors (6 [9%] vs 128 [32%]; p < 0.001). In multivariate analysis, older age at diagnosis (odds ratio [OR]: 1.35 [per decade]; 95% confidence interval [CI]: 1.10–1.67), absence of tumor (OR: 0.14; 95% CI: 0.05–0.43), and less need for intensive care unit admission (OR: 0.35; 95% CI: 0.18–0.69) were independent variables associated with the absence of serum NMDAR antibodies. Time to diagnosis, treatment with immunotherapy, relapses, and outcome were similar in seronegative and seropositive patients.ConclusionsNMDAR antibodies are not detected in the serum of 15% of the patients with anti-NMDAR encephalitis. These patients appear to be older and have milder neurologic symptoms with less frequency of tumors.
Published: 2020

31. Neuropathological validation of the MDS-PSP criteria with PSP and other frontotemporal lobar degeneration

Author: Maria Grazia Spillantini, James B. Rowe, Kieren Allinson, Deborah A. Hall, Gesine Respondek, Yaroslau Compta, Stefano Gazzina, Günter U. Höglinger, Irene Litvan, Laura Molina-Porcel, Shirin Moftakhar, Mar Guasp-Verdaguer, and Stephen G. Reich
Subjects: 0303 health sciences, Pediatrics, medicine.medical_specialty, business.industry, Frontotemporal lobar degeneration, medicine.disease, Supranuclear gaze palsy, eye diseases, Progressive supranuclear palsy, Primary progressive aphasia, 03 medical and health sciences, 0302 clinical medicine, medicine, False positive paradox, Corticobasal degeneration, business, 030217 neurology & neurosurgery, 030304 developmental biology, Frontotemporal dementia
Abstract: BackgroundProgressive supranuclear palsy (PSP) is clinically heterogeneous. Clinical diagnostic criteria were revised in 2017, to increase sensitivity and operationalize the diagnosis of PSP Richardson’s syndrome (PSP-RS) and “variant” syndromes (vPSP).ObjectivesTo determine the (1) sensitivity and specificity of the 1996 NINDS-SPSP and 2017 MDS-PSP criteria; (2) false positive rates in frontotemporal dementia with frontotemporal lobar degeneration (FTLD); and (3) clinical evolution of variant PSP syndromes (vPSP).MethodsRetrospective multicenter review of 108 neuropathologically-confirmed PSP patients and 81 patients with other forms of FTLD: 38 behavioral variant frontotemporal dementia (bvFTD), 14 non-fluent/agrammatic variant primary progressive aphasia (nfvPPA), and 29 corticobasal degeneration (CBD), Sensitivity and specificity of the MDS-PSP criteria were compared to the NINDS-SPSP criteria at baseline. In a subset of cases, the timing and frequency of clinical features were compared across groups over six years.ResultsSensitivity for recognition of probable and possible PSP pathology was higher by MDS-PSP criteria (72.2-100%) than NINDS-SPSP criteria (48.1-61.1%). Specificity was higher by NINDS-SPSP criteria (97.5-100%) than MDS-PSP criteria (53.1-95.1%). False positives by MDS-PSP criteria were few for bvFTD (10.5-18.4%) but common for CBD and nfvPPA (fulfilling “suggestive of’ PSP). Most vPSP cases developed PSP-RS-like features within six years, including falls and supranuclear gaze palsy, distinguishing frontal presentations of PSP from bvFTD, and speech/language presentations of PSP from nfvPPA.ConclusionsThe 2017 MDS-PSP criteria successfully identify PSP, including variant phenotypes. This independent validation of the revised clinical diagnostic criteria strengthens the case for novel therapeutic strategies against PSP to include variant presentations.
Published: 2019
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32. Cerebellar ataxia and autoantibodies restricted to glutamic acid decarboxylase 67 (GAD67)

