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1. Electrodiagnostic subtyping in Guillain–Barré syndrome patients in the International Guillain–Barré Outcome Study

Author

Arends, S, Drenthen, J, de Koning, L, van den Bergh, P, Hadden, R, Kuwabara, S, Reisin, R, Shahrizaila, N, Ajroud-Driss, S, Antonini, G, Attarian, S, Balducci, C, Bertorini, T, Brannagan, T, Cavaletti, G, Chao, C, Chavada, G, Dillmann, K, Dimachkie, M, Galassi, G, Gutierrez-Gutierrez, G, Harbo, T, Islam, B, Islam, Z, Katzberg, H, Kusunoki, S, Manganelli, F, Miller, J, Pardo, J, Pereon, Y, Rajabally, Y, Sindrup, S, Stettner, M, Uncini, A, Verhamme, C, Vytopil, M, Waheed, W, Jacobs, B, Cornblath, D, Addington, J, Badrising, U, Barroso, F, Bateman, K, Bella, I, Benedetti, L, van den Berg, B, Bhavaraju-Sanka, R, Briani, C, Buermann, J, Busby, M, Butterworth, S, Casasnovas, C, Chen, S, Claeys, K, Conti, E, Cosgrove, J, Dalakas, M, van Damme, P, Dardiotis, E, Davidson, A, Doets, A, van Doorn, P, Echaniz-Laguna, A, Eftimov, F, Faber, K, Fazio, R, Feasby, T, Fehmi, J, Fokke, C, Fujioka, T, Fulgenzi, E, Garssen, M, Gijsbers, C, Gilchrist, J, Gilhuis, J, Goldstein, J, Gorson, K, Goyal, N, Granit, V, Gutmann, L, Hartung, H, Holt, J, Hsieh, S, Htut, M, Hughes, R, Jerico-Pascual, I, Kaida, K, Karafiath, S, Khoshnoodi, M, Kiers, L, Kleiweg, R, Kokubun, N, Kolb, N, van Koningsveld, R, van der Kooi, A, Kramers, H, Kuitwaard, K, Kwan, J, Ladha, S, Lassen, L, Lawson, V, Lehmann, H, Cejas, L, Leonhard, S, Luijten, L, Lunn, M, Manji, H, Marfia, G, Infante, C, Martin-Aguilar, L, Martinez-Hernandez, E, Mataluni, G, Mattiazzi, M, Mcdermott, C, Meekins, G, Mohammad, Q, Monges, S, de la Tassa, G, Nascimbene, C, Nobile-Orazio, E, Nowak, R, Osei-Bonsu, M, Pelouto, F, Pulley, M, Gutierrez, L, Reddel, S, van der Ree, T, Rinaldi, S, Ripellino, P, Roberts, R, Rojas-Marcos, I, Roodbol, J, Rudnicki, S, Sachs, G, Samijn, J, Santoro, L, Schenone, A, Tous, M, Sheikh, K, Silvestri, N, Sundrup, S, Sommer, C, Stein, B, Stino, A, Thomma, R, Twydell, P, Varrato, J, Vermeij, F, Verschuuren, J, Visser, L, Walgaard, C, Wang, Y, Willison, H, Wirtz, P, van Woerkom, M, Zivkovic, S, Arends S., Drenthen J., de Koning L., van den Bergh P., Hadden R. D. M., Kuwabara S., Reisin R. C., Shahrizaila N., Ajroud-Driss S., Antonini G., Attarian S., Balducci C., Bertorini T., Brannagan T. H., Cavaletti G., Chao C. -C., Chavada G., Dillmann K. -U., Dimachkie M. M., Galassi G., Gutierrez-Gutierrez G., Harbo T., Islam B., Islam Z., Katzberg H., Kusunoki S., Manganelli F., Miller J. A. L., Pardo J., Pereon Y., Rajabally Y. A., Sindrup S., Stettner M., Uncini A., Verhamme C., Vytopil M., Waheed W., Jacobs B. C., Cornblath D. R., Addington J. M., Badrising U. A., Barroso F. A., Bateman K., Bella I., Benedetti L., van den Berg B., Bhavaraju-Sanka R., Briani C., Buermann J., Busby M., Butterworth S., Casasnovas C., Chen S., Claeys K., Conti E., Cosgrove J. S., Dalakas M., van Damme P., Dardiotis E., Davidson A., Doets A., van Doorn P., Echaniz-Laguna A., Eftimov F., Faber K. G., Fazio R., Feasby T. E., Fehmi J., Fokke C., Fujioka T., Fulgenzi E., Garssen M. P. J., Gijsbers C. J., Gilchrist J. M., Gilhuis J., Goldstein J. M., Gorson K. C., Goyal N., Granit V., Gutmann L., Hartung H. -P., Holt J. K. L., Hsieh S. -T., Htut M., Hughes R. A. C., Jerico-Pascual I., Kaida K., Karafiath S., Khoshnoodi M. A., Kiers L., Kleiweg R. P., Kokubun N., Kolb N. A., van Koningsveld R., van der Kooi A. J., Kramers H., Kuitwaard K., Kwan J. Y., Ladha S. S., Lassen L. L., Lawson V. H., Lehmann H., Cejas L. L., Leonhard S. E., Luijten L., Lunn M. P. T., Manji H., Marfia G. A., Infante C. M., Martin-Aguilar L., Martinez-Hernandez E., Mataluni G., Mattiazzi M., McDermott C., Meekins G., Mohammad Q. D., Monges S., de la Tassa G. M., Nascimbene C., Nobile-Orazio E., Nowak R. J., Osei-Bonsu M., Pelouto F., Pulley M. T., Gutierrez L. Q., Reddel S. W., van der Ree T., Rinaldi S., Ripellino P., Roberts R. C., Rojas-Marcos I., Roodbol J., Rudnicki S. A., Sachs G. M., Samijn J. P. A., Santoro L., Schenone A., Tous M. J. S., Sheikh K. A., Silvestri N. J., Sundrup S. H., Sommer C., Stein B., Stino A. M., Thomma R. C. M., Twydell P., Varrato J. D., Vermeij F. H., Verschuuren J., Visser L. H., Walgaard C., Wang Y., Willison H. J., Wirtz P. W., van Woerkom M., Zivkovic S. A., Arends, S, Drenthen, J, de Koning, L, van den Bergh, P, Hadden, R, Kuwabara, S, Reisin, R, Shahrizaila, N, Ajroud-Driss, S, Antonini, G, Attarian, S, Balducci, C, Bertorini, T, Brannagan, T, Cavaletti, G, Chao, C, Chavada, G, Dillmann, K, Dimachkie, M, Galassi, G, Gutierrez-Gutierrez, G, Harbo, T, Islam, B, Islam, Z, Katzberg, H, Kusunoki, S, Manganelli, F, Miller, J, Pardo, J, Pereon, Y, Rajabally, Y, Sindrup, S, Stettner, M, Uncini, A, Verhamme, C, Vytopil, M, Waheed, W, Jacobs, B, Cornblath, D, Addington, J, Badrising, U, Barroso, F, Bateman, K, Bella, I, Benedetti, L, van den Berg, B, Bhavaraju-Sanka, R, Briani, C, Buermann, J, Busby, M, Butterworth, S, Casasnovas, C, Chen, S, Claeys, K, Conti, E, Cosgrove, J, Dalakas, M, van Damme, P, Dardiotis, E, Davidson, A, Doets, A, van Doorn, P, Echaniz-Laguna, A, Eftimov, F, Faber, K, Fazio, R, Feasby, T, Fehmi, J, Fokke, C, Fujioka, T, Fulgenzi, E, Garssen, M, Gijsbers, C, Gilchrist, J, Gilhuis, J, Goldstein, J, Gorson, K, Goyal, N, Granit, V, Gutmann, L, Hartung, H, Holt, J, Hsieh, S, Htut, M, Hughes, R, Jerico-Pascual, I, Kaida, K, Karafiath, S, Khoshnoodi, M, Kiers, L, Kleiweg, R, Kokubun, N, Kolb, N, van Koningsveld, R, van der Kooi, A, Kramers, H, Kuitwaard, K, Kwan, J, Ladha, S, Lassen, L, Lawson, V, Lehmann, H, Cejas, L, Leonhard, S, Luijten, L, Lunn, M, Manji, H, Marfia, G, Infante, C, Martin-Aguilar, L, Martinez-Hernandez, E, Mataluni, G, Mattiazzi, M, Mcdermott, C, Meekins, G, Mohammad, Q, Monges, S, de la Tassa, G, Nascimbene, C, Nobile-Orazio, E, Nowak, R, Osei-Bonsu, M, Pelouto, F, Pulley, M, Gutierrez, L, Reddel, S, van der Ree, T, Rinaldi, S, Ripellino, P, Roberts, R, Rojas-Marcos, I, Roodbol, J, Rudnicki, S, Sachs, G, Samijn, J, Santoro, L, Schenone, A, Tous, M, Sheikh, K, Silvestri, N, Sundrup, S, Sommer, C, Stein, B, Stino, A, Thomma, R, Twydell, P, Varrato, J, Vermeij, F, Verschuuren, J, Visser, L, Walgaard, C, Wang, Y, Willison, H, Wirtz, P, van Woerkom, M, Zivkovic, S, Arends S., Drenthen J., de Koning L., van den Bergh P., Hadden R. D. M., Kuwabara S., Reisin R. C., Shahrizaila N., Ajroud-Driss S., Antonini G., Attarian S., Balducci C., Bertorini T., Brannagan T. H., Cavaletti G., Chao C. -C., Chavada G., Dillmann K. -U., Dimachkie M. M., Galassi G., Gutierrez-Gutierrez G., Harbo T., Islam B., Islam Z., Katzberg H., Kusunoki S., Manganelli F., Miller J. A. L., Pardo J., Pereon Y., Rajabally Y. A., Sindrup S., Stettner M., Uncini A., Verhamme C., Vytopil M., Waheed W., Jacobs B. C., Cornblath D. R., Addington J. M., Badrising U. A., Barroso F. A., Bateman K., Bella I., Benedetti L., van den Berg B., Bhavaraju-Sanka R., Briani C., Buermann J., Busby M., Butterworth S., Casasnovas C., Chen S., Claeys K., Conti E., Cosgrove J. S., Dalakas M., van Damme P., Dardiotis E., Davidson A., Doets A., van Doorn P., Echaniz-Laguna A., Eftimov F., Faber K. G., Fazio R., Feasby T. E., Fehmi J., Fokke C., Fujioka T., Fulgenzi E., Garssen M. P. J., Gijsbers C. J., Gilchrist J. M., Gilhuis J., Goldstein J. M., Gorson K. C., Goyal N., Granit V., Gutmann L., Hartung H. -P., Holt J. K. L., Hsieh S. -T., Htut M., Hughes R. A. C., Jerico-Pascual I., Kaida K., Karafiath S., Khoshnoodi M. A., Kiers L., Kleiweg R. P., Kokubun N., Kolb N. A., van Koningsveld R., van der Kooi A. J., Kramers H., Kuitwaard K., Kwan J. Y., Ladha S. S., Lassen L. L., Lawson V. H., Lehmann H., Cejas L. L., Leonhard S. E., Luijten L., Lunn M. P. T., Manji H., Marfia G. A., Infante C. M., Martin-Aguilar L., Martinez-Hernandez E., Mataluni G., Mattiazzi M., McDermott C., Meekins G., Mohammad Q. D., Monges S., de la Tassa G. M., Nascimbene C., Nobile-Orazio E., Nowak R. J., Osei-Bonsu M., Pelouto F., Pulley M. T., Gutierrez L. Q., Reddel S. W., van der Ree T., Rinaldi S., Ripellino P., Roberts R. C., Rojas-Marcos I., Roodbol J., Rudnicki S. A., Sachs G. M., Samijn J. P. A., Santoro L., Schenone A., Tous M. J. S., Sheikh K. A., Silvestri N. J., Sundrup S. H., Sommer C., Stein B., Stino A. M., Thomma R. C. M., Twydell P., Varrato J. D., Vermeij F. H., Verschuuren J., Visser L. H., Walgaard C., Wang Y., Willison H. J., Wirtz P. W., van Woerkom M., and Zivkovic S. A.

