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1. Second intravenous immunoglobulin dose in patients with Guillain-Barré syndrome with poor prognosis (SID-GBS): a double-blind, randomised, placebo-controlled trial

Author

Walgaard, Christa, Jacobs, Bart C., Lingsma, Hester F., Steyerberg, Ewout W., Van den Berg, Bianca, Doets, Alexandra Y., Leonhard, Sonja E., Verboon, Christine, Huizinga, Ruth, Drenthen, Judith, Arends, Samuel, Kleine Budde, Ilona, Kleyweg, Ruud P., Kuitwaard, Krista, Van der Meulen, Marjon F.G., Samijn, Johnny P.A., Vermeij, Frederique H., Kuks, Jan B.M., Van Dijk, Gert W., Wirtz, Paul W., Eftimov, Filip, Van der Kooi, Anneke J., Garssen, Marcel P.J., Gijsbers, Cees J., De Rijk, Maarten C., Visser, Leo H., Blom, Roderik J., Linssen, Wim H.J.P., Van der Kooi, Elly L., Verschuuren, Jan J.G.M., Van Koningsveld, Rinske, Dieks, Rita J.G., Gilhuis, H. Job, Jellema, Korné, Van der Ree, Taco C., Bienfait, Henriette M.E., Faber, Karin G., Lovenich, Harry, Van Engelen, Baziel G.M., Groen, Rutger J., Merkies, Ingemar S.J., Van Oosten, Bob W., Van der Pol, W. Ludo, Van der Meulen, Willem D.M., Badrising, Umesh A., Stevens, Martijn, Breukelman, Albert-Jan J., Zwetsloot, Casper P., Van der Graaff, Maaike M., Wohlgemuth, Marielle, Hughes, Richard A.C., Cornblath, David R., Van Doorn, Pieter A., Althingh van Geusau, R.B., Van Boheemen, C.J.M., Bronner, I.M., Feenstra, B., Fokke, C., Hoogendoorn, T.A., Van Houten, R., Hovestad, A., Jansen, P.J.H.W., Keuter, E., Krudde, J., Linn, F.H.H., Lion, J., Manschot, S.M., Mellema, S.J., Molenaar, D.S.M., Nieuwkamp, D.J., Oenema, D.G., Van Oostrom, J.C.H., Van Orshoven, N.P., Van der Ploeg, R.J.O., Polman, S., Ruitenberg, A., Ruts, L., Schyns-Soeterboek, A.J.G.M., Trip, R., Jacobs, Bart C, Lingsma, Hester F, Steyerberg, Ewout W, van den Berg, Bianca, Doets, Alexandra Y, Leonhard, Sonja E, Budde, Ilona Kleine, Kleyweg, Ruud P, van der Meulen, Marjon F G, Samijn, Johnny P A, Vermeij, Frederique H, Kuks, Jan B M, van Dijk, Gert W, Wirtz, Paul W, van der Kooi, Anneke J, Garssen, Marcel P J, Gijsbers, Cees J, de Rijk, Maarten C, Visser, Leo H, Blom, Roderik J, Linssen, Wim H J P, van der Kooi, Elly L, Verschuuren, Jan J G M, van Koningsveld, Rinske, Dieks, Rita J G, Gilhuis, H Job, van der Ree, Taco C, Bienfait, Henriette M E, Faber, Catharina G, van Engelen, Baziel G M, Groen, Rutger J, Merkies, Ingemar S J, van Oosten, Bob W, van der Pol, W Ludo, van der Meulen, Willem D M, Badrising, Umesh A, Breukelman, Albert-Jan J, Zwetsloot, Casper P, van der Graaff, Maaike M, Hughes, Richard A C, Cornblath, David R, and van Doorn, Pieter A

Published
2021
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3. Guillain-Barré syndrome during the Zika virus outbreak in Northeast Brazil: An observational cohort study

Author

Leonhard, Sonja E., Halstead, Susan, Lant, Suzannah B., Militão de Albuquerque, Maria de Fatima Pessoa, de Brito, Carlos Alexandre Antunes, de Albuquerque, Lívia Brito Bezerra, Ellul, Mark A., de Oliveira França, Rafael Freitas, Gourlay, Dawn, Griffiths, Michael J., de Miranda Henriques-Souza, Adélia Maria, de Morais Machado, Maria Í., Medialdea-Carrera, Raquel, Mehta, Ravi, da Paz Melo, Roberta, Mesquita, Solange D., Moreira, Álvaro J.P., Pena, Lindomar J., Santos, Marcela Lopes, Turtle, Lance, Solomon, Tom, Willison, Hugh J., Jacobs, Bart C., and Brito Ferreira, Maria L.

Published
2021
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4. Neurological disease in adults with Zika and chikungunya virus infection in Northeast Brazil: a prospective observational study

Author

Brito Ferreira, Maria Lúcia, Militão de Albuquerque, Maria de Fatima Pessoa, de Brito, Carlos Alexandre Antunes, de Oliveira França, Rafael Freitas, Porto Moreira, Álvaro José, de Morais Machado, Maria Íris, da Paz Melo, Roberta, Medialdea-Carrera, Raquel, Dornelas Mesquita, Solange, Lopes Santos, Marcela, Mehta, Ravi, Ramos e Silva, Rafael, Leonhard, Sonja E, Ellul, Mark, Rosala-Hallas, Anna, Burnside, Girvan, Turtle, Lance, Griffiths, Michael J, Jacobs, Bart C, Bhojak, Maneesh, Willison, Hugh J, Pena, Lindomar José, Pardo, Carlos A, Ximenes, Ricardo A A, Martelli, Celina Maria Turchi, Brown, David W G, Cordeiro, Marli Tenório, Lant, Suzannah, and Solomon, Tom

Published
2020
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5. Predicting Outcome in Guillain-Barré Syndrome: International Validation of the Modified Erasmus GBS Outcome Score

Author

Doets, Alex Y, Lingsma, Hester F, Walgaard, Christa, Islam, Badrul, Papri, Nowshin, Davidson, Amy, Yamagishi, Yuko, Kusunoki, Susumu, Dimachkie, Mazen M, Waheed, Waqar, Kolb, Noah, Islam, Zhahirul, Mohammad, Quazi Deen, Harbo, Thomas, Sindrup, Soren H, Chavada, Govindsinh, Willison, Hugh J, Casasnovas, Carlos, Bateman, Kathleen, Miller, James AL, van den Berg, Bianca, Verboon, Christine, Roodbol, Joyce, Leonhard, Sonja E, Benedetti, Luana, Kuwabara, Satoshi, Van den Bergh, Peter, Monges, Soledad, Marfia, Girolama A, Shahrizaila, Nortina, Galassi, Giuliana, Péréon, Yann, Bürmann, Jan, Kuitwaard, Krista, Kleyweg, Ruud P, Marchesoni, Cintia, Sedano Tous, María J, Querol, Luis, Illa, Isabel, Wang, Yuzhong, Nobile-Orazio, Eduardo, Rinaldi, Simon, Schenone, Angelo, Pardo, Julio, Vermeij, Frederique H, Lehmann, Helmar C, Granit, Volkan, Cavaletti, Guido, Gutiérrez-Gutiérrez, Gerardo, Barroso, Fabio A, Visser, Leo H, Katzberg, Hans D, Dardiotis, Efthimios, Attarian, Shahram, van der Kooi, Anneke J, Eftimov, Filip, Wirtz, Paul W, Samijn, Johnny PA, Gilhuis, H Jacobus, Hadden, Robert DM, Holt, James KL, Sheikh, Kazim A, Karafiath, Summer, Vytopil, Michal, Antonini, Giovanni, Feasby, Thomas E, Faber, Catharina G, Gijsbers, Cees J, Busby, Mark, Roberts, Rhys C, Silvestri, Nicholas J, Fazio, Raffaella, van Dijk, Gert W, Garssen, Marcel PJ, Straathof, Chiara SM, Gorson, Kenneth C, and Jacobs, Bart C

Published
2021
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6. Diagnosis and management of Guillain–Barré syndrome in ten steps

Author

Leonhard, Sonja E., Mandarakas, Melissa R., Gondim, Francisco A. A., Bateman, Kathleen, Ferreira, Maria L. B., Cornblath, David R., van Doorn, Pieter A., Dourado, Mario E., Hughes, Richard A. C., Islam, Badrul, Kusunoki, Susumu, Pardo, Carlos A., Reisin, Ricardo, Sejvar, James J., Shahrizaila, Nortina, Soares, Cristiane, Umapathi, Thirugnanam, Wang, Yuzhong, Yiu, Eppie M., Willison, Hugh J., and Jacobs, Bart C.

Published
2019
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7. CSF Findings in Relation to Clinical Characteristics, Subtype, and Disease Course in Patients With Guillain-Barré Syndrome

Author

Al-Hakem, Helle, Doets, Alex Y, Stino, Amro Maher, Zivkovic, Sasha A, Andersen, Henning, Willison, Hugh J, Cornblath, David R, Gorson, Kenneth C, Islam, Zhahirul, Mohammad, Quazi Deen, Sindrup, Søren Hein, Kusunoki, Susumu, Davidson, Amy, Casasnovas, Carlos, Bateman, Kathleen, Miller, James A L, van den Berg, Bianca, Verboon, Christine, Roodbol, Joyce, Leonhard, Sonja E, Arends, Samuel, Luijten, Linda W G, Benedetti, Luana, Kuwabara, Satoshi, Van den Bergh, Peter, Monges, Soledad, Marfia, Girolama A, Shahrizaila, Nortina, Galassi, Giuliana, Pereon, Yann, Bürmann, Jan, Kuitwaard, Krista, Kleyweg, Ruud P, Marchesoni, Cintia, Sedano Tous, María J, Querol, Luis, Martín-Aguilar, Lorena, Wang, Yuzhong, Nobile-Orazio, Eduardo, Rinaldi, Simon, Schenone, Angelo, Pardo, Julio, Vermeij, Frederique H, Waheed, Waqar, Lehmann, Helmar C, Granit, Volkan, Stein, Beth, Samijn, Johnny P A, van Dijk, Gert W, Jacobs, Bart C, Neurology, Medical Microbiology & Infectious Diseases, Public Health, and Immunology