Author: Nuria Sola-Valls, Josep Dalmau, M Pilar Gil, Francesc Graus, Eugenia Martinez-Hernandez, Helena Ariño, Mar Guasp, Luis Brieva, Cristina González, and Albert Saiz
Subjects: 0301 basic medicine, Gene isoform, endocrine system, medicine.medical_specialty, Cerebellar Ataxia, endocrine system diseases, Immunology, Glutamate decarboxylase, Article, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, Humans, Immunology and Allergy, Autoantibodies, biology, Cerebellar ataxia, Glutamate Decarboxylase, Autoantibody, nutritional and metabolic diseases, Middle Aged, Titer, 030104 developmental biology, Endocrinology, Neurology, Etiology, biology.protein, Biomarker (medicine), Female, Neurology (clinical), Antibody, medicine.symptom, Biomarkers, 030217 neurology & neurosurgery
Abstract: Cerebellar ataxia is one of the most frequent syndromes associated with autoantibodies against glutamic acid de-carboxylase (GAD-ab). Antibodies recognize the isoform GAD65, which is the standard biomarker, but additional immunoreactivity against GAD67 is found in high proportion of patients with GAD-ab-associated neurological disorders. We describe the case of a 59-year-old woman who presented with pancerebellar syndrome of subacute onset (9 weeks to nadir). In the etiological study, high titers of GAD-ab were found, but these only recognized the GAD67 isoform and not the GAD65. Screening of GAD67-ab should be considered in late-onset cerebellar ataxia when GAD65-ab are absent.
Published: 2016
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33. Antibody-associated CNS syndromes without signs of inflammation in the elderly

Author: Helena Ariño, Mar Guasp, Francesc Graus, Josep Dalmau, Eugenia Martinez-Hernandez, Carles Gaig, and Domingo Escudero
Subjects: 0301 basic medicine, Central Nervous System, Male, medicine.medical_specialty, Aging, Cell Adhesion Molecules, Neuronal, Gastroenterology, Antibodies, Article, 03 medical and health sciences, 0302 clinical medicine, Antigen, CSF pleocytosis, Internal medicine, medicine, Humans, Aged, Retrospective Studies, Autoimmune encephalitis, Aged, 80 and over, Inflammation, Sleep disorder, Cerebellar ataxia, Clinical pathology, business.industry, Age Factors, Intracellular Signaling Peptides and Proteins, Proteins, Chorea, Middle Aged, medicine.disease, 030104 developmental biology, HEK293 Cells, Receptors, GABA-B, Female, Neurology (clinical), Differential diagnosis, medicine.symptom, Nervous System Diseases, business, 030217 neurology & neurosurgery
Abstract: Objective:To report the CNS syndromes of patients ≥60 years of age with antibodies against neuronal surface antigens but no evidence of brain MRI and CSF inflammatory changes.Methods:This was a retrospective clinical analysis of patients with antibodies against neuronal surface antigens who fulfilled 3 criteria: age ≥60 years, no inflammatory abnormalities in brain MRI, and no CSF pleocytosis. Antibodies were determined with reported techniques.Results:Among 155 patients ≥60 years of age with neurologic syndromes related to antibodies against neuronal surface antigens, 35 (22.6%) fulfilled the indicated criteria. The median age of these 35 patients was 68 years (range 60–88 years). Clinical manifestations included faciobrachial dystonic seizures (FBDS) in 11 of 35 (31.4%) patients, all with LGI1 antibodies; a combination of gait instability, brainstem dysfunction, and sleep disorder associated with IgLON5 antibodies in 10 (28.6%); acute confusion, memory loss, and behavioral changes suggesting autoimmune encephalitis (AE) in 9 (25.7%; 2 patients with AMPAR, 2 with NMDAR, 2 with GABAbR, 2 with LGI1, and 1 with CASPR2 antibodies); and rapidly progressive cognitive deterioration in 5 (14.3%; 3 patients with IgLON5 antibodies, 1 with chorea; 1 with DPPX antibody–associated cerebellar ataxia and arm rigidity; and 1 with CASPR2 antibodies).Conclusions:In patients ≥60 years of age, the correct identification of characteristic CNS syndromes (FBDS, anti-IgLON5 syndrome, AE) should prompt antibody testing even without evidence of inflammation in MRI and CSF studies. Up to 15% of the patients developed rapidly progressive cognitive deterioration, which further complicated the differential diagnosis with a neurodegenerative disorder.
Published: 2017

34. Clinical Neuropathology image 4-2017: High-resolution 7 Tesla MRI of postmortem brain specimens: improving neuroimaging-neuropathology correlations

Author: Núria Bargalló, Raquel Sánchez-Valle, Oriol Grau-Rivera, Alberto Prats-Galino, Ellen Gelpi, Mar Guasp-Verdaguer, and Guadalupe Soria
Subjects: medicine.medical_specialty, Pathology, 7T, High resolution, Neuroimaging, Neuropathology, postmortem brain imaging, 030218 nuclear medicine & medical imaging, Pathology and Forensic Medicine, 03 medical and health sciences, 0302 clinical medicine, medicine, Humans, 7 tesla mri, medicine.diagnostic_test, Postmortem brain, business.industry, Brief Report, Brain, Magnetic resonance imaging, General Medicine, Magnetic Resonance Imaging, Neurology, high-resolution MRI, Autopsy, Neurology (clinical), Radiology, business, 030217 neurology & neurosurgery
Abstract: No Abstract available.
Published: 2017
Full Text: View/download PDF