Abstract

Background and purpose: Various electrodiagnostic criteria have been developed in Guillain–Barré syndrome (GBS). Their performance in a broad representation of GBS patients has not been evaluated. Motor conduction data from the International GBS Outcome Study (IGOS) cohort were used to compare two widely used criterion sets and relate these to diagnostic amyotrophic lateral sclerosis criteria. Methods: From the first 1500 patients in IGOS, nerve conduction studies from 1137 (75.8%) were available for the current study. These patients were classified according to nerve conduction studies criteria proposed by Hadden and Rajabally. Results: Of the 1137 studies, 68.3% (N = 777) were classified identically according to criteria by Hadden and Rajabally: 111 (9.8%) axonal, 366 (32.2%) demyelinating, 195 (17.2%) equivocal, 35 (3.1%) inexcitable and 70 (6.2%) normal. Thus, 360 studies (31.7%) were classified differently. The areas of differences were as follows: 155 studies (13.6%) classified as demyelinating by Hadden and axonal by Rajabally; 122 studies (10.7%) classified as demyelinating by Hadden and equivocal by Rajabally; and 75 studies (6.6%) classified as equivocal by Hadden and axonal by Rajabally. Due to more strictly defined cutoffs fewer patients fulfilled demyelinating criteria by Rajabally than by Hadden, making more patients eligible for axonal or equivocal classification by Rajabally. In 234 (68.6%) axonal studies by Rajabally the revised El Escorial (amyotrophic lateral sclerosis) criteria were fulfilled; in axonal cases by Hadden this was 1.8%. Conclusions and discussion: This study shows that electrodiagnosis in GBS is dependent on the criterion set utilized, both of which are based on expert opinion. Reappraisal of electrodiagnostic subtyping in GBS is warranted.

Published
2024

5. Autoantibody screening in Guillain-Barré syndrome

Author

Lleixà, Cinta, Martín-Aguilar, Lorena, Pascual-Goñi, Elba, Franco-Leyva, Teresa, Caballero, Marta, de Luna Salva, Noemí, Gallardo, Eduard, Suarez-Calvet, Xavier, Martínez-Martínez, Laura, Diaz-Manera, Jordi, Rojas-Garcia, Ricard, Cortés-Vicente, Elena, Turón, Joana, Casasnovas, Carlos, Homedes, C., Gutiérrez-Gutiérrez, G., Jimeno-Montero, M. C., Berciano, José, Sedano-Tous, M. J., García-Sobrino, Tania, Pardo, Julio, Márquez-Infante, C., Rojas-Marcos, I., Jericó, Ivonne, Martínez-Hernández, E., Morís, Germán, Domínguez-González, C., Juarez, Candido, Illa, Isabel, Querol, Luis, Universitat Autònoma de Barcelona, and Universidad de Cantabria

Subjects
Male, Cohort Studies, Pathogenesis, Ganglia, Spinal, Mass Screening, Prospective Studies, Neurons, biology, Guillain-Barre syndrome, General Neuroscience, Middle Aged, Guillain-Barré syndrome, Prognosis, medicine.anatomical_structure, Neurology, Nervous system--Diseases, Immunohistochemistry, Female, Antibody, Immunology, Immunocytochemistry, Immunoglobulins, Guillain-Barre Syndrome, Malalties del sistema nerviós, Cellular and Molecular Neuroscience, Antigen, Cell Line, Tumor, medicine, Guillain–Barré syndrome (GBS), Animals, Humans, RC346-429, Aged, Autoantibodies, Anti-ganglioside, business.industry, Research, Autoantibody, Nervous system Diseases, Neuron, medicine.disease, Rats, Spain, biology.protein, Macaca, Neurology. Diseases of the nervous system, Immunoglobulines, business
Abstract

Background Guillain–Barré syndrome (GBS) is an acute inflammatory neuropathy with a heterogeneous presentation. Although some evidences support the role of autoantibodies in its pathogenesis, the target antigens remain unknown in a substantial proportion of GBS patients. The objective of this study is to screen for autoantibodies targeting peripheral nerve components in Guillain–Barré syndrome. Methods Autoantibody screening was performed in serum samples from all GBS patients included in the International GBS Outcome study by 11 different Spanish centres. The screening included testing for anti-ganglioside antibodies, anti-nodo/paranodal antibodies, immunocytochemistry on neuroblastoma-derived human motor neurons and murine dorsal root ganglia (DRG) neurons, and immunohistochemistry on monkey peripheral nerve sections. We analysed the staining patterns of patients and controls. The prognostic value of anti-ganglioside antibodies was also analysed. Results None of the GBS patients (n = 100) reacted against the nodo/paranodal proteins tested, and 61 (61%) were positive for, at least, one anti-ganglioside antibody. GBS sera reacted strongly against DRG neurons more frequently than controls both with IgG (6% vs 0%; p = 0.03) and IgM (11% vs 2.2%; p = 0.02) immunodetection. No differences were observed in the proportion of patients reacting against neuroblastoma-derived human motor neurons. Reactivity against monkey nerve tissue was frequently detected both in patients and controls, but specific patterns were only detected in GBS patients: IgG from 13 (13%) patients reacted strongly against Schwann cells. Finally, we confirmed that IgG anti-GM1 antibodies are associated with poorer outcomes independently of other known prognostic factors. Conclusion Our study confirms that (1) GBS patients display a heterogeneous repertoire of autoantibodies targeting nerve cells and structures; (2) gangliosides are the most frequent antigens in GBS patients and have a prognostic value; (3) further antigen-discovery experiments may elucidate other potential antigens in GBS.