Subjects
Neurology (clinical)
Abstract

BACKGROUND AND OBJECTIVES: To investigate CSF findings in relation to clinical and electrodiagnostic subtypes, severity, and outcome of Guillain-Barré syndrome (GBS) based on 1,500 patients in the International GBS Outcome Study. METHODS: Albuminocytologic dissociation (ACD) was defined as an increased protein level (>0.45 g/L) in the absence of elevated white cell count (4 days 84%. High CSF protein levels were associated with a demyelinating subtype, proximal or global muscle weakness, and a reduced likelihood of being able to run at week 2 (odds ratio [OR] 0.42, 95% CI 0.25-0.70; p = 0.001) and week 4 (OR 0.44, 95% CI 0.27-0.72; p = 0.001). Patients with the Miller Fisher syndrome, distal predominant weakness, and normal or equivocal nerve conduction studies were more likely to have lower CSF protein levels. CSF cell count was

Published
2023

9. Cerebrospinal Fluid Findings in Relation to Clinical Characteristics, Subtype, and Disease Course in Patients With Guillain-Barré Syndrome

Author

Al-Hakem, Helle, primary, Doets, Alex Y, additional, Stino, Amro Maher, additional, Zivkovic, Sasha A., additional, Andersen, Henning, additional, Willison, Hugh J, additional, Cornblath, David R, additional, Gorson, Kenneth C, additional, Islam, Zhahirul, additional, Mohammad, Quazi Deen, additional, Sindrup, Søren Hein, additional, Kusunoki, Susumu, additional, Davidson, Amy, additional, Casasnovas, Carlos, additional, Bateman, Kathleen, additional, Miller, James AL, additional, van den Berg, Bianca, additional, Verboon, Christine, additional, Roodbol, Joyce, additional, Leonhard, Sonja E, additional, Arends, Samuel, additional, Luijten, Linda W G, additional, Benedetti, Luana, additional, Kuwabara, Satoshi, additional, Van den Bergh, Peter, additional, Monges, Soledad, additional, Marfia, Girolama A, additional, Shahrizaila, Nortina, additional, Galassi, Giuliana, additional, Pereon, Yann, additional, Bürmann, Jan, additional, Kuitwaard, Krista, additional, Kleyweg, Ruud P, additional, Marchesoni, Cintia, additional, Sedano Tous, María J, additional, Querol, Luis, additional, Martín-Aguilar, Lorena, additional, Wang, Yuzhong, additional, Nobile-Orazio, Eduardo, additional, Rinaldi, Simon, additional, Schenone, Angelo, additional, Pardo, Julio, additional, Vermeij, Frederique H, additional, Waheed, Waqar, additional, Lehmann, Helmar C, additional, Granit, Volkan, additional, Stein, Beth, additional, Cavaletti, Guido, additional, Gutiérrez-Gutiérrez, Gerardo, additional, Barroso, Fabio A, additional, Visser, Leo H, additional, Katzberg, Hans D, additional, Dardiotis, Efthimios, additional, Attarian, Shahram, additional, van der Kooi, Anneke J, additional, Eftimov, Filip, additional, Wirtz, Paul W, additional, PA Samijn, Johnny, additional, Gilhuis, H Jacobus, additional, DM Hadden, Robert, additional, Holt, James KL, additional, Sheikh, Kazim A, additional, Kolb, Noah, additional, Karafiath, Summer, additional, Vytopil, Michal, additional, Antonini, Giovanni, additional, Feasby, Thomas E, additional, Faber, Catharina, additional, Kramers, Hans, additional, Busby, Mark, additional, Roberts, Rhys C, additional, Silvestri, Nicholas J, additional, Fazio, Raffaella, additional, van Dijk, Gert W, additional, Garssen, Marcel PJ, additional, Verschuuren, Jan, additional, Harbo, Thomas, additional, and Jacobs, Bart C, additional

Published
2023
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12. Guillain-Barré syndrome following SARS-CoV-2 vaccination in the UK: a prospective surveillance study

Author

Tamborska, Arina A, Singh, Bhagteshwar, Leonhard, Sonja E, Hodel, Eva Maria, Stowe, Julia, Watson-Fargie, Taylor, Fernandes, Peter M, Themistocleous, Andreas C, Roelofs, Jacob, Brennan, Kathryn, Morrice, Caroline, Michael, Benedict D, Jacobs, Bart C, McDonald, Helen, Solomon, Tom, and UK Covid Vaccine GBS Study Group

Abstract

Objective: To investigate features of Guillain-Barré syndrome (GBS) following SARS-CoV-2 vaccines and evaluate for a causal link between the two. Methods: We captured cases of GBS after SARS-CoV-2 vaccination through a national, open-access, online surveillance system. For each case, the certainty of GBS was graded using the Brighton criteria, and the relationship to the vaccine was examined using modified WHO Causality Assessment criteria. We compared age distribution of cases with that of prepandemic GBS cases and clinical features with the International GBS Outcome Study (IGOS). Results: Between 1 January and 30 June 2021, we received 67 reports of GBS following the ChAdOx1 vaccine (65 first doses) and three reports following the BNT162b2 vaccine (all first doses). The causal association with the vaccine was classified as probable for 56 (80%, all ChAdOx1), possible for 12 (17%, 10 ChAdOx1) and unlikely for two (3%, 1 ChAdOx1). A greater proportion of cases occurred in the 50-59 age group in comparison with prepandemic GBS. Most common clinical variants were sensorimotor GBS (n=55; 79%) and facial diplegia with paraesthesias (n=10; 14%). 10% (n=7/69) of patients reported an antecedent infection, compared with 77% (n=502/652) of the IGOS cohort (p

Published
2022

13. An International Perspective on Preceding Infections in Guillain-Barre Syndrome The IGOS-1000 Cohort

Author

Leonhard, Sonja E., van der Eijk, Annemiek A., Andersen, Henning, Antonini, Giovanni, Arends, Samuel, Attarian, Shahram, Barroso, Fabio A., Bateman, Kathleen J., Batstra, Manou R., Benedetti, Luana, van den Berg, Bianca, Van den Bergh, Peter, Burmann, Jan, Busby, Mark, Casasnovas, Carlos, Cornblath, David R., Davidson, Amy, Doets, Alex Y., van Doorn, Pieter A., de la Cour, Charlotte Dornonville, Feasby, Thomas E., Fehmi, Janev, Garcia-Sobrino, Tania, Goldstein, Jonathan M., Gorson, Kenneth C., Granit, Volkan, Hadden, Robert D. M., Harbo, Thomas, Hartung, Hans-Peter, Hasan, Imran, Holbech, Jakob, V, Holt, James K. L., Jahan, Israt, Islam, Zhahirul, Karafiath, Summer, Katzberg, Hans D., Kleyweg, Ruud P., Kolb, Noah, Kuitwaard, Krista, Kuwahara, Motoi, Kusunoki, Susumu, Luijten, Linda W. G., Kuwabara, Satoshi, Pan, Edward Lee, Lehmann, Helmar C., Maas, Marijke, Martin-Aguilar, Lorena, Al Miller, James, Mohammad, Quazi Deen, Monges, Soledad, Nedkova-Hristova, Velina, Nobile-Orazio, Eduardo, Pardo, Julio, Pereon, Yann, Querol, Luis, Reisin, Ricardo, Van Rijs, Wouter, Rinaldi, Simon, Roberts, Rhys C., Roodbol, Joyce, Shahrizaila, Nortina, Sindrup, Soren Hein, Stein, Beth, Cheng-Yin, Tan, Tankisi, Hatice, Tio-Gillen, Anne P., Tous, Maria J. Sedano, Verboon, Christine, Vermeij, Frederique H., Visser, Leo H., Huizinga, Ruth, Willison, Hugh J., Jacobs, Bart C., Leonhard, Sonja E., van der Eijk, Annemiek A., Andersen, Henning, Antonini, Giovanni, Arends, Samuel, Attarian, Shahram, Barroso, Fabio A., Bateman, Kathleen J., Batstra, Manou R., Benedetti, Luana, van den Berg, Bianca, Van den Bergh, Peter, Burmann, Jan, Busby, Mark, Casasnovas, Carlos, Cornblath, David R., Davidson, Amy, Doets, Alex Y., van Doorn, Pieter A., de la Cour, Charlotte Dornonville, Feasby, Thomas E., Fehmi, Janev, Garcia-Sobrino, Tania, Goldstein, Jonathan M., Gorson, Kenneth C., Granit, Volkan, Hadden, Robert D. M., Harbo, Thomas, Hartung, Hans-Peter, Hasan, Imran, Holbech, Jakob, V, Holt, James K. L., Jahan, Israt, Islam, Zhahirul, Karafiath, Summer, Katzberg, Hans D., Kleyweg, Ruud P., Kolb, Noah, Kuitwaard, Krista, Kuwahara, Motoi, Kusunoki, Susumu, Luijten, Linda W. G., Kuwabara, Satoshi, Pan, Edward Lee, Lehmann, Helmar C., Maas, Marijke, Martin-Aguilar, Lorena, Al Miller, James, Mohammad, Quazi Deen, Monges, Soledad, Nedkova-Hristova, Velina, Nobile-Orazio, Eduardo, Pardo, Julio, Pereon, Yann, Querol, Luis, Reisin, Ricardo, Van Rijs, Wouter, Rinaldi, Simon, Roberts, Rhys C., Roodbol, Joyce, Shahrizaila, Nortina, Sindrup, Soren Hein, Stein, Beth, Cheng-Yin, Tan, Tankisi, Hatice, Tio-Gillen, Anne P., Tous, Maria J. Sedano, Verboon, Christine, Vermeij, Frederique H., Visser, Leo H., Huizinga, Ruth, Willison, Hugh J., and Jacobs, Bart C.