35. Encefalitis autoinmunes

Author: Mar Guasp Verdaguer, Helena Ariño, and Josep Dalmau Obrador
Subjects: Neurology (clinical), General Medicine
Published: 2018
Full Text: View/download PDF

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35 results on '"Mar Guasp"'

1. ALLOSTERIC MODULATION OF NMDAR REVERSES ANTI-NMDAR ENCEPHALITIS IN A MOUSE MODEL OF THIS DISEASE

2. The diagnosis of anti-LGI1 encephalitis varies with the type of immunodetection assay and sample examined

3. CSF Biomarkers in COVID-19 Associated Encephalopathy and Encephalitis Predict Long-Term Outcome

4. Limitations of a Commercial Assay as Diagnostic Test of Autoimmune Encephalitis

5. Cycloid psychosis as a psychiatric expression of anti‐NMDAR encephalitis. A systematic review of case reports accomplished with the authors' cooperation

6. Evidence of neurophysiological improvement of early manifestations of small-fiber dysfunction after liver transplantation in a patient with familial amyloid neuropathy

7. Association of Time of Day With Outcomes Among Patients Triaged for a Suspected Severe Stroke in Nonurban Catalonia

8. Workflows and Outcomes in Patients With Suspected Large Vessel Occlusion Stroke Triaged in Urban and Nonurban Areas

9. Clinical characterisation of patients in the post-acute stage of anti-NMDA receptor encephalitis: a prospective cohort study and comparison with patients with schizophrenia spectrum disorders

10. Predicting Functional Outcome and Response to Therapy of Anti-NMDAR Encephalitis, Already at Diagnosis: The NEOS2 Model. (P5-5.009)

11. Clinical characterisation of patients in the post-acute stage of anti-NMDA receptor encephalitis: a prospective cohort study and comparison with patients with schizophrenia spectrum disorders (S22.006)

12. Clinical, Neuroimmunologic, and CSF Investigations in First Episode Psychosis

13. Association of PSP phenotypes with survival: A brain-bank study

14. Author Response: Clinical, Neuroimmunologic, and CSF Investigations in First Episode Psychosis

15. Neurofilament Light Chain Levels in Anti-NMDAR Encephalitis and Primary Psychiatric Psychosis

16. Thyrotoxic Psychosis Associated With Amiodarone

17. Clinical significance of anti-NMDAR concurrent with glial or neuronal surface antibodies

18. Author Response: Neurofilament Light Chain Levels in Anti-NMDAR Encephalitis and Primary Psychiatric Psychosis

19. Thymoma and Autoimmune Encephalitis: Clinical Manifestations and Antibodies

20. Incidence and Impact of COVID-19 in MS A Survey From a Barcelona MS Unit

21. Seizure-related 6 homolog like 2 autoimmunity Neurologic syndrome and antibody effects

22. Evolution of clinical‐pathological correlation of early‐onset Alzheimer's disease: 1994–2009 vs 2010–2017

23. Absence of GluD2 Antibodies in Patients With Opsoclonus-Myoclonus Syndrome

24. Encephalitis with Autoantibodies against the Glutamate Kainate Receptors GluK2

25. Encefalitis por anticuerpos contra el receptor de NMDA

26. Psychiatric and Cognitive Profile in the Post-Acute Stage of Anti-NMDA Receptor Encephalitis: A Comparative Analysis with Patients with Schizophrenia. (1806)

27. Evidence of neurophysiological improvement of early manifestations of small-fiber dysfunction after liver transplantation in a patient with familial amyloid neuropathy

28. Author Response: Clinical, Neuroimmunologic, and CSF Investigations in First Episode Psychosis

29. Higher Solar Irradiance Is Associated With a Lower Incidence of Coronavirus Disease 2019

30. Clinical features of seronegative, but CSF antibody-positive, anti-NMDA receptor encephalitis

31. Neuropathological validation of the MDS-PSP criteria with PSP and other frontotemporal lobar degeneration

32. Cerebellar ataxia and autoantibodies restricted to glutamic acid decarboxylase 67 (GAD67)

33. Antibody-associated CNS syndromes without signs of inflammation in the elderly

34. Clinical Neuropathology image 4-2017: High-resolution 7 Tesla MRI of postmortem brain specimens: improving neuroimaging-neuropathology correlations

35. Encefalitis autoinmunes

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35 results on '"Mar Guasp"'

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