Published
2021

6. Current treatment practice of Guillain-Barré syndrome

Author

Verboon, C, Doets, A, Galassi, G, Davidson, A, Waheed, W, Pereon, Y, Shahrizaila, N, Kusunoki, S, Lehmann, H, Harbo, T, Monges, S, Van Den Bergh, P, Willison, H, Cornblath, D, Jacobs, B, Hughes, R, Gorson, K, Hartung, H, Van Doorn, P, Van den Berg, B, Roodbol, J, Van Woerkom, M, Reisin, R, Reddel, S, Islam, Z, Islam, B, Mohammad, Q, Feasby, T, Dardiotis, E, Nobile-Orazio, E, Bateman, K, Illa, I, Querol, L, Hsieh, S, Chavada, G, Addington, J, Ajroud-Driss, S, Andersen, H, Antonini, G, Ariatti, A, Attarian, S, Badrising, U, Barroso, F, Benedetti, L, Beronio, A, Bianco, M, Binda, D, Briani, C, Bunschoten, C, Burmann, J, Bella, I, Bertorini, T, Bhavaraju-Sanka, R, Brannagan, T, Busby, M, Butterworth, S, Casasnovas, C, Cavaletti, G, Chao, C, Chen, S, Chetty, S, Claeys, K, Conti, M, Cosgrove, J, Dalakas, M, Demichelis, C, Derejko, M, Dillmann, U, Dimachkie, M, Doppler, K, Dornonville de la Cour, C, Echaniz-Laguna, A, Eftimov, F, Faber, C, Fazio, R, Fokke, C, Fujioka, T, Fulgenzi, E, Garcia-Sobrino, T, Garssen, M, Georgios, H, Gijsbers, C, Gilchrist, J, Gilhuis, J, Giorli, E, Goldstein, J, Goyal, N, Granit, V, Grapperon, A, Gutierrez, G, Hadden, R, Holbech, J, Holt, J, Pedret, C, Htut, M, Jellema, K, Pascual, I, Jimeno-Montero, M, Kaida, K, Karafiath, S, Katzberg, H, Kiers, L, Kieseier, B, Kimpinski, K, Kleyweg, R, Kokubun, N, Kolb, N, Kuitwaard, K, Kuwabara, S, Kwan, J, Ladha, S, Lassen, L, Lawson, V, Ledingham, D, Lucy, S, Lunn, M, Magot, A, Manji, H, Marchesoni, C, Marfia, G, Infante, C, Hernandez, E, Mataluni, G, Mattiazi, M, Mcdermott, C, Meekins, G, Miller, J, Moris de la Tassa, G, Physiotherapist, J, Nascimbene, C, Nowak, R, Balaguer, P, Osei-Bonsu, M, Pan, E, Pardal, A, Pardo, J, Pasnoor, M, Pulley, M, Rajabally, Y, Rinaldi, S, Ritter, C, Roberts, R, Rojas-Marcos, I, Rudnicki, S, Ruiz, M, Sachs, G, Samijn, J, Santoro, L, Savransky, A, Schenone, A, Schwindling, L, Tous, M, Sekiguchi, Y, Sheikh, K, Silvestri, N, Sindrup, S, Sommer, C, Stein, B, Stino, A, Spyropoulos, A, Srinivasan, J, Styliani, R, Suzuki, H, Tankisi, H, Tigner, D, Twydell, P, Van Damme, P, Van der Kooi, A, Van Dijk, G, Van der Ree, T, Van Koningsveld, R, Valzania, F, Varrato, J, Vermeij, F, Verschuuren, J, Visser, L, Vytopil, M, Wilken, M, Wilkerson, C, Wirtz, P, Yamagishi, Y, Zhou, L, Zivkovic, S, Verboon C., Doets A. Y., Galassi G., Davidson A., Waheed W., Pereon Y., Shahrizaila N., Kusunoki S., Lehmann H. C., Harbo T., Monges S., Van Den Bergh P., Willison H. J., Cornblath D. R., Jacobs B. C., Hughes R. A. C., Gorson K. C., Hartung H. P., Van Doorn P. A., Van den Berg B., Roodbol J., Van Woerkom M., Reisin R. C., Reddel S. W., Islam Z., Islam B., Mohammad Q. D., Feasby T. E., Dardiotis E., Nobile-Orazio E., Bateman K., Illa I., Querol L., Hsieh S. T., Chavada G., Addington J. M., Ajroud-Driss S., Andersen H., Antonini G., Ariatti A., Attarian S., Badrising U. A., Barroso F. A., Benedetti L., Beronio A., Bianco M., Binda D., Briani C., Bunschoten C., Burmann J., Bella I. R., Bertorini T. E., Bhavaraju-Sanka R., Brannagan T. H., Busby M., Butterworth S., Casasnovas C., Cavaletti G., Chao C. C., Chen S., Chetty S., Claeys K. G., Conti M. E., Cosgrove J. S., Dalakas MC., Demichelis C., Derejko M. A., Dillmann U., Dimachkie M. M., Doppler K., Dornonville de la Cour C., Echaniz-Laguna A., Eftimov F., Faber C. G., Fazio R., Fokke C., Fujioka T., Fulgenzi E. A., Garcia-Sobrino T., Garssen M. P. J., Georgios H. M., Gijsbers C. J., Gilchrist J. M., Gilhuis J., Giorli E., Goldstein J. M., Goyal N. A., Granit V., Grapperon A., Gutierrez G., Hadden R. D. M., Holbech J. V., Holt J. K. L., Pedret C. H., Htut M., Jellema K., Pascual I. J., Jimeno-Montero M. C., Kaida K., Karafiath S., Katzberg H. D., Kiers L., Kieseier B. C., Kimpinski K., Kleyweg R. P., Kokubun N., Kolb N. A., Kuitwaard K., Kuwabara S., Kwan J. Y., Ladha S. S., Lassen L. L., Lawson V., Ledingham D., Lucy S. T., Lunn M. P. T., Magot A., Manji H., Marchesoni C., Marfia G. A., Infante C. M., Hernandez E. M., Mataluni G., Mattiazi M., McDermott C. J., Meekins G. D., Miller J. A. L., Moris de la Tassa G., Physiotherapist J. M., Nascimbene C., Nowak R. J., Balaguer P. O., Osei-Bonsu M., Pan E. B. L., Pardal A. M., Pardo J., Pasnoor M., Pulley M., Rajabally Y. A., Rinaldi S., Ritter C., Roberts R. C., Rojas-Marcos I., Rudnicki S. A., Ruiz M., Sachs G. M., Samijn J. P. A., Santoro L., Savransky A., Schenone A., Schwindling L., Tous M. J. S., Sekiguchi Y., Sheikh K. A., Silvestri N. J., Sindrup S. H., Sommer C. L., Stein B., Stino A. M., Spyropoulos A., Srinivasan J., Styliani R., Suzuki H., Tankisi H., Tigner D., Twydell P., Van Damme P., Van der Kooi A. J., Van Dijk G. W., Van der Ree T., Van Koningsveld R., Valzania F., Varrato J. D., Vermeij F. H., Verschuuren J., Visser L. H., Vytopil M. V., Wilken M., Wilkerson C., Wirtz P. W., Yamagishi Y., Zhou L., Zivkovic S. A., Verboon, C, Doets, A, Galassi, G, Davidson, A, Waheed, W, Pereon, Y, Shahrizaila, N, Kusunoki, S, Lehmann, H, Harbo, T, Monges, S, Van Den Bergh, P, Willison, H, Cornblath, D, Jacobs, B, Hughes, R, Gorson, K, Hartung, H, Van Doorn, P, Van den Berg, B, Roodbol, J, Van Woerkom, M, Reisin, R, Reddel, S, Islam, Z, Islam, B, Mohammad, Q, Feasby, T, Dardiotis, E, Nobile-Orazio, E, Bateman, K, Illa, I, Querol, L, Hsieh, S, Chavada, G, Addington, J, Ajroud-Driss, S, Andersen, H, Antonini, G, Ariatti, A, Attarian, S, Badrising, U, Barroso, F, Benedetti, L, Beronio, A, Bianco, M, Binda, D, Briani, C, Bunschoten, C, Burmann, J, Bella, I, Bertorini, T, Bhavaraju-Sanka, R, Brannagan, T, Busby, M, Butterworth, S, Casasnovas, C, Cavaletti, G, Chao, C, Chen, S, Chetty, S, Claeys, K, Conti, M, Cosgrove, J, Dalakas, M, Demichelis, C, Derejko, M, Dillmann, U, Dimachkie, M, Doppler, K, Dornonville de la Cour, C, Echaniz-Laguna, A, Eftimov, F, Faber, C, Fazio, R, Fokke, C, Fujioka, T, Fulgenzi, E, Garcia-Sobrino, T, Garssen, M, Georgios, H, Gijsbers, C, Gilchrist, J, Gilhuis, J, Giorli, E, Goldstein, J, Goyal, N, Granit, V, Grapperon, A, Gutierrez, G, Hadden, R, Holbech, J, Holt, J, Pedret, C, Htut, M, Jellema, K, Pascual, I, Jimeno-Montero, M, Kaida, K, Karafiath, S, Katzberg, H, Kiers, L, Kieseier, B, Kimpinski, K, Kleyweg, R, Kokubun, N, Kolb, N, Kuitwaard, K, Kuwabara, S, Kwan, J, Ladha, S, Lassen, L, Lawson, V, Ledingham, D, Lucy, S, Lunn, M, Magot, A, Manji, H, Marchesoni, C, Marfia, G, Infante, C, Hernandez, E, Mataluni, G, Mattiazi, M, Mcdermott, C, Meekins, G, Miller, J, Moris de la Tassa, G, Physiotherapist, J, Nascimbene, C, Nowak, R, Balaguer, P, Osei-Bonsu, M, Pan, E, Pardal, A, Pardo, J, Pasnoor, M, Pulley, M, Rajabally, Y, Rinaldi, S, Ritter, C, Roberts, R, Rojas-Marcos, I, Rudnicki, S, Ruiz, M, Sachs, G, Samijn, J, Santoro, L, Savransky, A, Schenone, A, Schwindling, L, Tous, M, Sekiguchi, Y, Sheikh, K, Silvestri, N, Sindrup, S, Sommer, C, Stein, B, Stino, A, Spyropoulos, A, Srinivasan, J, Styliani, R, Suzuki, H, Tankisi, H, Tigner, D, Twydell, P, Van Damme, P, Van der Kooi, A, Van Dijk, G, Van der Ree, T, Van Koningsveld, R, Valzania, F, Varrato, J, Vermeij, F, Verschuuren, J, Visser, L, Vytopil, M, Wilken, M, Wilkerson, C, Wirtz, P, Yamagishi, Y, Zhou, L, Zivkovic, S, Verboon C., Doets A. Y., Galassi G., Davidson A., Waheed W., Pereon Y., Shahrizaila N., Kusunoki S., Lehmann H. C., Harbo T., Monges S., Van Den Bergh P., Willison H. J., Cornblath D. R., Jacobs B. C., Hughes R. A. C., Gorson K. C., Hartung H. P., Van Doorn P. A., Van den Berg B., Roodbol J., Van Woerkom M., Reisin R. C., Reddel S. W., Islam Z., Islam B., Mohammad Q. D., Feasby T. E., Dardiotis E., Nobile-Orazio E., Bateman K., Illa I., Querol L., Hsieh S. T., Chavada G., Addington J. M., Ajroud-Driss S., Andersen H., Antonini G., Ariatti A., Attarian S., Badrising U. A., Barroso F. A., Benedetti L., Beronio A., Bianco M., Binda D., Briani C., Bunschoten C., Burmann J., Bella I. R., Bertorini T. E., Bhavaraju-Sanka R., Brannagan T. H., Busby M., Butterworth S., Casasnovas C., Cavaletti G., Chao C. C., Chen S., Chetty S., Claeys K. G., Conti M. E., Cosgrove J. S., Dalakas MC., Demichelis C., Derejko M. A., Dillmann U., Dimachkie M. M., Doppler K., Dornonville de la Cour C., Echaniz-Laguna A., Eftimov F., Faber C. G., Fazio R., Fokke C., Fujioka T., Fulgenzi E. A., Garcia-Sobrino T., Garssen M. P. J., Georgios H. M., Gijsbers C. J., Gilchrist J. M., Gilhuis J., Giorli E., Goldstein J. M., Goyal N. A., Granit V., Grapperon A., Gutierrez G., Hadden R. D. M., Holbech J. V., Holt J. K. L., Pedret C. H., Htut M., Jellema K., Pascual I. J., Jimeno-Montero M. C., Kaida K., Karafiath S., Katzberg H. D., Kiers L., Kieseier B. C., Kimpinski K., Kleyweg R. P., Kokubun N., Kolb N. A., Kuitwaard K., Kuwabara S., Kwan J. Y., Ladha S. S., Lassen L. L., Lawson V., Ledingham D., Lucy S. T., Lunn M. P. T., Magot A., Manji H., Marchesoni C., Marfia G. A., Infante C. M., Hernandez E. M., Mataluni G., Mattiazi M., McDermott C. J., Meekins G. D., Miller J. A. L., Moris de la Tassa G., Physiotherapist J. M., Nascimbene C., Nowak R. J., Balaguer P. O., Osei-Bonsu M., Pan E. B. L., Pardal A. M., Pardo J., Pasnoor M., Pulley M., Rajabally Y. A., Rinaldi S., Ritter C., Roberts R. C., Rojas-Marcos I., Rudnicki S. A., Ruiz M., Sachs G. M., Samijn J. P. A., Santoro L., Savransky A., Schenone A., Schwindling L., Tous M. J. S., Sekiguchi Y., Sheikh K. A., Silvestri N. J., Sindrup S. H., Sommer C. L., Stein B., Stino A. M., Spyropoulos A., Srinivasan J., Styliani R., Suzuki H., Tankisi H., Tigner D., Twydell P., Van Damme P., Van der Kooi A. J., Van Dijk G. W., Van der Ree T., Van Koningsveld R., Valzania F., Varrato J. D., Vermeij F. H., Verschuuren J., Visser L. H., Vytopil M. V., Wilken M., Wilkerson C., Wirtz P. W., Yamagishi Y., Zhou L., and Zivkovic S. A.

Abstract

ObjectiveTo define the current treatment practice of Guillain-Barré syndrome (GBS).MethodsThe study was based on prospective observational data from the first 1,300 patients included in the International GBS Outcome Study. We described the treatment practice of GBS in general, and for (1) severe forms (unable to walk independently), (2) no recovery after initial treatment, (3) treatment-related fluctuations, (4) mild forms (able to walk independently), and (5) variant forms including Miller Fisher syndrome, taking patient characteristics and hospital type into account.ResultsWe excluded 88 (7%) patients because of missing data, protocol violation, or alternative diagnosis. Patients from Bangladesh (n = 189, 15%) were described separately because 83% were not treated. IV immunoglobulin (IVIg), plasma exchange (PE), or other immunotherapy was provided in 941 (92%) of the remaining 1,023 patients, including patients with severe GBS (724/743, 97%), mild GBS (126/168, 75%), Miller Fisher syndrome (53/70, 76%), and other variants (33/40, 83%). Of 235 (32%) patients who did not improve after their initial treatment, 82 (35%) received a second immune modulatory treatment. A treatment-related fluctuation was observed in 53 (5%) of 1,023 patients, of whom 36 (68%) were re-treated with IVIg or PE.ConclusionsIn current practice, patients with mild and variant forms of GBS, or with treatment-related fluctuations and treatment failures, are frequently treated, even in absence of trial data to support this choice. The variability in treatment practice can be explained in part by the lack of evidence and guidelines for effective treatment in these situations.