Abstract

Background and Objectives Infections play a key role in the development of Guillain-Barre syndrome (GBS) and have been associated with specific clinical features and disease severity. The clinical variation of GBS across geographical regions has been suggested to be related to differences in the distribution of preceding infections, but this has not been studied on a large scale. Methods We analyzed the first 1,000 patients included in the International GBS Outcome Study with available biosamples (n = 768) for the presence of a recent infection with Campylobacter jejuni, hepatitis E virus, Mycoplasma pneumoniae, cytomegalovirus, and Epstein-Barr virus. Results Serologic evidence of a recent infection with C. jejuni was found in 228 (30%), M. pneumoniae in 77 (10%), hepatitis E virus in 23 (3%), cytomegalovirus in 30 (4%), and Epstein-Barr virus in 7 (1%) patients. Evidence of more than 1 recent infection was found in 49 (6%) of these patients. Symptoms of antecedent infections were reported in 556 patients (72%), and this proportion did not significantly differ between those testing positive or negative for a recent infection. The proportions of infections were similar across continents. The sensorimotor variant and the demyelinating electrophysiologic subtype were most frequent across all infection groups, although proportions were significantly higher in patients with a cytomegalovirus and significantly lower in those with a C. jejuni infection. C. jejuni-positive patients were more severely affected, indicated by a lower Medical Research Council sum score at nadir (p = 0.004) and a longer time to regain the ability to walk independently (p = 0.005). The pure motor variant and axonal electrophysiologic subtype were more frequent in Asian compared with American or European C. jejuni-positive patients (p < 0.001, resp. p = 0.001). Time to nadir was longer in the cytomegalovirus-positive patients (p = 0.004). Discussion Across geographical regions, the distribution o

Published
2022

14. Predicting Outcome in Guillain-Barre Syndrome International Validation of the Modified Erasmus GBS Outcome Score

Author

Doets, Alex Y., Lingsma, Hester F., Walgaard, Christa, Islam, Badrul, Papri, Nowshin, Davidson, Amy, Kusunoki, Susumu, Dimachkie, Mazen M., Waheed, Waqar, Kolb, Noah, Islam, Zhahirul, Mohammad, Quazi Deen, Harbo, Thomas, Sindrup, Soren H., Chavada, Govindsinh, Willison, Hugh J., Casasnovas, Carlos, Bateman, Kathleen, Miller, James A. L., van den Berg, Bianca, Verboon, Christine, Roodbol, Joyce, Leonhard, Sonja E., Benedetti, Luana, Kuwabara, Satoshi, Van den Bergh, Peter, Monges, Soledad, Marfia, Girolama A., Shahrizaila, Nortina, Galassi, Giuliana, Pereon, Yann, Burmann, Jan, Kuitwaard, Krista, Kleyweg, Ruud P., Marchesoni, Cintia, Tous, Maria J. Sedano, Querol, Luis, Illa, Isabel, Wang, Yuzhong, Nobile-Orazio, Eduardo, Rinaldi, Simon, Schenone, Angelo, Pardo, Julio, Vermeij, Frederique H., Lehmann, Helmar C., Granit, Volkan, Cavaletti, Guido, Gutierrez-Gutierrez, Gerardo, Barroso, Fabio A., Visser, Leo H., Katzberg, Hans D., Dardiotis, Efthimios, Attarian, Shahram, van der Kooi, Anneke J., Eftimov, Filip, Wirtz, Paul W., Samijn, Johnny P. A., Gilhuis, H. Jacobus, Hadden, Robert D. M., Holt, James K. L., Sheikh, Kazim A., Karafiath, Summer, Vytopil, Michal, Antonini, Giovanni, Feasby, Thomas E., Faber, Catharina G., Gijsbers, Cees J., Busby, Mark, Roberts, Rhys C., Silvestri, Nicholas J., Fazio, Raffaella, van Dijk, Gert W., Garssen, Marcel P. J., Straathof, Chiara S. M., Gorson, Kenneth C., Jacobs, Bart C., Doets, Alex Y., Lingsma, Hester F., Walgaard, Christa, Islam, Badrul, Papri, Nowshin, Davidson, Amy, Kusunoki, Susumu, Dimachkie, Mazen M., Waheed, Waqar, Kolb, Noah, Islam, Zhahirul, Mohammad, Quazi Deen, Harbo, Thomas, Sindrup, Soren H., Chavada, Govindsinh, Willison, Hugh J., Casasnovas, Carlos, Bateman, Kathleen, Miller, James A. L., van den Berg, Bianca, Verboon, Christine, Roodbol, Joyce, Leonhard, Sonja E., Benedetti, Luana, Kuwabara, Satoshi, Van den Bergh, Peter, Monges, Soledad, Marfia, Girolama A., Shahrizaila, Nortina, Galassi, Giuliana, Pereon, Yann, Burmann, Jan, Kuitwaard, Krista, Kleyweg, Ruud P., Marchesoni, Cintia, Tous, Maria J. Sedano, Querol, Luis, Illa, Isabel, Wang, Yuzhong, Nobile-Orazio, Eduardo, Rinaldi, Simon, Schenone, Angelo, Pardo, Julio, Vermeij, Frederique H., Lehmann, Helmar C., Granit, Volkan, Cavaletti, Guido, Gutierrez-Gutierrez, Gerardo, Barroso, Fabio A., Visser, Leo H., Katzberg, Hans D., Dardiotis, Efthimios, Attarian, Shahram, van der Kooi, Anneke J., Eftimov, Filip, Wirtz, Paul W., Samijn, Johnny P. A., Gilhuis, H. Jacobus, Hadden, Robert D. M., Holt, James K. L., Sheikh, Kazim A., Karafiath, Summer, Vytopil, Michal, Antonini, Giovanni, Feasby, Thomas E., Faber, Catharina G., Gijsbers, Cees J., Busby, Mark, Roberts, Rhys C., Silvestri, Nicholas J., Fazio, Raffaella, van Dijk, Gert W., Garssen, Marcel P. J., Straathof, Chiara S. M., Gorson, Kenneth C., and Jacobs, Bart C.

Abstract

Background and Objectives The clinical course and outcome of the Guillain-Barre syndrome (GBS) are diverse and vary among regions. The modified Erasmus GBS Outcome Score (mEGOS), developed with data from Dutch patients, is a clinical model that predicts the risk of walking inability in patients with GBS. The study objective was to validate the mEGOS in the International GBS Outcome Study (IGOS) cohort and to improve its performance and region specificity. Methods We used prospective data from the first 1,500 patients included in IGOS, aged >= 6 years and unable to walk independently. We evaluated whether the mEGOS at entry and week 1 could predict the inability to walk unaided at 4 and 26 weeks in the full cohort and in regional subgroups, using 2 measures for model performance: (1) discrimination: area under the receiver operating characteristic curve (AUC) and (2) calibration: observed vs predicted probability of being unable to walk independently. To improve the model predictions, we recalibrated the model containing the overall mEGOS score, without changing the individual predictive factors. Finally, we assessed the predictive ability of the individual factors. Results For validation of mEGOS at entry, 809 patients were eligible (Europe/North America [n = 677], Asia [n = 76], other [n = 56]), and 671 for validation of mEGOS at week 1 (Europe/North America [n = 563], Asia [n = 65], other [n = 43]). AUC values were >0.7 in all regional subgroups. In the Europe/North America subgroup, observed outcomes were worse than predicted; in Asia, observed outcomes were better than predicted. Recalibration improved model accuracy and enabled the development of a region-specific version for Europe/North America (mEGOS-Eu/NA). Similar to the original mEGOS, severe limb weakness and higher age were the predominant predictors of poor outcome in the IGOS cohort. Discussion mEGOS is a validated tool to predict the inability to walk unaided at 4 and 26 weeks in patients with GBS, a

Published
2022

15. Guillain-Barré syndrome following SARS-CoV-2 vaccination in the UK:A prospective surveillance study

Author

Tamborska, Arina A., Singh, Bhagteshwar, Leonhard, Sonja E., Hodel, Eva Maria, Stowe, Julia, Watson-Fargie, Taylor, Fernandes, Peter M., Themistocleous, Andreas C., Roelofs, Jacob, Brennan, Kathryn, Morrice, Caroline, Michael, Benedict D., Jacobs, Bart C., McDonald, Helen, Solomon, Tom, Tamborska, Arina A., Singh, Bhagteshwar, Leonhard, Sonja E., Hodel, Eva Maria, Stowe, Julia, Watson-Fargie, Taylor, Fernandes, Peter M., Themistocleous, Andreas C., Roelofs, Jacob, Brennan, Kathryn, Morrice, Caroline, Michael, Benedict D., Jacobs, Bart C., McDonald, Helen, and Solomon, Tom

Abstract

Objective To investigate features of Guillain-Barré syndrome (GBS) following SARS-CoV-2 vaccines and evaluate for a causal link between the two. Methods We captured cases of GBS after SARS-CoV-2 vaccination through a national, open-access, online surveillance system. For each case, the certainty of GBS was graded using the Brighton criteria, and the relationship to the vaccine was examined using modified WHO Causality Assessment criteria. We compared age distribution of cases with that of prepandemic GBS cases and clinical features with the International GBS Outcome Study (IGOS). Results Between 1 January and 30 June 2021, we received 67 reports of GBS following the ChAdOx1 vaccine (65 first doses) and three reports following the BNT162b2 vaccine (all first doses). The causal association with the vaccine was classified as probable for 56 (80%, all ChAdOx1), possible for 12 (17%, 10 ChAdOx1) and unlikely for two (3%, 1 ChAdOx1). A greater proportion of cases occurred in the 50-59 age group in comparison with prepandemic GBS. Most common clinical variants were sensorimotor GBS (n=55; 79%) and facial diplegia with paraesthesias (n=10; 14%). 10% (n=7/69) of patients reported an antecedent infection, compared with 77% (n=502/652) of the IGOS cohort (p<0.00001). Facial weakness (63% (n=44/70) vs 36% (n=220/620); p<0.00001) and sensory dysfunction (93% (n=63/68) vs 69% (n=408/588); p=0.00005) were more common but disease severity and outcomes were similar to the IGOS study. Interpretation Most reports of GBS followed the first dose of ChAdOx1 vaccine. While our study cannot confirm or refute causation, this observation, together with the absence of alternative aetiologies, different than expected age distribution and the presence of unusual clinical features support a causal link. Clinicians and surveillance bodies should remain vigilant to the possibility of this very rare adverse event and its atypical variants.

Published
2022

16. Predicting Outcome in Guillain-Barré Syndrome: International Validation of the Modified Erasmus GBS Outcome Score

Author

Doets, A, Lingsma, H, Walgaard, C, Islam, B, Papri, N, Davidson, A, Yamagishi, Y, Kusunoki, S, Dimachkie, M, Waheed, W, Kolb, N, Islam, Z, Mohammad, Q, Harbo, T, Sindrup, S, Chavada, G, Willison, H, Casasnovas, C, Bateman, K, Miller, J, van den Berg, B, Verboon, C, Roodbol, J, Leonhard, S, Benedetti, L, Kuwabara, S, Van den Bergh, P, Monges, S, Marfia, G, Shahrizaila, N, Galassi, G, Péréon, Y, Bürmann, J, Kuitwaard, K, Kleyweg, R, Marchesoni, C, Sedano Tous, M, Querol, L, Illa, I, Wang, Y, Nobile-Orazio, E, Rinaldi, S, Schenone, A, Pardo, J, Vermeij, F, Lehmann, H, Granit, V, Cavaletti, G, Gutiérrez-Gutiérrez, G, Barroso, F, Visser, L, Katzberg, H, Dardiotis, E, Attarian, S, van der Kooi, A, Eftimov, F, Wirtz, P, Samijn, J, Gilhuis, H, Hadden, R, Holt, J, Sheikh, K, Karafiath, S, Vytopil, M, Antonini, G, Feasby, T, Faber, C, Gijsbers, C, Busby, M, Roberts, R, Silvestri, N, Fazio, R, van Dijk, G, Garssen, M, Straathof, C, Gorson, K, Jacobs, B, Doets, Alex Y, Lingsma, Hester F, Walgaard, Christa, Islam, Badrul, Papri, Nowshin, Davidson, Amy, Yamagishi, Yuko, Kusunoki, Susumu, Dimachkie, Mazen M, Waheed, Waqar, Kolb, Noah, Islam, Zhahirul, Mohammad, Quazi Deen, Harbo, Thomas, Sindrup, Soren H, Chavada, Govindsinh, Willison, Hugh J, Casasnovas, Carlos, Bateman, Kathleen, Miller, James A L, van den Berg, Bianca, Verboon, Christine, Roodbol, Joyce, Leonhard, Sonja E, Benedetti, Luana, Kuwabara, Satoshi, Van den Bergh, Peter, Monges, Soledad, Marfia, Girolama A, Shahrizaila, Nortina, Galassi, Giuliana, Péréon, Yann, Bürmann, Jan, Kuitwaard, Krista, Kleyweg, Ruud P, Marchesoni, Cintia, Sedano Tous, María J, Querol, Luis, Illa, Isabel, Wang, Yuzhong, Nobile-Orazio, Eduardo, Rinaldi, Simon, Schenone, Angelo, Pardo, Julio, Vermeij, Frederique H, Lehmann, Helmar C, Granit, Volkan, Cavaletti, Guido, Gutiérrez-Gutiérrez, Gerardo, Barroso, Fabio A, Visser, Leo H, Katzberg, Hans D, Dardiotis, Efthimios, Attarian, Shahram, van der Kooi, Anneke J, Eftimov, Filip, Wirtz, Paul W, Samijn, Johnny P A, Gilhuis, H Jacobus, Hadden, Robert D M, Holt, James K L, Sheikh, Kazim A, Karafiath, Summer, Vytopil, Michal, Antonini, Giovanni, Feasby, Thomas E, Faber, Catharina G, Gijsbers, Cees J, Busby, Mark, Roberts, Rhys C, Silvestri, Nicholas J, Fazio, Raffaella, van Dijk, Gert W, Garssen, Marcel P J, Straathof, Chiara S M, Gorson, Kenneth C, Jacobs, Bart C, Doets, A, Lingsma, H, Walgaard, C, Islam, B, Papri, N, Davidson, A, Yamagishi, Y, Kusunoki, S, Dimachkie, M, Waheed, W, Kolb, N, Islam, Z, Mohammad, Q, Harbo, T, Sindrup, S, Chavada, G, Willison, H, Casasnovas, C, Bateman, K, Miller, J, van den Berg, B, Verboon, C, Roodbol, J, Leonhard, S, Benedetti, L, Kuwabara, S, Van den Bergh, P, Monges, S, Marfia, G, Shahrizaila, N, Galassi, G, Péréon, Y, Bürmann, J, Kuitwaard, K, Kleyweg, R, Marchesoni, C, Sedano Tous, M, Querol, L, Illa, I, Wang, Y, Nobile-Orazio, E, Rinaldi, S, Schenone, A, Pardo, J, Vermeij, F, Lehmann, H, Granit, V, Cavaletti, G, Gutiérrez-Gutiérrez, G, Barroso, F, Visser, L, Katzberg, H, Dardiotis, E, Attarian, S, van der Kooi, A, Eftimov, F, Wirtz, P, Samijn, J, Gilhuis, H, Hadden, R, Holt, J, Sheikh, K, Karafiath, S, Vytopil, M, Antonini, G, Feasby, T, Faber, C, Gijsbers, C, Busby, M, Roberts, R, Silvestri, N, Fazio, R, van Dijk, G, Garssen, M, Straathof, C, Gorson, K, Jacobs, B, Doets, Alex Y, Lingsma, Hester F, Walgaard, Christa, Islam, Badrul, Papri, Nowshin, Davidson, Amy, Yamagishi, Yuko, Kusunoki, Susumu, Dimachkie, Mazen M, Waheed, Waqar, Kolb, Noah, Islam, Zhahirul, Mohammad, Quazi Deen, Harbo, Thomas, Sindrup, Soren H, Chavada, Govindsinh, Willison, Hugh J, Casasnovas, Carlos, Bateman, Kathleen, Miller, James A L, van den Berg, Bianca, Verboon, Christine, Roodbol, Joyce, Leonhard, Sonja E, Benedetti, Luana, Kuwabara, Satoshi, Van den Bergh, Peter, Monges, Soledad, Marfia, Girolama A, Shahrizaila, Nortina, Galassi, Giuliana, Péréon, Yann, Bürmann, Jan, Kuitwaard, Krista, Kleyweg, Ruud P, Marchesoni, Cintia, Sedano Tous, María J, Querol, Luis, Illa, Isabel, Wang, Yuzhong, Nobile-Orazio, Eduardo, Rinaldi, Simon, Schenone, Angelo, Pardo, Julio, Vermeij, Frederique H, Lehmann, Helmar C, Granit, Volkan, Cavaletti, Guido, Gutiérrez-Gutiérrez, Gerardo, Barroso, Fabio A, Visser, Leo H, Katzberg, Hans D, Dardiotis, Efthimios, Attarian, Shahram, van der Kooi, Anneke J, Eftimov, Filip, Wirtz, Paul W, Samijn, Johnny P A, Gilhuis, H Jacobus, Hadden, Robert D M, Holt, James K L, Sheikh, Kazim A, Karafiath, Summer, Vytopil, Michal, Antonini, Giovanni, Feasby, Thomas E, Faber, Catharina G, Gijsbers, Cees J, Busby, Mark, Roberts, Rhys C, Silvestri, Nicholas J, Fazio, Raffaella, van Dijk, Gert W, Garssen, Marcel P J, Straathof, Chiara S M, Gorson, Kenneth C, and Jacobs, Bart C

Abstract

Background and objectives: The clinical course and outcome of the Guillain-Barré syndrome (GBS) are diverse and vary among regions. The modified Erasmus GBS Outcome Score (mEGOS), developed with data from Dutch patients, is a clinical model that predicts the risk of walking inability in patients with GBS. The study objective was to validate the mEGOS in the International GBS Outcome Study (IGOS) cohort and to improve its performance and region specificity. Methods: We used prospective data from the first 1,500 patients included in IGOS, aged ≥6 years and unable to walk independently. We evaluated whether the mEGOS at entry and week 1 could predict the inability to walk unaided at 4 and 26 weeks in the full cohort and in regional subgroups, using 2 measures for model performance: (1) discrimination: area under the receiver operating characteristic curve (AUC) and (2) calibration: observed vs predicted probability of being unable to walk independently. To improve the model predictions, we recalibrated the model containing the overall mEGOS score, without changing the individual predictive factors. Finally, we assessed the predictive ability of the individual factors. Results: For validation of mEGOS at entry, 809 patients were eligible (Europe/North America [n = 677], Asia [n = 76], other [n = 56]), and 671 for validation of mEGOS at week 1 (Europe/North America [n = 563], Asia [n = 65], other [n = 43]). AUC values were >0.7 in all regional subgroups. In the Europe/North America subgroup, observed outcomes were worse than predicted; in Asia, observed outcomes were better than predicted. Recalibration improved model accuracy and enabled the development of a region-specific version for Europe/North America (mEGOS-Eu/NA). Similar to the original mEGOS, severe limb weakness and higher age were the predominant predictors of poor outcome in the IGOS cohort. Discussion: mEGOS is a validated tool to predict the inability to walk unaided at 4 and 26 weeks in patients with

Published
2022

17. Guía basada en la evidencia. Diagnóstico y manejo del síndrome de Guillain-Barré en diez pasos = Evidence based guidelines. Diagnosis and management of Guillain-Barré syndrome in ten steps

Author

Leonhard, Sonja E., Mandarakas, Melissa R., de Assis Aquino Gondim, Francisco, Bateman, Kathleen, Brito Ferreira, Maria L., Cornblath, David R., Van Doorn, Pieter A., Dourado, Mario E., Hughes, Richard A.C., Islam, Badrul, Kusunoki, Susumu, Pardo, Carlos A., Reisin, Ricardo, Sejvar, James J., Shahrizaila, Nortina, Soares, Cristiane, Umapathi, Thirugnanam, Wang, Yuzhong, Yiu, Eppie M., Willison, Hugh J., and Jacobs, Bart C.

Abstract

Guillain-Barré syndrome (GBS) is a rare, but potentially fatal, immune-mediated disease of the peripheral nerves and nerve roots that is usually triggered by infections. The incidence of GBS can therefore increase during outbreaks of infectious diseases, as was seen during the Zika virus epidemics in 2013 in French Polynesia and in 2015 in Latin America. Diagnosis and management of GBS can be complicated as its clinical presentation and disease course are heterogeneous, and no international clinical guidelines are currently available. To support clinicians, especially in the context of an outbreak, we have developed a globally applicable guideline for the diagnosis and management of GBS. The guideline is based on current literature and expert consensus, and has a ten-step structure to facilitate its use in clinical practice. We first provide an introduction to the diagnostic criteria, clinical variants and differential diagnoses of GBS. The ten steps then cover early recognition and diagnosis of GBS, admission to the intensive care unit, treatment indication and selection, monitoring and treatment of disease progression, prediction of clinical course and outcome, and management of complications and sequelae.