Published
2019

7. Original research: Second IVIg course in Guillain-Barré syndrome with poor prognosis: the non-randomised ISID study

Author

Verboon, C, van den Berg, B, Cornblath, Dr, Venema, E, Gorson, Kc, Lunn, Mp, Lingsma, H, Van den Bergh, P, Harbo, T, Bateman, K, Pereon, Y, Sindrup, Sh, Kusunoki, S, Miller, J, Islam, Z, Hartung, Hp, Chavada, G, Jacobs, Bc, Hughes, Rac, van Doorn PA, Ajroud-Driss S, IGOS Consortium., Antonini, G, Attarian, S, Barroso, Fa, Benedetti, L, Bertorini, Te, Brannagan, Th, Briani, C, Bhavaraju-Sanka, R, Butterworth, S, Casasnovas, C, Cavaletti, G, Chen, S, Claeys, Kg, Cosgrove, Js, Davidson, A, Dardiotis, E, Dornonville de la Cour, C, Faber, Cg, Feasby, Te, Fujioka, T, Galassi, G, Gilchrist, Jm, Goyal, Na, Granit, V, Gutiérrez-Gutiérrez, G, Hadden, Rdm, Holt, Jkl, Htut, M, Jericó Pascual, I, Karafiath, S, Katzberg, Hd, Kiers, L, Kieseier, Bc, Kimpinski, K, Kuwabara, S, Kwan, Jy, Ladha, Ss, Lawson, V, Lehmann, H, Manji, H, Marfia, Ga, Márquez Infante, C, Mattiazzi, Mg, Mcdermott, Cj, Monges, Ms, Morís de la Tassa, G, Nascimbene, C, Nobile Orazio, E, Nowak, Rj, Osei-Bonsu, M, Pardo Fernandez, J, Querol Gutierrez, L, Reisin, R, Rinaldi, S, Rojas-Marcos, I, Rudnicki, Sa, Schenone, A, Sedano Tous MJ, Shahrizaila, N, Sheikh, K, Silvestri, Nj, Sommer, Cl, Varrato, Jd, Verschuuren, J, Vytopil, Mv, Zhou, L, Bella, Ir, Bunschoten, C, Bürmann, J, Busby, M, Chao, Cc, Conti, Me, Dalakas, Mc, Van Damme, P, Doets, A, van Dijk GW, Dimachkie, Mm, Doppler, K, Echaniz-Laguna, A, Eftimov, F, Fazio, R, Fokke, C, Fulgenzi, Ea, Garssen, Mpj, Gijsbers, Cj, Gilhuis, J, Grapperon, A, Hsieh, St, Illa, I, Islam, B, Jellema, K, Kaida, K, Kokubun, N, Kolb, N, van Koningsveld, R, van der Kooi AJ, Kuitwaard, K, Landschoff Lassen, L, Leonhard, Se, Mandarakas, M, Martinez Hernandez, E, Mohammad, Qd, Pulley, M, Rajabally, Ya, Reddel, Sw, van der Ree, T, Roodbol, J, Sachs, Gm, Samijn, Jpa, Santoro, L, Stein, B, Vermeij, Fh, Visser, Lh, Willison, Hj, Wirtz, P, Zivkovich, Sa., Neurology, Public Health, Immunology, and ANS - Neuroinfection & -inflammation

Subjects
Adult, Male, second IVIg course, Pediatrics, medicine.medical_specialty, Poor prognosis, Time Factors, Guillain-Barre Syndrome, Original research, Drug Administration Schedule, Disease course, Disability Evaluation, 03 medical and health sciences, 0302 clinical medicine, hemic and lymphatic diseases, Clinical endpoint, Humans, Immunologic Factors, Medicine, In patient, Aged, treatment, Guillain-Barre syndrome, business.industry, Immunoglobulins, Intravenous, Middle Aged, poor prognosis, Prognosis, Guillain-Barré syndrome, medicine.disease, Psychiatry and Mental health, Treatment Outcome, Immunoglobulin G, 030220 oncology & carcinogenesis, Female, Surgery, Observational study, Neurology (clinical), business, 030217 neurology & neurosurgery
Abstract

ObjectiveTo compare disease course in patients with Guillain-Barré syndrome (GBS) with a poor prognosis who were treated with one or with two intravenous immunoglobulin (IVIg) courses.MethodsFrom the International GBS Outcome Study, we selected patients whose modified Erasmus GBS Outcome Score at week 1 predicted a poor prognosis. We compared those treated with one IVIg course to those treated with two IVIg courses. The primary endpoint, the GBS disability scale at 4 weeks, was assessed with multivariable ordinal regression.ResultsOf 237 eligible patients, 199 patients received a single IVIg course. Twenty patients received an ‘early’ second IVIg course (1–2 weeks after start of the first IVIg course) and 18 patients a ‘late’ second IVIg course (2–4 weeks after start of IVIg). At baseline and 1 week, those receiving two IVIg courses were more disabled than those receiving one course. Compared with the one course group, the adjusted OR for a better GBS disability score at 4 weeks was 0.70 (95%CI 0.16 to 3.04) for the early group and 0.66 (95%CI 0.18 to 2.50) for the late group. The secondary endpoints were not in favour of a second IVIg course.ConclusionsThis observational study did not show better outcomes after a second IVIg course in GBS with poor prognosis. The study was limited by small numbers and baseline imbalances. Lack of improvement was likely an incentive to start a second IVIg course. A prospective randomised trial is needed to evaluate whether a second IVIg course improves outcome in GBS.

Published
2019

8. Intravenous immunoglobulin treatment for mild Guillain-Barré syndrome. An international observational study

Author

Verboon, C., Harbo, T., Cornblath, D. R., Hughes, R. A. C., Van Doorn, P. A., Lunn, M. P., Gorson, K. C., Barroso, F., Kuwabara, S., Galassi, G., Lehmann, H. C., Kusunoki, S., Reisin, R. C., Binda, D., Cavaletti, G., Andersen, Jacobs B. C. H., PhD (Aarhus University Hospital, Aarhus, Denmark), Attarian, S., PhD (CHU Timone, Marseille, France), Badrising, U. A., PhD (Leiden University Medical Centre, Leiden, The, Netherlands), Bateman, K., PhD (Groote Schuur Hospital, Cape, Town, South-Africa), Benedetti, L., PhD (Ospedale Sant’ Andrea La Spezia, Spezia, La, Italy), van den Berg, B., MD (Franciscus Gasthuis, Rotterdam, Van den Bergh, P., Luc, PhD (University Clinic St., Leuven, Belgium), Bertorini, T. E., MD (The University of Tennessee Health Science Center (UTHSC), Memphis, USA), Bhavaraju-Sanka, R., MD (University Hospital/ University of Texas Health Science Center, San Antonio Texas, USA), Bianco (Milan University, M., Humanitas Clinicala and Research Institute Milan, Briani, C., MD (University of Padova, Padova, Italy), Bürmann, J., MD (Universitätsklinikum des Saarlandes, Homburg, Germany), Casasnovas, C., Ciberer, PhD (Bellvitge University Hospital - IDIBELL Neurometabolic Diseases Group., Barcelona, Spain), Chao, C. C., PhD (National Taiwan University Hospital, Taipei, Taiwan), Chavada, G., PhD (Glasgow University, Glasgow, UK), Claeys, K. G., University Hospitals Leuven, PhD (1., Leuven, Belgium, KU Leuven, 2., Cosgrove, J. S., MD (Leeds General Infirmary, Leeds, UK), Dalakas, M. C., Thomas Jefferson University, MD (1., Philadelphia, Usa, National and Kapodistrian University of Athens, 2., Athens, Greece), Davidson, A., MD (University of Glasgow, van Dijk, G. W., MD (Canisius Wilhelmina Hospital, Nijmegen, Dardiotis, E., MD (University of Thessaly, Hospital of Larissa, Larissa, Greece), Derejko, M., MD (Odense University Hospital, Odense, Denmark), Dimachkie, M. M., MD (University of Kansas Medical Center, Kansas, City, Dornonville de la Cour, C., MD (National Hospital Copenhagen, Copenhagen, Denmark), Echaniz-Laguna, A., MD (Bicêtre University Hospital, Paris, France), Eftimov, F., PhD (Amsterdam University Medical Centre, Amsterdam, Faber, C. G., PhD (Maastricht University Medical Centre, Maastricht, Fazio, R., MD (Scientific Institute San Raffaele, Milan, Italy), Fulgenzi, J. Fehmi (University of Oxford E. A., MD (Hospital Cesar Milstein Buenos Aires, Buenos, Aires, Argentina), García-Sobrino, T., MD (Hospital Clínico de Santiago, Santiago de Compostela (A Coruña), Spain), Gijsbers, C. J., MD (Vlietland Hospital, Schiedam, Granit, V., MD (Montefiore Medical, Center, New, York, Grisanti, S., MD (Ospedale Sant’ Andrea La Spezia, Gutiérrez-Gutiérrez, G., MD (Hospital Universitario Infanta Sofia, San, Sebastian, Holbech, J. V., PhD (Odense University Hospital, Holt, J. K. L., Phd, FRCP (The Walton Centre, Liverpool, UK), Homedes, C., Ciberer, MD (Bellvitge University Hospital - IDIBELL Neurometabolic Diseases Group., Islam, B., PhD (International Centre for Diarrhoeal Disease Research, Bangladesh, (icddr, Dhaka, b), Bangladesh), Islam, Z., Jahan, I., PhD candidate (International Centre for Diarrhoeal Disease Research, Jericó Pascual, I., PhD (Complejo Hospitalario de Navarra, Pamplona, Spain), Karafiath, S., MD (University of Utah School of Medicine, Salt Lake City, Kerkhoff, H., PhD (Albert Schweitzer Hospital, Dordrecht, Kimpinski, K., MD (University Hospital, Lhsc, London-Ontario, Canada), Kohler, A., MD (Instituto de Investigaciones Neurológicas Raúl Carrea, Fleni, Kolb, N., MD (University of Vermont, Burlington, Vt, Kuitwaard, K., Albert Schweitzer Hospital, PhD (1., Erasmus MC, 2., Kuwahara, M., PhD (Kindai University, Osaka, Japan), Ladha, S. S., MD (Barrow Neurology Clinics, Phoenix, Arizona, Lee Pan, E., MBChB (Groote Schuur Hospital, Marfia, G. A., MD (Neurological Clinic, Policlinico Tor Vergata, Rome, Italy), Magot, A., MD (Reference Centre for NMD, Nantes University Hospital, France), Márquez Infante, C., MD (Hospital Universitario Virgen del Rocio, Seville, Spain), Martín-Aguilar, L., MD (Hospital de la Santa Creu, i Sant Pau, Universitat Autònoma de Barcelona, Martinez Hernandez, E., MD (Institut d’Investigacions Biomèdiques August Pi, i Sunyer (IDIBAPS), Hospital, Clinic, Mataluni, G., PhD (Neurological Clinic, Meekins, G., MD (University of Minnesota, Miller, J. A. L., PhD (Royal Victoria Infirmary, Newcastle, UK), Monges, M. S., Garrahan, MD (Hospital de Pediatría J. P., Nobile Orazio, E., PhD (Milan University, Pardal, A., MD (Hospital Britanico, Pardo Fernandez (Hospital Clínico de Santiago, J., Péréon, Y., PhD (Reference Centre for NMD, Pulley, M., MD (University of Florida, Jacksonville, USA), Querol Gutierrez, L., PhD (Hospital de la Santa Creu, i Sant Pau, Reddel, S. W., PhD (Concord Repatriation General Hospital, Sydney, Australia), van der Ree, T., (Westfriesgasthuis, Md, Hoorn, Rinaldi, S., Mbchb, Samijn, PhD (University of Oxford J. P. A., MD (Maasstad Hospital, Samukawa, M., Santoro, L., PhD (University Federico II, Napels, Italy), Savransky, A., Garrahan, PhD (Hospital de Pediatría J. P., Schwindling, L., Sedano Tous, M. J., MD (Hospital Universitario Marques de Valdecilla, Santander, Cantabria, Sekiguchi, Y., PhD (Chiba University, Chiba, Japan), Shahrizaila, N., MD (Neurology Unit, Department of Medicine, Faculty of Medicine, University of Malaya, Malaya), Silvestri, N. J., Sindrup, MD (Buffalo Jacobs School of Medicine S., Sommer, C. L., MD (Universitätsklinikum Würzburg, Würzburg, Germany), Spyropoulos (Royal Victoria Infirmary, A., Stein, B., Joseph’s Regional Medical Center, MD (St., Paterson, USA), Tan, C. Y., MRCP (Neurology Unit, Tankisi, H., Vermeij, F., Vytopil, M. V., Wirtz, PhD (Tufts University School of Medicine Lahey Hospital P. W., Phd, (HagaZiekenhuis, The, Hague, Waheed, W., MD (University of Vermont Medical Center, Burlington, Addington, USA). Other collaborators were:J. M., MD (University of Virginia, Charlottesville, USA), Ajroud-Driss, S., MD (Northwestern University Feinberg, Chicago, USA), Antonini, G., MD (Mental Health and Sensory Organs (NESMOS), Sapienza, University, Sant’Andrea, Hospital, Bella, I. R., MD (University of Mass Medical School, Worcester, USA), Brannagan, T. H., MD (Columbia University, New York City, Bunschoten, C., PhD candidate (Erasmus University Medical Centre, Busby, M., Bradford, UK), Butterworth, S., MD (Pinderfields Hospital, Wakefield, UK), Conti, M. E., MD (University Hospital Clinicas, Chen, S., Phd, (Rutgers, Robert Wood Johnson University Hospital, New, Brunswick, Doets, A., Feasby, T. E., MD (University of Calgary, Calgary, Canada), Fokke, C., MD (Gelre Hospital, Zutphen and Apeldoorn, Fujioka, T., MD (Toho University Medical Center, Tokyo, Japan), Garssen, M. P. J., PhD (Jeroen Bosch Hospital, Hertogenbosch, ’S, Gilchrist, J. M., MD (Soulthern Illinois University School of Medicine, Springfield, USA), Gilhuis, J., PhD (Reinier de Graaf Gasthuis, Delft, Goldstein, J. M., MD (Yale University School of Medicine, New, Haven, Goyal, N. A., MD (University of California, Irvine, USA), Hadden, R. D. M., PhD (King’s College Hospital, London, UK), Hsieh, S. T., Htut, M., George’s Hospital, MD (St., Illa, I., Jellema, K., PhD (Haaglanden Medisch Centrum, Kaida, K., PhD (National Defense Medical College, Saitama, Japan), Katzberg, H. D., MD (University of Toronto, Toronto, Canada), Kiers, L., MD (University of Melbourne, Royal Melbourne Hospital, Parkville, Australia), Kokubun, N., MD (Dokkyo Medical University, Tochigi, Japan), van Koningsveld, R., PhD (Elkerliek Hospital, Helmond and Deurne, van der Kooi, A. J., Kwan, J. Y., MD (University of Maryland School of Medicine, Baltimore, USA), Landschoff Lassen, L., MD (Glostrup Hospital, Glostrup, Denmark), Lawson, V., MD (Wexner Medical Center at The Ohio State University, Columbus, USA), Leonhard, S. E., Mandarakas, M., PhD (Erasmus University Medical Centre, Manji, H., FRCP (Ipswich Hospital, Ipswich, UK), Mattiazzi, M. G., MD (Hospital Militar Central, Mcdermott, C. J., MD (Royal Hallamshire Hospital, Nihr, Clinical, Sheffield, UK), Mohammad, Q. D., PhD (National Institute of Neurosciences and Hospital, Dhaka, Bangladesh), Morís de la Tassa, G., MD (Hospital UniversitarioCentral de Asturias, Asturias, Spain), Nascimbene, C., PhD (Luigi Sacco Hospital, Niks, E. H., Nowak, R. J., Osei-Bonsu, M., PhD (James Cook University Hospital, Middlesbrough, UK), Pascuzzi, R. M., MD (University of Indiana School of Medicine, Indianapolis, USA), Roberts, R. C., MD (Addenbrooke’s Hospital Cambridge, Cambridge, UK), Rojas-Marcos, I., MD (Hospital Univesitario Reina Sofia, Cordoba, Spain), Roodbol, J., Rudnicki, S. A., MD (University of Arkansas, Fayetteville, USA), Sachs, G. M., MD (University of Rhode Island, Providence, USA), Schenone, A., Department of Neurosciences, PhD (1., Rehabilitation, Ophthalmology, Genetics and Maternal and Infantile Sciences (DINOGMI), University of Genova, Genova, IRCCS Policlinico San Martino, Italy 2., Genova, Italy), Sheikh, K., PhD (The University of Texas Health Science Center at Houston, Houston, USA), Twydell, P., DO (Spectrum Health System, Grand, Rapids, Van Damme, P., PhD (University Hospital Leuven, Varrato, J. D., DO (Lehigh Valley Health Network, Allentown, USA), Visser, L. H., PhD (Elisabeth-TweeSteden Hospital, Tilburg and Waalwijk, Willison, H. J., PhD (University of Glasgow, van Woerkom (Erasmus MC, M., Zhou, L., PhD (Icahn School, Verboon, C, Harbo, T, Cornblath, D, Hughes, R, Van Doorn, P, Lunn, M, Gorson, K, Barroso, F, Kuwabara, S, Galassi, G, Lehmann, H, Kusunoki, S, Reisin, R, Binda, D, Cavaletti, G, Jacobs, B, consortium, IGOS, consortium, GOS, Neurosurgery, Neurology, and Immunology