Published
2021

18. Antecedent infections in Guillain‐Barré syndrome in endemic areas of arbovirus transmission: A multinational case‐control study

Author

Leonhard, Sonja E., primary, Tan, Cheng Yin, additional, Eijk, Annemiek A., additional, Reisin, Ricardo R., additional, Franken, Suzanne C., additional, Huizinga, Ruth, additional, Arends, Samuel, additional, Batstra, Manou R., additional, Bezerra Jeronimo, Selma M., additional, Drenthen, Judith, additional, Koning, Laura, additional, Leon Cejas, Luciana, additional, Marchesoni, Cintia, additional, Marques, Wilson, additional, Shahrizaila, Nortina, additional, Casas, Dardo F., additional, Sotelo, Andrea, additional, Tillard, Belen, additional, Dourado, Mario‐Emilio, additional, and Jacobs, Bart C., additional

Published
2021
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19. Guillain-Barr�� syndrome after SARS-CoV-2 infection in an international prospective cohort study

Author

Luijten, Linda W G, Leonhard, Sonja E, van der Eijk, Annemiek A, Doets, Alex Y, Appeltshauser, Luise, Arends, Samuel, Attarian, Shahram, Benedetti, Luana, Briani, Chiara, Casasnovas, Carlos, Castellani, Francesca, Dardiotis, Efthimios, Echaniz-Laguna, Andoni, Garssen, Marcel P J, Harbo, Thomas, Huizinga, Ruth, Humm, Andrea M, Jellema, Korn��, van der Kooi, Anneke J, Kuitwaard, Krista, Kuntzer, Thierry, Kusunoki, Susumu, Lascano, Agustina M, Martinez-Hernandez, Eugenia, Rinaldi, Simon, Samijn, Johnny P A, Scheidegger, Olivier, Tsouni, Pinelopi, Vicino, Alex, Visser, Leo H, Walgaard, Christa, Wang, Yuzhong, Wirtz, Paul W, Ripellino, Paolo, and Jacobs, Bart C

Subjects
610 Medicine & health
Abstract

In the wake of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic, an increasing number of patients with neurological disorders, including Guillain-Barr�� syndrome (GBS), have been reported following this infection. It remains unclear, however, if these cases are coincidental or not, as most publications were case reports or small regional retrospective cohort studies. The International GBS Outcome Study is an ongoing prospective observational cohort study enrolling patients with GBS within 2 weeks from onset of weakness. Data from patients included in this study, between 30 January 2020 and 30 May 2020, were used to investigate clinical and laboratory signs of a preceding or concurrent SARS-CoV-2 infection and to describe the associated clinical phenotype and disease course. Patients were classified according to the SARS-CoV-2 case definitions of the European Centre for Disease Prevention and Control and laboratory recommendations of the World Health Organization. Forty-nine patients with GBS were included, of whom eight (16%) had a confirmed and three (6%) a probable SARS-CoV-2 infection. Nine of these 11 patients had no serological evidence of other recent preceding infections associated with GBS, whereas two had serological evidence of a recent Campylobacter jejuni infection. Patients with a confirmed or probable SARS-CoV-2 infection frequently had a sensorimotor variant 8/11 (73%) and facial palsy 7/11 (64%). The eight patients who underwent electrophysiological examination all had a demyelinating subtype, which was more prevalent than the other patients included in the same time window [14/30 (47%), P = 0.012] as well as historical region and age-matched control subjects included in the International GBS Outcome Study before the pandemic [23/44 (52%), P = 0.016]. The median time from the onset of infection to neurological symptoms was 16 days (interquartile range 12-22). Patients with SARS-CoV-2 infection shared uniform neurological features, similar to those previously described in other post-viral GBS patients. The frequency (22%) of a preceding SARS-CoV-2 infection in our study population was higher than estimates of the contemporaneous background prevalence of SARS-CoV-2, which may be a result of recruitment bias during the pandemic, but could also indicate that GBS may rarely follow a recent SARS-CoV-2 infection. Consistent with previous studies, we found no increase in patient recruitment during the pandemic for our ongoing International GBS Outcome Study compared to previous years, making a strong relationship of GBS with SARS-CoV-2 unlikely. A case-control study is required to determine if there is a causative link or not.

Published
2021
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20. Guillain-Barré syndrome after SARS-CoV-2 infection in an international prospective cohort study

Author

Luijten, L, Leonhard, S, van der Eijk, A, Doets, A, Appeltshauser, L, Arends, S, Attarian, S, Benedetti, L, Briani, C, Casasnovas, C, Castellani, F, Dardiotis, E, Echaniz-Laguna, A, Garssen, M, Harbo, T, Huizinga, R, Humm, A, Jellema, K, van der Kooi, A, Kuitwaard, K, Kuntzer, T, Kusunoki, S, Lascano, A, Martinez-Hernandez, E, Rinaldi, S, Samijn, J, Scheidegger, O, Tsouni, P, Vicino, A, Visser, L, Walgaard, C, Wang, Y, Wirtz, P, Ripellino, P, Jacobs, B, Cavaletti, G, Luijten, Linda W G, Leonhard, Sonja E, van der Eijk, Annemiek A, Doets, Alex Y, Appeltshauser, Luise, Arends, Samuel, Attarian, Shahram, Benedetti, Luana, Briani, Chiara, Casasnovas, Carlos, Castellani, Francesca, Dardiotis, Efthimios, Echaniz-Laguna, Andoni, Garssen, Marcel P J, Harbo, Thomas, Huizinga, Ruth, Humm, Andrea M, Jellema, Korné, van der Kooi, Anneke J, Kuitwaard, Krista, Kuntzer, Thierry, Kusunoki, Susumu, Lascano, Agustina M, Martinez-Hernandez, Eugenia, Rinaldi, Simon, Samijn, Johnny P A, Scheidegger, Olivier, Tsouni, Pinelopi, Vicino, Alex, Visser, Leo H, Walgaard, Christa, Wang, Yuzhong, Wirtz, Paul W, Ripellino, Paolo, Jacobs, Bart C, Cavaletti, Guido, Luijten, L, Leonhard, S, van der Eijk, A, Doets, A, Appeltshauser, L, Arends, S, Attarian, S, Benedetti, L, Briani, C, Casasnovas, C, Castellani, F, Dardiotis, E, Echaniz-Laguna, A, Garssen, M, Harbo, T, Huizinga, R, Humm, A, Jellema, K, van der Kooi, A, Kuitwaard, K, Kuntzer, T, Kusunoki, S, Lascano, A, Martinez-Hernandez, E, Rinaldi, S, Samijn, J, Scheidegger, O, Tsouni, P, Vicino, A, Visser, L, Walgaard, C, Wang, Y, Wirtz, P, Ripellino, P, Jacobs, B, Cavaletti, G, Luijten, Linda W G, Leonhard, Sonja E, van der Eijk, Annemiek A, Doets, Alex Y, Appeltshauser, Luise, Arends, Samuel, Attarian, Shahram, Benedetti, Luana, Briani, Chiara, Casasnovas, Carlos, Castellani, Francesca, Dardiotis, Efthimios, Echaniz-Laguna, Andoni, Garssen, Marcel P J, Harbo, Thomas, Huizinga, Ruth, Humm, Andrea M, Jellema, Korné, van der Kooi, Anneke J, Kuitwaard, Krista, Kuntzer, Thierry, Kusunoki, Susumu, Lascano, Agustina M, Martinez-Hernandez, Eugenia, Rinaldi, Simon, Samijn, Johnny P A, Scheidegger, Olivier, Tsouni, Pinelopi, Vicino, Alex, Visser, Leo H, Walgaard, Christa, Wang, Yuzhong, Wirtz, Paul W, Ripellino, Paolo, Jacobs, Bart C, and Cavaletti, Guido

Abstract

In the wake of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic, an increasing number of patients with neurological disorders, including Guillain-Barré syndrome (GBS), have been reported following this infection. It remains unclear, however, if these cases are coincidental or not, as most publications were case reports or small regional retrospective cohort studies. The International GBS Outcome Study is an ongoing prospective observational cohort study enrolling patients with GBS within 2 weeks from onset of weakness. Data from patients included in this study, between 30 January 2020 and 30 May 2020, were used to investigate clinical and laboratory signs of a preceding or concurrent SARS-CoV-2 infection and to describe the associated clinical phenotype and disease course. Patients were classified according to the SARS-CoV-2 case definitions of the European Centre for Disease Prevention and Control and laboratory recommendations of the World Health Organization. Forty-nine patients with GBS were included, of whom eight (16%) had a confirmed and three (6%) a probable SARS-CoV-2 infection. Nine of these 11 patients had no serological evidence of other recent preceding infections associated with GBS, whereas two had serological evidence of a recent Campylobacter jejuni infection. Patients with a confirmed or probable SARS-CoV-2 infection frequently had a sensorimotor variant 8/11 (73%) and facial palsy 7/11 (64%). The eight patients who underwent electrophysiological examination all had a demyelinating subtype, which was more prevalent than the other patients included in the same time window [14/30 (47%), P = 0.012] as well as historical region and age-matched control subjects included in the International GBS Outcome Study before the pandemic [23/44 (52%), P = 0.016]. The median time from the onset of infection to neurological symptoms was 16 days (interquartile range 12-22). Patients with SARS-CoV-2 infection shared uniform neurological feat

Published
2021

21. Guillain-Barré syndrome after SARS-CoV-2 infection in an international prospective cohort study

Author

Luijten, Linda W.G., Leonhard, Sonja E., Van Der Eijk, Annemiek A., Doets, Alex Y., Appeltshauser, Luise, Arends, Samuel, Attarian, Shahram, Benedetti, Luana, Briani, Chiara, Casasnovas, Carlos, Castellani, Francesca, Dardiotis, Efthimios, Echaniz-Laguna, Andoni, Garssen, Marcel P.J., Harbo, Thomas, Huizinga, Ruth, Humm, Andrea M., Jellema, Korné, Van Der Kooi, Anneke J., Kuitwaard, Krista, Kuntzer, Thierry, Kusunoki, Susumu, Lascano, Agustina M., Martinez-Hernandez, Eugenia, Rinaldi, Simon, Samijn, Johnny P.A., Scheidegger, Olivier, Tsouni, Pinelopi, Vicino, Alex, Visser, Leo H., Walgaard, Christa, Wang, Yuzhong, Wirtz, Paul W., Ripellino, Paolo, Jacobs, Bart C., Luijten, Linda W.G., Leonhard, Sonja E., Van Der Eijk, Annemiek A., Doets, Alex Y., Appeltshauser, Luise, Arends, Samuel, Attarian, Shahram, Benedetti, Luana, Briani, Chiara, Casasnovas, Carlos, Castellani, Francesca, Dardiotis, Efthimios, Echaniz-Laguna, Andoni, Garssen, Marcel P.J., Harbo, Thomas, Huizinga, Ruth, Humm, Andrea M., Jellema, Korné, Van Der Kooi, Anneke J., Kuitwaard, Krista, Kuntzer, Thierry, Kusunoki, Susumu, Lascano, Agustina M., Martinez-Hernandez, Eugenia, Rinaldi, Simon, Samijn, Johnny P.A., Scheidegger, Olivier, Tsouni, Pinelopi, Vicino, Alex, Visser, Leo H., Walgaard, Christa, Wang, Yuzhong, Wirtz, Paul W., Ripellino, Paolo, and Jacobs, Bart C.

Abstract

In the wake of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic, an increasing number of patients with neurological disorders, including Guillain-Barré syndrome (GBS), have been reported following this infection. It remains unclear, however, if these cases are coincidental or not, as most publications were case reports or small regional retrospective cohort studies. The International GBS Outcome Study is an ongoing prospective observational cohort study enrolling patients with GBS within 2 weeks from onset of weakness. Data from patients included in this study, between 30 January 2020 and 30 May 2020, were used to investigate clinical and laboratory signs of a preceding or concurrent SARS-CoV-2 infection and to describe the associated clinical phenotype and disease course. Patients were classified according to the SARS-CoV-2 case definitions of the European Centre for Disease Prevention and Control and laboratory recommendations of the World Health Organization. Forty-nine patients with GBS were included, of whom eight (16%) had a confirmed and three (6%) a probable SARS-CoV-2 infection. Nine of these 11 patients had no serological evidence of other recent preceding infections associated with GBS, whereas two had serological evidence of a recent Campylobacter jejuni infection. Patients with a confirmed or probable SARS-CoV-2 infection frequently had a sensorimotor variant 8/11 (73%) and facial palsy 7/11 (64%). The eight patients who underwent electrophysiological examination all had a demyelinating subtype, which was more prevalent than the other patients included in the same time window [14/30 (47%), P = 0.012] as well as historical region and age-matched control subjects included in the International GBS Outcome Study before the pandemic [23/44 (52%), P = 0.016]. The median time from the onset of infection to neurological symptoms was 16 days (interquartile range 12-22). Patients with SARS-CoV-2 infection shared uniform neurological fe

Published
2021

22. Antecedent infections in Guillain-Barré syndrome in endemic areas of arbovirus transmission:A multinational case-control study

Author

Leonhard, Sonja E., Tan, Cheng Yin, van der Eijk, Annemiek A., Reisin, Ricardo R., Franken, Suzanne C., Huizinga, Ruth, Arends, Samuel, Batstra, Manou R., Bezerra Jeronimo, Selma M., Drenthen, Judith, de Koning, Laura, Leon Cejas, Luciana, Marchesoni, Cintia, Marques, Wilson, Shahrizaila, Nortina, Casas, Dardo F., Sotelo, Andrea, Tillard, Belen, Dourado, Mario Emilio, Jacobs, Bart C., Leonhard, Sonja E., Tan, Cheng Yin, van der Eijk, Annemiek A., Reisin, Ricardo R., Franken, Suzanne C., Huizinga, Ruth, Arends, Samuel, Batstra, Manou R., Bezerra Jeronimo, Selma M., Drenthen, Judith, de Koning, Laura, Leon Cejas, Luciana, Marchesoni, Cintia, Marques, Wilson, Shahrizaila, Nortina, Casas, Dardo F., Sotelo, Andrea, Tillard, Belen, Dourado, Mario Emilio, and Jacobs, Bart C.

Abstract

Half of the world's population is at risk of arthropod-borne virus (arbovirus) infections. Several arbovirus infections have been associated with Guillain-Barré syndrome (GBS). We investigated whether arboviruses are driving GBS beyond epidemic phases of transmission and studied the antibody response to glycolipids. The protocol of the International Guillain-Barré syndrome Outcome Study (IGOS), an observational prospective cohort study, was adapted to a case-control design. Serum samples were tested for a recent infection with Zika virus (ZIKV), dengue virus (DENV), chikungunya (CHIKV) virus, hepatitis E virus, Epstein-Barr virus (EBV), cytomegalovirus (CMV), Campylobacter jejuni, and Mycoplasma pneumoniae, and for antibodies to glycolipids. Forty-nine patients were included from Brazil (63%), Argentina (14%), and Malaysia (22%). Evidence of a recent infection was found in 27/49 (55%) patients: C jejuni (n = 15, 31%), M pneumoniae (n = 5, 10%), CHIKV (n = 2, 4%), EBV (n = 1, 2%), C jejuni and M pneumoniae (n = 2, 4%), CMV and DENV (n = 1, 2%), and C jejuni and DENV (n = 1, 2%). In 22 patients, 35 paired controls were collected. Odds ratio for recent infections did not significantly differ between cases and controls. No typical anti-ganglioside antibody binding was associated with recent arbovirus infection. We conclude that arbovirus infections occur in GBS patients outside of epidemic viral transmission, although not significantly more than in controls. Broad infection and anti-ganglioside antibody serology are important to establish the most likely pathogenic trigger in GBS patients. Larger studies are necessary to determine the association between arboviruses and GBS.

Published
2021

23. Guía basada en la evidencia. Diagnóstico y manejo del síndrome de guillain-barré en diez pasos

Author

Leonhard, Sonja E., Mandarakas, Melissa R., Gondim, Francisco De Assis Aquino, Bateman, Kathleen, Ferreira, Maria L.Brito, Cornblath, David R., Van doorn, Pieter A., Dourado, Mario E., Hughes, Richard A.C., Islam, Badrul, Kusunoki, Susumu, Pardo, Carlos A., Reisin, Ricardo, Sejvar, James J., Shahrizaila, Nortina, Soares, Cristiane, Umapathi, Thirugnanam, Wang, Yuzhong, Yiu, Eppie M., Willison, Hugh J., Jacobs, Bart C., Leonhard, Sonja E., Mandarakas, Melissa R., Gondim, Francisco De Assis Aquino, Bateman, Kathleen, Ferreira, Maria L.Brito, Cornblath, David R., Van doorn, Pieter A., Dourado, Mario E., Hughes, Richard A.C., Islam, Badrul, Kusunoki, Susumu, Pardo, Carlos A., Reisin, Ricardo, Sejvar, James J., Shahrizaila, Nortina, Soares, Cristiane, Umapathi, Thirugnanam, Wang, Yuzhong, Yiu, Eppie M., Willison, Hugh J., and Jacobs, Bart C.

Abstract

Guillain-Barré syndrome (GBS) is a rare, but potentially fatal, immune-mediated disease of the peripheral nerves and nerve roots that is usually triggered by infections. The incidence of GBS can therefore increase during outbreaks of infectious diseases, as was seen during the Zika virus epidemics in 2013 in French Polynesia and in 2015 in Latin America. Diagnosis and management of GBS can be complicated as its clinical presentation and disease course are heterogeneous, and no international clinical guidelines are currently available. To support clinicians, especially in the context of an outbreak, we have developed a globally applicable guideline for the diagnosis and management of GBS. The guideline is based on current literature and expert consensus, and has a ten-step structure to facilitate its use in clinical practice. We first provide an introduction to the diagnostic criteria, clinical variants and differential diagnoses of GBS. The ten steps then cover early recognition and diagnosis of GBS, admission to the intensive care unit, treatment indication and selection, monitoring and treatment of disease progression, prediction of clinical course and outcome, and management of complications and sequelae.

Published
2021

24. Guillain-Barré syndrome during the Zika virus outbreak in Northeast Brazil:An observational cohort study

Author

Leonhard, Sonja E., Halstead, Susan, Lant, Suzannah B., Militão de Albuquerque, Maria de Fatima Pessoa, de Brito, Carlos Alexandre Antunes, de Albuquerque, Lívia Brito Bezerra, Ellul, Mark A., de Oliveira França, Rafael Freitas, Gourlay, Dawn, Griffiths, Michael J., de Miranda Henriques-Souza, Adélia Maria, de Morais Machado, Maria, Medialdea-Carrera, Raquel, Mehta, Ravi, da Paz Melo, Roberta, Mesquita, Solange D., Moreira, Álvaro J.P., Pena, Lindomar J., Santos, Marcela Lopes, Turtle, Lance, Solomon, Tom, Willison, Hugh J., Jacobs, Bart C., Brito Ferreira, Maria L., Leonhard, Sonja E., Halstead, Susan, Lant, Suzannah B., Militão de Albuquerque, Maria de Fatima Pessoa, de Brito, Carlos Alexandre Antunes, de Albuquerque, Lívia Brito Bezerra, Ellul, Mark A., de Oliveira França, Rafael Freitas, Gourlay, Dawn, Griffiths, Michael J., de Miranda Henriques-Souza, Adélia Maria, de Morais Machado, Maria, Medialdea-Carrera, Raquel, Mehta, Ravi, da Paz Melo, Roberta, Mesquita, Solange D., Moreira, Álvaro J.P., Pena, Lindomar J., Santos, Marcela Lopes, Turtle, Lance, Solomon, Tom, Willison, Hugh J., Jacobs, Bart C., and Brito Ferreira, Maria L.

Abstract

Objective: To determine the clinical phenotype of Guillain-Barré syndrome (GBS) after Zika virus (ZIKV) infection, the anti-glycolipid antibody signature, and the role of other circulating arthropod-borne viruses, we describe a cohort of GBS patients identified during ZIKV and chikungunya virus (CHIKV) outbreaks in Northeast Brazil. Methods: We prospectively recruited GBS patients from a regional neurology center in Northeast Brazil between December 2014 and February 2017. Serum and CSF were tested for ZIKV, CHIKV, and dengue virus (DENV), by RT-PCR and antibodies, and serum was tested for GBS-associated antibodies to glycolipids. Results: Seventy-one patients were identified. Forty-eight (68%) had laboratory evidence of a recent arbovirus infection; 25 (52%) ZIKV, 8 (17%) CHIKV, 1 (2%) DENV, and 14 (29%) ZIKV and CHIKV. Most patients with a recent arbovirus infection had motor and sensory symptoms (72%), a demyelinating electrophysiological subtype (67%) and a facial palsy (58%). Patients with a recent infection with ZIKV and CHIKV had a longer hospital admission and more frequent mechanical ventilation compared to the other patients. No specific anti-glycolipid antibody signature was identified in association with arbovirus infection, although significant antibody titres to GM1, GalC, LM1, and GalNAc-GD1a were found infrequently. Conclusion: A large proportion of cases had laboratory evidence of a recent infection with ZIKV or CHIKV, and recent infection with both viruses was found in almost one third of patients. Most patients with a recent arbovirus infection had a sensorimotor, demyelinating GBS. We did not find a specific anti-glycolipid antibody signature in association with arbovirus-related GBS.