Subjects
Adult, Male, medicine.medical_specialty, intravenous immunoglobulins, DIAGNOSIS, Guillain-Barre Syndrome, Settore MED/26, DISEASE, Disease course, Disability Evaluation, 03 medical and health sciences, 0302 clinical medicine, SDG 3 - Good Health and Well-being, hemic and lymphatic diseases, Internal medicine, Clinical endpoint, medicine, Humans, In patient, guillain-barré syndrome, 030212 general & internal medicine, NEUROPATHIES, biology, Guillain-Barre syndrome, business.industry, Guillain-Barré syndrome (GBS), treatment, course, Confounding, Immunoglobulins, Intravenous, Middle Aged, medicine.disease, Confidence interval, Psychiatry and Mental health, Treatment Outcome, biology.protein, Female, Surgery, Observational study, Neurology (clinical), Antibody, business, 030217 neurology & neurosurgery
Abstract

ObjectiveTo compare the disease course in patients with mild Guillain-Barré syndrome (GBS) who were treated with intravenous immunoglobulin (IVIg) or supportive care only.MethodsWe selected patients from the prospective observational International GBS Outcome Study (IGOS) who were able to walk independently at study entry (mild GBS), treated with one IVIg course or supportive care. The primary endpoint was the GBS disability score four weeks after study entry, assessed by multivariable ordinal regression analysis.ResultsOf 188 eligible patients, 148 (79%) were treated with IVIg and 40 (21%) with supportive care. The IVIg group was more disabled at baseline. IVIg treatment was not associated with lower GBS disability scores at 4 weeks (adjusted OR (aOR) 1.62, 95% CI 0.63 to 4.13). Nearly all secondary endpoints showed no benefit from IVIg, although the time to regain full muscle strength was shorter (28 vs 56 days, p=0.03) and reported pain at 26 weeks was lower (n=26/121, 22% vs n=12/30, 40%, p=0.04) in the IVIg treated patients. In the subanalysis with persistent mild GBS in the first 2 weeks, the aOR for a lower GBS disability score at 4 weeks was 2.32 (95% CI 0.76 to 7.13). At 1 year, 40% of all patients had residual symptoms.ConclusionIn patients with mild GBS, one course of IVIg did not improve the overall disease course. The certainty of this conclusion is limited by confounding factors, selection bias and wide confidence limits. Residual symptoms were often present after one year, indicating the need for better treatments in mild GBS.

Published
2021

9. Additional file 4 of Autoantibody screening in Guillain���Barr�� syndrome

Author

Lleix��, Cinta, Mart��n-Aguilar, Lorena, Pascual-Go��i, Elba, Franco, Teresa, Caballero, Marta, de Luna, Noem��, Gallardo, Eduard, Su��rez-Calvet, Xavier, Mart��nez-Mart��nez, Laura, Diaz-Manera, Jordi, Rojas-Garc��a, Ricard, Cort��s-Vicente, Elena, Tur��n, Joana, Casasnovas, Carlos, Homedes, Christian, Guti��rrez-Guti��rrez, Gerardo, Jimeno-Montero, Mar��a Concepci��n, Berciano, Jos��, Sedano-Tous, Maria Jos��, Garc��a-Sobrino, Tania, Pardo-Fern��ndez, Julio, M��rquez-Infante, Celedonio, Rojas-Marcos, I��igo, Jeric��-Pascual, Ivonne, Mart��nez-Hern��ndez, Eugenia, Mor��s de la Tassa, Germ��n, Dom��nguez-Gonz��lez, Cristina, Ju��rez, C��ndido, Illa, Isabel, and Querol, Luis

Abstract

Additional file 4: Table 1. Statistical analysis of structures observed in IHC over monkey peripheral nerve. Table 2. Statistical comparison between GBS patients with and without anti-ganglioside antibodies.

Published
2021
Full Text
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10. International Guillain-Barré Syndrome Outcome Study