Published
2021

25. Diretrizes Baseadas em Evidências Diagnóstico e manejo da Síndrome de Guillain–Barré em dez etapas

Author

Leonhard, Sonja E., primary, Mandarakas, Melissa R., additional, Gondim, Francisco A.A., additional, Bateman, Kathleen, additional, Ferreira, Maria L.B., additional, Cornblath, David R., additional, Van Doorn, Pieter A., additional, Dourado, Mario E., additional, Hughes, Richard A.C., additional, Islam, Badrul, additional, Kusunoki, Susumu, additional, Pardo, Carlos A., additional, Reisin, Ricardo, additional, Sejvar, James J., additional, Shahrizaila, Nortina, additional, Soares, Cristiane, additional, Umapathi, Thirugnanam, additional, Wang, Yuzhong, additional, Yiu, Eppie M., additional, Willison, Hugh J., additional, and Jacobs, Bart C., additional

Published
2021
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26. Guillain-Barré Syndrome Outbreak in Peru 2019 Associated With Campylobacter jejuni Infection

Author

Ramos, Ana P., primary, Leonhard, Sonja E., additional, Halstead, Susan K., additional, Cuba, Mireya A., additional, Castañeda, Carlos C., additional, Dioses, Jose A., additional, Tipismana, Martin A., additional, Abanto, Jesus T., additional, Llanos, Alejandro, additional, Gourlay, Dawn, additional, Grogl, Max, additional, Ramos, Mariana, additional, Rojas, Jesus D., additional, Meza, Rina, additional, Puiu, Daniela, additional, Sherman, Rachel M., additional, Salzberg, Steven L., additional, Simner, Patricia J., additional, Willison, Hugh J., additional, Jacobs, Bart C., additional, Cornblath, David R., additional, Umeres, Hugo F., additional, and Pardo, Carlos A., additional

Published
2021
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27. An International Perspective on Preceding Infections in Guillain-Barré Syndrome

Author

Leonhard, Sonja E., van der Eijk, Annemiek A., Andersen, Henning, Antonini, Giovanni, Arends, Samuel, Attarian, Shahram, Barroso, Fabio A., Bateman, Kathleen J., Batstra, Manou R., Benedetti, Luana, van den Berg, Bianca, Van den Bergh, Peter, Bürmann, Jan, Busby, Mark, Casasnovas, Carlos, Cornblath, David R., Davidson, Amy, Doets, Alex Y., van Doorn, Pieter A., Dornonville de la Cour, Charlotte, Feasby, Thomas E., Fehmi, Janev, Garcia-Sobrino, Tania, Goldstein, Jonathan M., Gorson, Kenneth C., Granit, Volkan, Hadden, Robert D.M., Harbo, Thomas, Hartung, Hans-Peter, Hasan, Imran, Holbech, Jakob V., Holt, James K.L., Jahan, Israt, Islam, Zhahirul, Karafiath, Summer, Katzberg, Hans D., Kleyweg, Ruud P., Kolb, Noah, Kuitwaard, Krista, Kuwahara, Motoi, Kusunoki, Susumu, Luijten, Linda W.G., Kuwabara, Satoshi, Lee Pan, Edward, Lehmann, Helmar C., Maas, Marijke, Martín-Aguilar, Lorena, Miller, James A.L., Mohammad, Quazi Deen, Monges, Soledad, Nedkova-Hristova, Velina, Nobile-Orazio, Eduardo, Pardo, Julio, Pereon, Yann, Querol, Luis, Reisin, Ricardo, Van Rijs, Wouter, Rinaldi, Simon, Roberts, Rhys C., Roodbol, Joyce, Shahrizaila, Nortina, Sindrup, Søren Hein, Stein, Beth, Cheng-Yin, Tan, Tankisi, Hatice, Tio-Gillen, Anne P., Sedano Tous, María J., Verboon, Christine, Vermeij, Frederique H., Visser, Leo H., Huizinga, Ruth, Willison, Hugh J., and Jacobs, Bart C.

Published
2022
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31. Predicting Outcome in Guillain-Barré Syndrome

Author

Doets, Alex Y., Lingsma, Hester F., Walgaard, Christa, Islam, Badrul, Papri, Nowshin, Davidson, Amy, Yamagishi, Yuko, Kusunoki, Susumu, Dimachkie, Mazen M., Waheed, Waqar, Kolb, Noah, Islam, Zhahirul, Mohammad, Quazi Deen, Harbo, Thomas, Sindrup, Soren H., Chavada, Govindsinh, Willison, Hugh J., Casasnovas, Carlos, Bateman, Kathleen, Miller, James A.L., van den Berg, Bianca, Verboon, Christine, Roodbol, Joyce, Leonhard, Sonja E., Benedetti, Luana, Kuwabara, Satoshi, Van den Bergh, Peter, Monges, Soledad, Marfia, Girolama A., Shahrizaila, Nortina, Galassi, Giuliana, Péréon, Yann, Bürmann, Jan, Kuitwaard, Krista, Kleyweg, Ruud P., Marchesoni, Cintia, Sedano Tous, María J., Querol, Luis, Illa, Isabel, Wang, Yuzhong, Nobile-Orazio, Eduardo, Rinaldi, Simon, Schenone, Angelo, Pardo, Julio, Vermeij, Frederique H., Lehmann, Helmar C., Granit, Volkan, Cavaletti, Guido, Gutiérrez-Gutiérrez, Gerardo, Barroso, Fabio A., Visser, Leo H., Katzberg, Hans D., Dardiotis, Efthimios, Attarian, Shahram, van der Kooi, Anneke J., Eftimov, Filip, Wirtz, Paul W., Samijn, Johnny P.A., Gilhuis, H. Jacobus, Hadden, Robert D.M., Holt, James K.L., Sheikh, Kazim A., Karafiath, Summer, Vytopil, Michal, Antonini, Giovanni, Feasby, Thomas E., Faber, Catharina G., Gijsbers, Cees J., Busby, Mark, Roberts, Rhys C., Silvestri, Nicholas J., Fazio, Raffaella, van Dijk, Gert W., Garssen, Marcel P.J., Straathof, Chiara S.M., Gorson, Kenneth C., Jacobs, Bart C., Hughes, R.A.C., Cornblath, D.R., Hartung, H.P., van Doorn, P.A., de Koning, L.C., van Woerkom, M., Mandarakas, M., MPhty, BHIthSci(Hons), Reisin, R.C., Reddel, S.W., Ripellino, P., Hsieh, S.T., Addington, J.M., Ajroud-Driss, S., Andersen, H., Badrising, U.A., Bella, I.R., Bertorini, T.E., Bhavaraju-Sanka, R., Bianco, M., Brannagan, T.H., Briani, Chiara, Butterworth, S., Chao, C.C., Chen, S., Claeys, K.G., Conti, M.E., Cosgrove, J.S., Dalakas, M.C., Dornonville de la Cour, C., Echaniz-Laguna, A., Fehmi, J., Fokke, C., Fujioka, T., Fulgenzi, E.A., García-Sobrino, T., Gilchrist, J.M., Goldstein, J.M., Goyal, N.A., Grisanti, S.G., Gutman, L., Holbech, J.V., Homedes, C., Htut, M., Jellema, K., Pascual, I. Jericó, JimenoMontero, M.C., Kaida, K., Khoshnoodi, M., Kiers, L., Kimpinski, K., Köhler, A.A., Kokubun, N., Kuwahara, M., Kwan, J.Y., Ladha, S.S., Lassen, L. Landschoff, Lawson, V., Pan, E.B. Lee, Cejas, L. Léon, Lunn, M.P.T., Magot, A., Manji, H., Infante, C. Márquez, Martín-Aguilar, L., Hernandez, E. Martinez, Mataluni, G., Mattiazzi, M.G., McDermott, C.J., Meekins, G.D., Morís de la Tassa, G., Nascimbene, C., Nowak, R.J., Osei-Bonsu, M., Pascuzzi, R.M., Prada, V., Rojas-Marcos, I., Rudnicki, S.A., Sachs, G.M., Samukawa, M., Santoro, L., Savransky, A.G., Schwindling, L., Sekiguchi, Y., Sommer, C.L., Spyropoulos, A., Stein, B., Stino, A.M., Tan, C.Y., Tankisi, H., Twydell, P.T., van Damme, P., van der Ree, T., van Koningsveld, R., Varrato, J.D., Xing, C., Zhou, L., and Zivkovic, S.

Published
2022
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33. Neurosarcoidosis in a Tertiary Referral Center

Author

Leonhard, Sonja E., Fritz, Daan, Eftimov, Filip, van der Kooi, Anneke J., van de Beek, Diederik, and Brouwer, Matthijs C.

Subjects
Adult, Male, Sarcoidosis, Observational Study, Middle Aged, Cohort Studies, Tertiary Care Centers, Cross-Sectional Studies, Central Nervous System Diseases, ComputingMethodologies_DOCUMENTANDTEXTPROCESSING, Humans, Female, Research Article
Abstract

Supplemental Digital Content is available in the text, The aim of this study was to evaluate clinical characteristics, diagnostic strategy, and treatment in patients with neurosarcoidosis in a tertiary referral centre. In a cross-sectional study, we included all patients with neurosarcoidosis treated at our tertiary referral center between September 2014 and April 2015. We identified 52 patients, among them 1 patient was categorized as having definite neurosarcoidosis, 37 probable neurosarcoidosis, and 14 possible neurosarcoidosis. Neurologic symptoms were the first manifestation of sarcoidosis in 37 patients (71%). Chronic aseptic meningitis was the most common presentation (19/52 patients [37%]), followed by cranial neuropathy (16/52 patients [31%]). Serum angiotensin-converting enzyme and lysozyme levels were elevated in 18 of 41 (44%) and 12 of 26 cases (46%). Pulmonary or lymph node sarcoidosis was identified by chest X-ray in 21 of 39 cases (54%) and by computed tomography of the chest in 25 of 31 cases (81%); 18Fluorodeoxyglucose-Positron emission tomography showed signs of sarcoidosis in 15 of 19 cases (79%). Thirty-one of the 46 cases receiving treatment (67%) improved, 13 cases (28%) stabilized, and 2 cases (4%) deteriorated. First-line treatment with corticosteroids resulted in satisfactory reduction of symptoms in 21 of 43 patients (49%). Seventeen patients (33%) needed second-line cytostatic treatment, and 10 patients (19%) were treated with tumor necrosis factor-α inhibitors. The majority of patients with neurosarcoidosis present with chronic meningitis without a history of systemic sarcoidosis. The diagnosis can be difficult to make because of the poor sensitivity of most diagnostic tests. Half of patients had a satisfactory reduction of symptoms on first-line therapy.