Author

Jacobs, B, van den Berg, B, Verboon, C, Chavada, G, Cornblath, D, Gorson, K, Harbo, T, Hartung, H, Hughes, R, Kusunoki, S, van Doorn, P, Willison, H, Consortium, I, van Woerkom, M, Roodbol, J, Reisin, R, Reddel, S, Islam, Z, Islam, B, Mohammad, Q, van den Bergh, P, Feasby, T, Wang, Y, Péréon, Y, Lehmann, H, Dardiotis, E, Nobile Orazio, E, Shahrizaila, N, Bateman, K, Illa, I, Querol, L, Hsieh, S, Davidson, A, Addington, J, Ajroud Driss, S, Andersen, H, Antonini, G, Attarian, S, Badrising, U, Barroso, F, Benedetti, L, Beronio, A, Bianco, M, Binda, D, Briani, C, Bürmann, J, Bella, I, Bertorini, T, Bhavaraju Sanka, R, Brannagan, T, Busby, M, Butterworth, S, Campagnolo, M, Casasnovas, C, Cavaletti, G, Chao, C, Chen, S, Chetty, S, Claeys, K, Cohen, J, Conti, M, Cosgrove, J, Dalakas, M, Dimachkie, M, Dillmann, U, Domínguez González, C, Doppler, K, Dornonville de la Cour, C, Echaniz Laguna, A, Eftimov, F, Faber, C, Fazio, R, Fokke, C, Fujioka, T, Fulgenzi, E, Galassi, G, Garcia, T, Garnero, M, Garssen, M, Gijsbers, C, Gilchrist, J, Gilhuis, H, Goldstein, J, Goyal, N, Granit, V, Grapperon, A, Gutiérrez Gutiérrez, G, Gutmann, L, Hadden, R, Holbech, J, Holt, J, Homedes Pedret, C, Htut, M, Jellema, K, Jericó Pascual, I, Kaida, K, Karafiath, S, Katzberg, H, Kiers, L, Kieseier, B, Kimpinski, K, Kleyweg, R, Kokubun, N, Kolb, N, Kuitwaard, K, Kuwabara, S, Kwan, J, Ladha, S, Landschoff Lassen, L, Lawson, V, Ledingham, D, Léon Cejas, L, Luciano, C, Lucy, S, Lunn, M, Magot, A, Manji, H, Marchesoni, C, Marfia, Ga, Márquez Infante, C, Martinez Hernandez, E, Mataluni, G, Mattiazi, M, Mcdermott, C, Meekins, G, Miller, J, Monges, M, Montero, M, Morís de la Tassa, G, Nascimbene, C, Neumann, C, Nowak, R, Orizaola Balaguer, P, Osei Bonsu, M, Pan, E, Pardo Fernandez, J, Pasnoor, M, Pulley, M, Rajabally, Y, Rinaldi, S, Ritter, C, Roberts, R, Rojas Marcos, I, Rudnicki, S, Sachs, G, Samijn, J, Santoro, L, Saperstein, D, Savransky, A, Schneider, H, Schenone, A, Sedano Tous, M, Sekiguchi, Y, Sheikh, K, Silvestri, N, Sindrup, S, Sommer, C, Stein, B, Stino, A, Spyropoulos, A, Srinivasan, J, Suzuki, H, Taylor, S, Tankisi, H, Tigner, D, Twydell, P, Valzania, F, van Damme, P, van der Kooi, A, van Dijk, G, van der Ree, T, van Koningsveld, R, Varrato, J, Vermeij, F, Verschuuren, J, Visser, L, Vytopil, M, Waheed, W, Wilken, M, Wilkerson, C, Wirtz, P, Yamagishi, Y, Yiu, E, Zhou, L, Zivkovic, S, Immunology, Neurology, Jacobs, B, van den Berg, B, Verboon, C, Chavada, G, Cornblath, D, Gorson, K, Harbo, T, Hartung, H, Hughes, R, Kusunoki, S, van Doorn, P, Willison, H, van Woerkom, M, Roodbol, J, Reisin, R, Reddel, S, Islam, Z, Islam, B, Mohammad, Q, van den Bergh, P, Feasby, T, Wang, Y, Pã©rã©on, Y, Lehmann, H, Dardiotis, E, Nobile Orazio, E, Shahrizaila, N, Bateman, K, Illa, I, Querol, L, Hsieh, S, Davidson, A, Addington, J, Ajroud Driss, S, Andersen, H, Antonini, G, Attarian, S, Badrising, U, Barroso, F, Benedetti, L, Beronio, A, Bianco, M, Binda, D, Briani, C, Bã¼rmann, J, Bella, I, Bertorini, T, Bhavaraju Sanka, R, Brannagan, T, Busby, M, Butterworth, S, Campagnolo, M, Casasnovas, C, Cavaletti, G, Chao, C, Chen, S, Chetty, S, Claeys, K, Cohen, J, Conti, M, Cosgrove, J, Dalakas, M, Dimachkie, M, Dillmann, U, Domínguez González, C, Doppler, K, Dornonville de la Cour, C, Echaniz Laguna, A, Eftimov, F, Faber, C, Fazio, R, Fokke, C, Fujioka, T, Fulgenzi, E, Galassi, G, Garcia, T, Garnero, M, Garssen, M, Gijsbers, C, Gilchrist, J, Gilhuis, H, Goldstein, J, Goyal, N, Granit, V, Grapperon, A, Gutiérrez Gutiérrez, G, Gutmann, L, Hadden, R, Holbech, J, Holt, J, Homedes Pedret, C, Htut, M, Jellema, K, Jericó Pascual, I, Kaida, K, Karafiath, S, Katzberg, H, Kiers, L, Kieseier, B, Kimpinski, K, Kleyweg, R, Kokubun, N, Kolb, N, Kuitwaard, K, Kuwabara, S, Kwan, J, Ladha, S, Landschoff Lassen, L, Lawson, V, Ledingham, D, Léon Cejas, L, Luciano, C, Lucy, S, Lunn, M, Magot, A, Manji, H, Marchesoni, C, Marfia, G, Márquez Infante, C, Martinez Hernandez, E, Mataluni, G, Mattiazi, M, Mcdermott, C, Meekins, G, Miller, J, Monges, M, Montero, M, Morís de la Tassa, G, Nascimbene, C, Neumann, C, Nowak, R, Orizaola Balaguer, P, Osei Bonsu, M, Pan, E, Pardo Fernandez, J, Pasnoor, M, Pulley, M, Rajabally, Y, Rinaldi, S, Ritter, C, Roberts, R, Rojas Marcos, I, Rudnicki, S, Sachs, G, Samijn, J, Santoro, L, Saperstein, D, Savransky, A, Schneider, H, Schenone, A, Sedano Tous, M, Sekiguchi, Y, Sheikh, K, Silvestri, N, Sindrup, S, Sommer, C, Stein, B, Stino, A, Spyropoulos, A, Srinivasan, J, Suzuki, H, Taylor, S, Tankisi, H, Tigner, D, Twydell, P, Valzania, F, van Damme, P, van der Kooi, A, van Dijk, G, van der Ree, T, van Koningsveld, R, Varrato, J, Vermeij, F, Verschuuren, J, Visser, L, Vytopil, M, Waheed, W, Wilken, M, Wilkerson, C, Wirtz, P, Yamagishi, Y, Yiu, E, Zhou, L, Zivkovic, S, Rehabilitation medicine, Internal medicine, and ANS - Neuroinfection & -inflammation

Subjects
Male, Pediatrics, PROGNOSIS, diagnosis, International Cooperation, Guillain-Barré syndrome, biomarkers, outcome, prognosis, treatment, Guillain-Barre syndrome, Guillain-Barré syndrome, Neuroscience (all), Neurology (clinical), Cohort Studies, 0302 clinical medicine, Epidemiology, Outcome Assessment, Health Care, INFECTION, CRITERIA, 030212 general & internal medicine, General Neuroscience, Biobank, Observational Studies as Topic, diagnosi, Disease Progression, biomarker, Female, Settore MED/26 - Neurologia, medicine.symptom, prognosi, Cohort study, medicine.medical_specialty, Weakness, Guillain-Barre Syndrome, CLASSIFICATION, VALIDATION, 03 medical and health sciences, medicine, Humans, INTRAVENOUS IMMUNOGLOBULIN, Protocol (science), business.industry, Polyradiculoneuropathy, medicine.disease, ANTIBODIES, Observational study, business, COLLECTION, 030217 neurology & neurosurgery
Abstract

Guillain-Barré syndrome (GBS) is an acute polyradiculoneuropathy with a highly variable clinical presentation, course, and outcome. The factors that determine the clinical variation of GBS are poorly understood which complicates the care and treatment of individual patients. The protocol of the ongoing International GBS Outcome Study (IGOS), a prospective, observational, multicenter cohort study that aims to identify the clinical and biological determinants and predictors of disease onset, subtype, course and outcome of GBS is presented here. Patients fulfilling the diagnostic criteria for GBS, regardless of age, disease severity, variant forms, or treatment, can participate if included within 2 weeks after onset of weakness. Information about demography, preceding infections, clinical features, diagnostic findings, treatment, course, and outcome is collected. In addition, cerebrospinal fluid and serial blood samples for serum and DNA is collected at standard time points. The original aim was to include at least 1,000 patients with a follow-up of 1-3 years. Data are collected via a web-based data entry system and stored anonymously. IGOS started in May 2012 and by January 2017 included more than 1,400 participants from 143 active centers in 19 countries across 5 continents. The IGOS data/biobank is available for research projects conducted by expertise groups focusing on specific topics including epidemiology, diagnostic criteria, clinimetrics, electrophysiology, antecedent events, antibodies, genetics, prognostic modeling, treatment effects, and long-term outcome of GBS. The IGOS will help to standardize the international collection of data and biosamples for future research of GBS.

Published
2017

11. Current treatment practice of Guillain-Barré syndrome

Author

Verboon C., Doets A. Y., Galassi G., Davidson A., Waheed W., Pereon Y., Shahrizaila N., Kusunoki S., Lehmann H. C., Harbo T., Monges S., Van Den Bergh P., Willison H. J., Cornblath D. R., Jacobs B. C., Hughes R. A. C., Gorson K. C., Hartung H. P., Van Doorn P. A., Van den Berg B., Roodbol J., Van Woerkom M., Reisin R. C., Reddel S. W., Islam Z., Islam B., Mohammad Q. D., Feasby T. E., Dardiotis E., Nobile-Orazio E., Bateman K., Illa I., Querol L., Hsieh S. T., Chavada G., Addington J. M., Ajroud-Driss S., Andersen H., Antonini G., Ariatti A., Attarian S., Badrising U. A., Barroso F. A., Benedetti L., Beronio A., Bianco M., Binda D., Briani C., Bunschoten C., Burmann J., Bella I. R., Bertorini T. E., Bhavaraju-Sanka R., Brannagan T. H., Busby M., Butterworth S., Casasnovas C., Cavaletti G., Chao C. C., Chen S., Chetty S., Claeys K. G., Conti M. E., Cosgrove J. S., Dalakas MC., Demichelis C., Derejko M. A., Dillmann U., Dimachkie M. M., Doppler K., Dornonville de la Cour C., Echaniz-Laguna A., Eftimov F., Faber C. G., Fazio R., Fokke C., Fujioka T., Fulgenzi E. A., Garcia-Sobrino T., Garssen M. P. J., Georgios H. M., Gijsbers C. J., Gilchrist J. M., Gilhuis J., Giorli E., Goldstein J. M., Goyal N. A., Granit V., Grapperon A., Gutierrez G., Hadden R. D. M., Holbech J. V., Holt J. K. L., Pedret C. H., Htut M., Jellema K., Pascual I. J., Jimeno-Montero M. C., Kaida K., Karafiath S., Katzberg H. D., Kiers L., Kieseier B. C., Kimpinski K., Kleyweg R. P., Kokubun N., Kolb N. A., Kuitwaard K., Kuwabara S., Kwan J. Y., Ladha S. S., Lassen L. L., Lawson V., Ledingham D., Lucy S. T., Lunn M. P. T., Magot A., Manji H., Marchesoni C., Marfia G. A., Infante C. M., Hernandez E. M., Mataluni G., Mattiazi M., McDermott C. J., Meekins G. D., Miller J. A. L., Moris de la Tassa G., Physiotherapist J. M., Nascimbene C., Nowak R. J., Balaguer P. O., Osei-Bonsu M., Pan E. B. L., Pardal A. M., Pardo J., Pasnoor M., Pulley M., Rajabally Y. A., Rinaldi S., Ritter C., Roberts R. C., Rojas-Marcos I., Rudnicki S. A., Ruiz M., Sachs G. M., Samijn J. P. A., Santoro L., Savransky A., Schenone A., Schwindling L., Tous M. J. S., Sekiguchi Y., Sheikh K. A., Silvestri N. J., Sindrup S. H., Sommer C. L., Stein B., Stino A. M., Spyropoulos A., Srinivasan J., Styliani R., Suzuki H., Tankisi H., Tigner D., Twydell P., Van Damme P., Van der Kooi A. J., Van Dijk G. W., Van der Ree T., Van Koningsveld R., Valzania F., Varrato J. D., Vermeij F. H., Verschuuren J., Visser L. H., Vytopil M. V., Wilken M., Wilkerson C., Wirtz P. W., Yamagishi Y., Zhou L., Zivkovic S. A., Neurology, AII - Infectious diseases, AII - Inflammatory diseases, ANS - Neuroinfection & -inflammation, Immunology, Erasmus MC other, UCL - SSS/IONS/NEUR - Clinical Neuroscience, UCL - (SLuc) Service de neurologie, Verboon, C, Doets, A, Galassi, G, Davidson, A, Waheed, W, Pereon, Y, Shahrizaila, N, Kusunoki, S, Lehmann, H, Harbo, T, Monges, S, Van Den Bergh, P, Willison, H, Cornblath, D, Jacobs, B, Hughes, R, Gorson, K, Hartung, H, Van Doorn, P, Van den Berg, B, Roodbol, J, Van Woerkom, M, Reisin, R, Reddel, S, Islam, Z, Islam, B, Mohammad, Q, Feasby, T, Dardiotis, E, Nobile-Orazio, E, Bateman, K, Illa, I, Querol, L, Hsieh, S, Chavada, G, Addington, J, Ajroud-Driss, S, Andersen, H, Antonini, G, Ariatti, A, Attarian, S, Badrising, U, Barroso, F, Benedetti, L, Beronio, A, Bianco, M, Binda, D, Briani, C, Bunschoten, C, Burmann, J, Bella, I, Bertorini, T, Bhavaraju-Sanka, R, Brannagan, T, Busby, M, Butterworth, S, Casasnovas, C, Cavaletti, G, Chao, C, Chen, S, Chetty, S, Claeys, K, Conti, M, Cosgrove, J, Dalakas, M, Demichelis, C, Derejko, M, Dillmann, U, Dimachkie, M, Doppler, K, Dornonville de la Cour, C, Echaniz-Laguna, A, Eftimov, F, Faber, C, Fazio, R, Fokke, C, Fujioka, T, Fulgenzi, E, Garcia-Sobrino, T, Garssen, M, Georgios, H, Gijsbers, C, Gilchrist, J, Gilhuis, J, Giorli, E, Goldstein, J, Goyal, N, Granit, V, Grapperon, A, Gutierrez, G, Hadden, R, Holbech, J, Holt, J, Pedret, C, Htut, M, Jellema, K, Pascual, I, Jimeno-Montero, M, Kaida, K, Karafiath, S, Katzberg, H, Kiers, L, Kieseier, B, Kimpinski, K, Kleyweg, R, Kokubun, N, Kolb, N, Kuitwaard, K, Kuwabara, S, Kwan, J, Ladha, S, Lassen, L, Lawson, V, Ledingham, D, Lucy, S, Lunn, M, Magot, A, Manji, H, Marchesoni, C, Marfia, G, Infante, C, Hernandez, E, Mataluni, G, Mattiazi, M, Mcdermott, C, Meekins, G, Miller, J, Moris de la Tassa, G, Physiotherapist, J, Nascimbene, C, Nowak, R, Balaguer, P, Osei-Bonsu, M, Pan, E, Pardal, A, Pardo, J, Pasnoor, M, Pulley, M, Rajabally, Y, Rinaldi, S, Ritter, C, Roberts, R, Rojas-Marcos, I, Rudnicki, S, Ruiz, M, Sachs, G, Samijn, J, Santoro, L, Savransky, A, Schenone, A, Schwindling, L, Tous, M, Sekiguchi, Y, Sheikh, K, Silvestri, N, Sindrup, S, Sommer, C, Stein, B, Stino, A, Spyropoulos, A, Srinivasan, J, Styliani, R, Suzuki, H, Tankisi, H, Tigner, D, Twydell, P, Van Damme, P, Van der Kooi, A, Van Dijk, G, Van der Ree, T, Van Koningsveld, R, Valzania, F, Varrato, J, Vermeij, F, Verschuuren, J, Visser, L, Vytopil, M, Wilken, M, Wilkerson, C, Wirtz, P, Yamagishi, Y, Zhou, L, and Zivkovic, S

Subjects
Adult, medicine.medical_specialty, Adolescent, Patient characteristics, PLASMA-EXCHANGE, 030204 cardiovascular system & hematology, Guillain-Barre Syndrome, Settore MED/26, Severity of Illness Index, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Severity of illness, Medicine, Effective treatment, INTRAVENOUS IMMUNOGLOBULIN, Humans, Prospective Studies, Child, Child, Preschool, Treatment Outcome, Prospective cohort study, Preschool, Guillain-Barre syndrome, business.industry, medicine.disease, RANDOMIZED-TRIAL, Prospective Studie, Hospital treatment, Treatment practice, Observational study, Neurology (clinical), business, 030217 neurology & neurosurgery, Human
Abstract

ObjectiveTo define the current treatment practice of Guillain-Barré syndrome (GBS).MethodsThe study was based on prospective observational data from the first 1,300 patients included in the International GBS Outcome Study. We described the treatment practice of GBS in general, and for (1) severe forms (unable to walk independently), (2) no recovery after initial treatment, (3) treatment-related fluctuations, (4) mild forms (able to walk independently), and (5) variant forms including Miller Fisher syndrome, taking patient characteristics and hospital type into account.ResultsWe excluded 88 (7%) patients because of missing data, protocol violation, or alternative diagnosis. Patients from Bangladesh (n = 189, 15%) were described separately because 83% were not treated. IV immunoglobulin (IVIg), plasma exchange (PE), or other immunotherapy was provided in 941 (92%) of the remaining 1,023 patients, including patients with severe GBS (724/743, 97%), mild GBS (126/168, 75%), Miller Fisher syndrome (53/70, 76%), and other variants (33/40, 83%). Of 235 (32%) patients who did not improve after their initial treatment, 82 (35%) received a second immune modulatory treatment. A treatment-related fluctuation was observed in 53 (5%) of 1,023 patients, of whom 36 (68%) were re-treated with IVIg or PE.ConclusionsIn current practice, patients with mild and variant forms of GBS, or with treatment-related fluctuations and treatment failures, are frequently treated, even in absence of trial data to support this choice. The variability in treatment practice can be explained in part by the lack of evidence and guidelines for effective treatment in these situations.

Published
2019

12. Second IVIg course in Guillain-Barré syndrome with poor prognosis. The non-randomised ISID study

Author

Verboon, C., Van Den Berg, B., Cornblath, D. R., Venema, E., Gorson, K. C., Lunn, M. P., Lingsma, H., Van Den Bergh, P., Harbo, T., Bateman, K., Pereon, Y., Sindrup, So. H., Kusunoki, S., Miller, J., Islam, Z., Hartung, H. -P., Chavada, G., Jacobs, B. C., Hughes, R. A. C., Addington, Van Doorn P. A. J. M., MD (University of Virginia, Charlottesville, USA), on October 7, S. Consortia. Protected by copyright., Downloaded from 8 Verboon C, 2019 at Uppsala Universitet BIBSAM http://jnnp. bmj. com/ J Neurol Neurosurg Psychiatry: first published as 10. 1136/jnnp-2019-321496 on 5 October 2019., J Neurol Neurosurg Psychiatry 2019, et al., 1136/jnnp-2019-321496 Neuromuscular Ajroud-Driss, 0:1–9. doi:10., MD (Northwestern University Feinberg, Chicago, USA), Antonini, G., MD (Mental Health and Sensory Organs (NESMOS), Sapienza, University, Sant’Andrea, Hospital, Rome, Italy), Attarian, S., PhD (CHU Timone, Marseille, France), Barroso, F. A., MD (Instituto de Investigaciones Neurológicas Raúl Carrea, Fleni, Buenos, Aires, Argentina), Benedetti, L., PhD (Ospedale Sant’ Andrea La Spezia, Spezia, La, Italy), Bertorini, T. E., MD (The University of Tennessee Health Science Center (UTHSC), Memphis, USA), Brannagan, T. H., MD (Columbia University, New York City, USA), Briani, C., MD (University of Padova, Padova, Italy), Bhavaraju-Sanka, R., MD (University Hospital/University of Texas Health Science Center, San Antonio Texas, Butterworth, S., MD (Pinderfields Hospital, Wakefield, UK), Casasnovas, C., Ciberer, PhD (Bellvitge University Hospital – IDIBELL Neurometabolic Diseases Group., Barcelona, Spain), Cavaletti, G., MD (University Milano-Bicocca, Monza, Italy), Chen, S., Phd, (Rutgers, Robert Wood Johnson University Hospital, New, Brunswick, Claeys, K. G., University Hospitals Leuven, PhD (1., Leuven, Belgium, KU Leuven, 2., Leuven, Belgium), Cosgrove, J. S., MD (Leeds General Infirmary, Leeds, UK), Davidson, A., MD (University of Glasgow, Glasgow, UK), Dardiotis, E., MD (University of Thessaly, Hospital of Larissa, Larissa, Greece), Dornonville de la Cour, C., MD (National Hospital Copenhagen, Copenhagen, Denmark), Faber, C. G., PhD (Maastricht University Medical Centre, Maastricht, The, Netherlands), Feasby, T. E., MD (University of Calgary, Calgary, Canada), Fujioka, T., MD (Toho University Medical Center, Tokyo, Japan), Galassi, G., MD (University Hospital of Modena, Modena, Italy), Gilchrist, J. M., MD (Soulthern Illinois University School of Medicine, Springfield, USA), Goyal, N. A., MD (University of California, Irvine, USA), Granit, V., MD (Montefiore Medical, Center, New, York, Gutiérrez-Gutiérrez, G., MD (Hospital Universitario Infanta Sofia, San, Sebastian, Spain), Hadden, R. D. M., PhD (King’s College Hospital, London, UK), Holt, J. K. L., Phd, FRCP (The Walton Centre, Liverpool, UK), Htut, M., George’s Hospital, MD (St., Jericó Pascual, I., PhD (Complejo Hospitalario de Navarra, Pamplona, Spain), Karafiath, S., MD (University of Utah School of Medicine, Salt Lake City, Katzberg, H. D., MD (University of Toronto, Toronto, Canada), Kiers, L., MD (University of Melbourne, Royal Melbourne Hospital, Parkville, Australia), Kieseier, B. C., MD (Heinrich Heine University, Düsseldorf, Germany), Kimpinski, K., MD (University Hospital, Lhsc, London-Ontario, Canada), Kuwabara, S., PhD (Chiba University, Chiba, Japan), Kwan, J. Y., MD (University of Maryland School of Medicine, Baltimore, USA), Ladha, S. S., MD (Barrow Neurology Clinics, Phoenix, Arizona, Lawson, V., MD (Wexner Medical Center at The Ohio State University, Columbus, USA), Lehmann, H., PhD (University Hospital of Cologne, Universitätsklinikum, Köln, Cologne, Germany), Manji, H., FRCP (Ipswich Hospital, Ipswich, UK), Marfia, G. A., MD (Neurological Clinic, Policlinico Tor Vergata, Márquez Infante, C., MD (Hospital Universitario Virgen del Rocio, Seville, Spain), Mattiazzi, M. G., MD (Hospital Militar Central, Mcdermott, C. J., MD (Royal Hallamshire Hospital, Nihr, Clinical, Sheffield, UK), Monges, M. S., Garrahan, MD (Hospital de Pediatría J. P., Morís de la Tassa, G., MD (Hospital Universitario Central de Asturias, Asturias, Spain), Nascimbene, C., PhD (Luigi Sacco Hospital, Milan, Italy), Nobile Orazio, E., PhD (Milan University, Humanitas Clinicala and Research Institute Milan, Nowak, R. J., MD (Yale University School of Medicine, New, Haven, Osei-Bonsu (James Cook University Hospital, M., Middlesbrough, UK), Pardo Fernandez (Hospital Clínico de Santiago, J., Santiago de Compostela (A Coruña), Querol Gutierrez, L., PhD (Hospital de la Santa Creu, i Sant Pau, Universitat Autònoma de Barcelona, Reisin (Hospital Britanico, R., Rinaldi, S., Mbchb, Roberts, PhD (University of Oxford R. C., MD (Addenbrooke’s Hospital Cambridge, Cambridge, UK), Rojas-Marcos, I., MD (Hospital Univesitario Reina Sofia, Cordoba, Spain), Rudnicki, S. A., MD (University of Arkansas, Fayetteville, USA), Schenone, A., Department of Neurosciences, PhD (1., Rehabilitation, Ophthalmology, Genetics and Maternal and Infantile Sciences (DINOGMI), University of Genova, Genova, IRCCS Policlinico San Martino, Italy 2., Genova, Italy), Sedano Tous, M. J., MD (Hospital Universitario Marques de Valdecilla, Santander, Cantabria, Shahrizaila, N., MD (Neurology Unit, Department of Medicine, Faculty of Medicine, University of Malaya, Malaya), Sheikh, K., PhD (The University of Texas Health Science Center at Houston, Houston, USA), Silvestri, N. J., MD (Buffalo General Medical Center, Buffalo, Ny, Sommer, C. L., MD (Universitätsklinikum Würzburg, Würzburg, Germany), Varrato, J. D., DO (Lehigh Valley Health Network, Allentown, USA), Verschuuren, J., PhD (Leiden University Medical Centre, Leiden, Vytopil, M. V., Waheed, PhD (Tufts University School of Medicine Lahey Hospital W., MD (University of Vermont Medical Center, Burlington, USA), Zhou, L., PhD (Icahn School of Medicine at Mount Sinai, Badrising, USA). Other collaborators were:U. A., Bella, I. R., MD (University of Mass Medical School, Worcester, USA), Bunschoten, C., PhD candidate (Erasmus University Medical Centre, Rotterdam, Bürmann, J., Universitätsklinikum des Saarlandes, Homburg, Germany), Busby, M., Bradford, UK), Chao, C. C., PhD (National Taiwan University Hospital, Taipei, Taiwan), Conti, M. E., MD (University Hospital Clinicas, Dalakas, M. C., Thomas Jefferson University, MD (1., Philadelphia, Usa, National and Kapodistrian University of Athens, 2., Athens, Greece), Van Damme, P., PhD (University Hospital Leuven, Doets, A., van Dijk, G. W., MD (Canisius Wilhelmina Hospital, Nijmegen, Dimachkie, M. M., MD (University of Kansas Medical Center, Kansas, City, Doppler, K., Echaniz-Laguna, A., MD (Hopital de Hautepierre, Strasbourgh, France), Eftimov, F., PhD (Amsterdam University Medical Centre, Amsterdam, Fazio, R., MD (Scientific Institute San Raffaele, Fokke, C., MD (Gelre Hospital, Zutphen and Apeldoorn, Fulgenzi, E. A., MD (Hospital Cesar Milstein Buenos Aires, Garssen, M. P. J., PhD (Jeroen Bosch Hospital, Hertogenbosch, ’S, Zaltbommel and Drunen, Gijsbers, C. J., MD (Vlietland Hospital, Schiedam, Gilhuis, J., PhD (Reinier de Graaf Gasthuis, Delft, Grapperon, A., MD (CHU Timone, Hsieh, S. T., Illa, I., Islam, B., PhD (International Centre for Diarrhoeal Disease Research, Bangladesh, (icddr, Dhaka, b), Bangladesh), Jellema, K., PhD (Haaglanden Medisch Centrum, The, Hague, Kaida, K., PhD (National Defense Medical College, Saitama, Japan), Kokubun, N., MD (Dokkyo Medical University, Tochigi, Japan), Kolb, N., MD (University of Vermont, Burlington, Vt, van Koningsveld, R., PhD (Elkerliek Hospital, Helmond and Deurne, van der Kooi, A. J., Kuitwaard, K., PhD (Albert Schweitzer Hospital, Dordrecht, Landschoff Lassen, L., MD (Glostrup Hospital, Glostrup, Denmark), Leonhard, S. E., Mandarakas, M., PhD (Erasmus University Medical Centre, Martinez Hernandez, E., MD (Institut d’Investigacions Biomèdiques August Pi, i Sunyer (IDIBAPS), Hospital, Clinic, Mohammad, Q. D., PhD (National Institute of Neurosciences and Hospital, Dhaka, Bangladesh), Pulley, M., MD (University of Florida, Jacksonville, USA), Rajabally, Y. A., PhD (Queen Elizabeth Hospital, Birmingham, UK), Reddel, S. W., PhD (Concord Repatriation General Hospital, Sydney, Australia), van der Ree, T., (Westfriesgasthuis, Md, Hoorn, Roodbol, J., Sachs, G. M., MD (University of Rhode Island, Providence, USA), Samijn, J. P. A., PhD (Maasstad Hospital, Santoro, L., PhD (University Federico II, Napels, Italy), Stein, B., Joseph’s Regional Medical Center, MD (St., Paterson, USA), Vermeij, F. H., MD (Franciscus Gasthuis, Visser, L. H., PhD (Elisabeth-TweeSteden Hospital, Tilburg and Waalwijk, Willison, H. J., PhD (University of Glasgow, Wirtz, P., Phd, (HagaZiekenhuis, Zivkovich, S. A., PhD (University of Pittsburgh Medical Center, and Pittsburgh, USA).