Published
2016

36. Guillain-Barré syndrome following SARS-CoV-2 vaccination in the UK: a prospective surveillance study.

Author

Tamborska AA, Singh B, Leonhard SE, Hodel EM, Stowe J, Watson-Fargie T, Fernandes PM, Themistocleous AC, Roelofs J, Brennan K, Morrice C, Michael BD, Jacobs BC, McDonald H, and Solomon T

Abstract

Objective: To investigate features of Guillain-Barré syndrome (GBS) following SARS-CoV-2 vaccines and evaluate for a causal link between the two., Methods: We captured cases of GBS after SARS-CoV-2 vaccination through a national, open-access, online surveillance system. For each case, the certainty of GBS was graded using the Brighton criteria, and the relationship to the vaccine was examined using modified WHO Causality Assessment criteria. We compared age distribution of cases with that of prepandemic GBS cases and clinical features with the International GBS Outcome Study (IGOS)., Results: Between 1 January and 30 June 2021, we received 67 reports of GBS following the ChAdOx1 vaccine (65 first doses) and three reports following the BNT162b2 vaccine (all first doses). The causal association with the vaccine was classified as probable for 56 (80%, all ChAdOx1), possible for 12 (17%, 10 ChAdOx1) and unlikely for two (3%, 1 ChAdOx1). A greater proportion of cases occurred in the 50-59 age group in comparison with prepandemic GBS. Most common clinical variants were sensorimotor GBS (n=55; 79%) and facial diplegia with paraesthesias (n=10; 14%). 10% (n=7/69) of patients reported an antecedent infection, compared with 77% (n=502/652) of the IGOS cohort (p<0.00001). Facial weakness (63% (n=44/70) vs 36% (n=220/620); p<0.00001) and sensory dysfunction (93% (n=63/68) vs 69% (n=408/588); p=0.00005) were more common but disease severity and outcomes were similar to the IGOS study., Interpretation: Most reports of GBS followed the first dose of ChAdOx1 vaccine. While our study cannot confirm or refute causation, this observation, together with the absence of alternative aetiologies, different than expected age distribution and the presence of unusual clinical features support a causal link. Clinicians and surveillance bodies should remain vigilant to the possibility of this very rare adverse event and its atypical variants., Competing Interests: Competing interests: TS was chair/cochair of the UK Research and Innovation/National Institute for Health Research COVID-19 Rapid Response and Rolling Funding Initiatives, was an Advisor to the UK COVID-19 Therapeutics Advisory Panel and is a member of the UK Medicines and Healthcare Products Regulatory Agency COVID-19 Vaccines Benefit Risk Expert Working Group. BCJ is a chair of Steering Committee of IGOS. HM is an invited expert for the Commission on Human Medicines COVID-19 Vaccines Safety Surveillance Methodologies Expert Working Group. The remaining authors have no relevant conflict of interest to declare., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY. Published by BMJ.)

Published
2022
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37. Guillain-Barré syndrome after SARS-CoV-2 infection in an international prospective cohort study.

Author

Luijten LWG, Leonhard SE, van der Eijk AA, Doets AY, Appeltshauser L, Arends S, Attarian S, Benedetti L, Briani C, Casasnovas C, Castellani F, Dardiotis E, Echaniz-Laguna A, Garssen MPJ, Harbo T, Huizinga R, Humm AM, Jellema K, van der Kooi AJ, Kuitwaard K, Kuntzer T, Kusunoki S, Lascano AM, Martinez-Hernandez E, Rinaldi S, Samijn JPA, Scheidegger O, Tsouni P, Vicino A, Visser LH, Walgaard C, Wang Y, Wirtz PW, Ripellino P, and Jacobs BC

Subjects
Adult, Aged, Cohort Studies, Female, Guillain-Barre Syndrome virology, Humans, Male, Middle Aged, Prospective Studies, SARS-CoV-2, COVID-19 complications, Guillain-Barre Syndrome epidemiology
Abstract

In the wake of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic, an increasing number of patients with neurological disorders, including Guillain-Barré syndrome (GBS), have been reported following this infection. It remains unclear, however, if these cases are coincidental or not, as most publications were case reports or small regional retrospective cohort studies. The International GBS Outcome Study is an ongoing prospective observational cohort study enrolling patients with GBS within 2 weeks from onset of weakness. Data from patients included in this study, between 30 January 2020 and 30 May 2020, were used to investigate clinical and laboratory signs of a preceding or concurrent SARS-CoV-2 infection and to describe the associated clinical phenotype and disease course. Patients were classified according to the SARS-CoV-2 case definitions of the European Centre for Disease Prevention and Control and laboratory recommendations of the World Health Organization. Forty-nine patients with GBS were included, of whom eight (16%) had a confirmed and three (6%) a probable SARS-CoV-2 infection. Nine of these 11 patients had no serological evidence of other recent preceding infections associated with GBS, whereas two had serological evidence of a recent Campylobacter jejuni infection. Patients with a confirmed or probable SARS-CoV-2 infection frequently had a sensorimotor variant 8/11 (73%) and facial palsy 7/11 (64%). The eight patients who underwent electrophysiological examination all had a demyelinating subtype, which was more prevalent than the other patients included in the same time window [14/30 (47%), P = 0.012] as well as historical region and age-matched control subjects included in the International GBS Outcome Study before the pandemic [23/44 (52%), P = 0.016]. The median time from the onset of infection to neurological symptoms was 16 days (interquartile range 12-22). Patients with SARS-CoV-2 infection shared uniform neurological features, similar to those previously described in other post-viral GBS patients. The frequency (22%) of a preceding SARS-CoV-2 infection in our study population was higher than estimates of the contemporaneous background prevalence of SARS-CoV-2, which may be a result of recruitment bias during the pandemic, but could also indicate that GBS may rarely follow a recent SARS-CoV-2 infection. Consistent with previous studies, we found no increase in patient recruitment during the pandemic for our ongoing International GBS Outcome Study compared to previous years, making a strong relationship of GBS with SARS-CoV-2 unlikely. A case-control study is required to determine if there is a causative link or not., (© The Author(s) (2021). Published by Oxford University Press on behalf of the Guarantors of Brain.)

Published
2021
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38. Guillain-Barré Syndrome Outbreak in Peru 2019 Associated With Campylobacter jejuni Infection.

Author

Ramos AP, Leonhard SE, Halstead SK, Cuba MA, Castañeda CC, Dioses JA, Tipismana MA, Abanto JT, Llanos A, Gourlay D, Grogl M, Ramos M, Rojas JD, Meza R, Puiu D, Sherman RM, Salzberg SL, Simner PJ, Willison HJ, Jacobs BC, Cornblath DR, Umeres HF, and Pardo CA

Subjects
Adult, Campylobacter Infections blood, Case-Control Studies, Female, Guillain-Barre Syndrome blood, Humans, Male, Middle Aged, Peru epidemiology, Campylobacter Infections diagnosis, Campylobacter Infections epidemiology, Campylobacter jejuni isolation & purification, Disease Outbreaks, Guillain-Barre Syndrome diagnosis, Guillain-Barre Syndrome epidemiology
Abstract

Objective: To identify the clinical phenotypes and infectious triggers in the 2019 Peruvian Guillain-Barré syndrome (GBS) outbreak., Methods: We prospectively collected clinical and neurophysiologic data of patients with GBS admitted to a tertiary hospital in Lima, Peru, between May and August 2019. Molecular, immunologic, and microbiological methods were used to identify causative infectious agents. Sera from 41 controls were compared with cases for antibodies to Campylobacter jejuni and gangliosides. Genomic analysis was performed on 4 C jejuni isolates., Results: The 49 included patients had a median age of 44 years (interquartile range [IQR] 30-54 years), and 28 (57%) were male. Thirty-two (65%) had symptoms of a preceding infection: 24 (49%) diarrhea and 13 (27%) upper respiratory tract infection. The median time between infectious to neurologic symptoms was 3 days (IQR 2-9 days). Eighty percent had a pure motor form of GBS, 21 (43%) had the axonal electrophysiologic subtype, and 18% the demyelinating subtype. Evidence of recent C jejuni infection was found in 28/43 (65%). No evidence of recent arbovirus infection was found. Twenty-three cases vs 11 controls (OR 3.3, confidence interval [CI] 95% 1.2-9.2, p < 0.01) had IgM and/or IgA antibodies against C jejuni . Anti-GM1:phosphatidylserine and/or anti-GT1a:GM1 heteromeric complex antibodies were strongly positive in cases (92.9% sensitivity and 68.3% specificity). Genomic analysis showed that the C jejuni strains were closely related and had the Asn51 polymorphism at cstII gene., Conclusions: Our study indicates that the 2019 Peruvian GBS outbreak was associated with C jejuni infection and that the C jejuni strains linked to GBS circulate widely in different parts of the world., (Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.)

Published
2021
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39. [Evidence based guidelines. Diagnosis and management of Guillain-Barré syndrome in ten steps].

Author

Leonhard SE, Mandarakas MR, de Assis Aquino Gondim F, Bateman K, Brito Ferreira ML, Cornblath DR, Van Doorn PA, Dourado ME, Hughes RAC, Islam B, Kusunoki S, Pardo CA, Reisin R, Sejvar JJ, Shahrizaila N, Soares C, Umapathi T, Wang Y, Yiu EM, Willison HJ, and Jacobs BC

Subjects
Disease Outbreaks, Humans, Incidence, Guillain-Barre Syndrome diagnosis, Guillain-Barre Syndrome epidemiology, Guillain-Barre Syndrome therapy, Zika Virus, Zika Virus Infection diagnosis, Zika Virus Infection epidemiology, Zika Virus Infection therapy
Abstract

Guillain-Barré syndrome (GBS) is a rare, but potentially fatal, immune-mediated disease of the peripheral nerves and nerve roots that is usually triggered by infections. The incidence of GBS can therefore increase during outbreaks of infectious diseases, as was seen during the Zika virus epidemics in 2013 in French Polynesia and in 2015 in Latin America. Diagnosis and management of GBS can be complicated as its clinical presentation and disease course are heterogeneous, and no international clinical guidelines are currently available. To support clinicians, especially in the context of an outbreak, we have developed a globally applicable guideline for the diagnosis and management of GBS. The guideline is based on current literature and expert consensus, and has a ten-step structure to facilitate its use in clinical practice. We first provide an introduction to the diagnostic criteria, clinical variants and differential diagnoses of GBS. The ten steps then cover early recognition and diagnosis of GBS, admission to the intensive care unit, treatment indication and selection, monitoring and treatment of disease progression, prediction of clinical course and outcome, and management of complications and sequelae.

Published
2021

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