Subjects
treatment, disability evaluation, drug administration schedule, adult, guillain-barré syndrome, poor prognosis, second ivig course, aged, female, guillain-barre syndrome, humans, immunoglobulin g, immunoglobulins, intravenous, immunologic factors, male, middle aged, prognosis, time factors, treatment outcome
Published
2020

13. Identification of serum microRNAs as potential biomarkers in Pompe disease

Author

Carrasco-Rozas, A, Fernandez-Simon, E, Lleixa, MC, Belmonte, I, Pedrosa-Hernandez, I, Montiel-Morillo, E, Nunez-Peralta, C, Rossello, JL, Segovia, S, De Luna, N, Suarez-Calvet, X, Illa, I, Diaz-Manera, J, Gallardo, E, Barba-Romero, MA, Barcena, J, Carzorla, MR, Creus, C, Coll-Canti, J, Diaz, M, Dominguez, C, Torron, RF, Antelo, MJG, Grau, JM, Caravaca, MTG, Hernandez, JCL, de Munain, AL, Martinez-Garcia, FA, Morgado, Y, Moreno, A, Moris, G, Munoz-Blanco, MA, Nascimento, A, Paradas, C, Pozo, JLP, Querol, L, Robledo-Strauss, A, Garcia, RR, Rojas-Marcos, I, Salazar, JA, and Uson, M

Abstract

Objective To analyze the microRNA profile in serum of patients with Adult Onset Pompe disease (AOPD). Methods We analyzed the expression of 185 microRNAs in serum of 15 AOPD patients and five controls using microRNA PCR Panels. The expression levels of microRNAs that were deregulated were further studied in 35 AOPD patients and 10 controls using Real-Time PCR. Additionally, the skeletal muscle expression of microRNAs which showed significant increase levels in serum samples was also studied. Correlations between microRNA serum levels and muscle function test, spirometry, and quantitative muscle MRI were performed (these data correspond to the study NCT01914536 at ClinicalTrials.gov). Results We identified 14 microRNAs that showed different expression levels in serum samples of AOPD patients compared to controls. We validated these results in a larger cohort of patients and we found increased levels of three microRNAs, the so called dystromirs: miR-1-3p, miR-133a-3p, and miR-206. These microRNAs are involved in muscle regeneration and the expression of these was increased in patients' muscle biopsies. Significant correlations between microRNA levels and muscle function test were found. Interpretation Serum expression levels of dystromirs may represent additional biomarkers for the follow-up of AOPD patients.

Published
2019

14. Regional variation of Guillain-Barré syndrome

Author

Doets, Alex Y., Verboon, Christine, Van Den Berg, Bianca, Harbo, Thomas, Cornblath, David R., Willison, Hugh J., Islam, Zhahirul, Attarian, Shahram, Barroso, Fabio A., Bateman, Kathleen, Benedetti, Luana, Van Den Bergh, Peter, Casasnovas, Carlos, Cavaletti, Guido, Chavada, Govindsinh, Claeys, Kristl G., Dardiotis, Efthimios, Davidson, Amy, Van Doorn, Pieter A., Feasby, Tom E., Galassi, Giuliana, Gorson, Kenneth C., Hartung, Hans-Peter, Hsieh, Sung-Tsang, Hughes, Richard A. C., Illa, Isabel, Islam, Badrul, Kusunoki, Susumu, Kuwabara, Satoshi, Lehmann, Helmar C., Miller, James A. L., Mohammad, Quazi Deen, Monges, Soledad, Nobile Orazio, Eduardo, Pardo, Julio, Pereon, Yann, Rinaldi, Simon, Querol, Luis, Reddel, Stephen W., Reisin, Ricardo C., Shahrizaila, Nortina, Sindrup, Soren H., Waqar, Waheed, Jacobs, Bart C., Jacobs, Bc, Hughes, Rac, Cornblath, Dr, Gorson, Kc, Hartung, Hp, Kusunoki, S, van Doorn PA, Willison, Hj, van Woerkom, M, van den Berg, B, Verboon, C, Doets, Ay, Roodbol, J, Reisin, Rc, Reddel, Sw, Islam, Z, Islam, B, Mohammad, Qd, van den Bergh, P, Feasby, Te, Harbo, T, Péréon, Y, Lehmann, Hc, Dardiotis, E, Nobile-Orazio, E, Shahrizaila, N, Bateman, K, Illa, I, Querol, L, Hsieh, St, Chavada, G, Davidson, A, Addington, Jm, Ajroud-Driss, S, Andersen, H, Antonini, G, Ariatti, A, Attarian, S, Badrising, Ua, Barroso, Fa, Benedetti, L, Beronio, A, Bianco, M, Binda, D, Briani, C, Bunschoten, C, Bürmann, J, Bella, Ir, Bertorini, Te, Bhavaraju-Sanka, R, Brannagan, Th, Busby, M, Butterworth, S, Casasnovas, C, Cavaletti, G, Chao, Cc, Chetty, S, Claeys, Kg, Conti, Me, Cosgrove, Js, Dalakas, Mc, Derejko, Ma, Dimachkie, Mm, Doppler, K, Dornonville de la Cour, C, Echaniz-Laguna, A, Eftimov, F, Faber, Cg, Fazio, R, Fujioka, T, Fulgenzi, Ea, Galassi, G, Garcia-Sobrino, T, Garnero, M, Garssen, Mpj, Gijsbers, Cj, Gilchrist, Jm, Goldstein, Jm, Granit, V, Grapperon, A, Gutiérrez, G, Hadden, Rdm, Holbech, Jv, Holt, Jkl, Homedes Pedret, C, Htut, M, Jericó Pascual, I, Kaida, K, Karafiath, S, Katzberg, Hd, Kiers, L, Kieseier, Bc, Kimpinski, K, Kleyweg, Rp, Kokubun, N, Kolb, Na, Kuitwaard, K, Kuwabara, S, Kwan, Jy, Ladha, Ss, Landschoff Lassen, L, Lawson, V, Ledingham, D, Léon Cejas, L, Lucy, St, Lunn, Mpt, Magot, A, Manji, H, Marchesoni, C, Marfia, Ga, Márquez Infante, C, Martinez Hernandez, E, Mataluni, G, Mcdermott, Cj, Meekins, Gd, Miller, Jal, Monges, Ms, Montero, Mcj, Morís de la Tassa, G, Mozzoni, J, Nascimbene, C, Nowak, Rj, Orizaloa Balaguer, P, Osei-Bonsu, M, Lee Pan EB, Pardo, J, Pasnoor, M, Rajabally, Ya, Rinaldi, S, Ritter, C, Roberts, Rc, Rojas-Marcos, I, Rudnicki, Sa, Ruiz, M, Sachs, Gm, Samijn, Jpa, Santoro, L, Schenone, A, Schwindling, L, Sedano Tous MJ, Sekiguchi, Y, Sheikh, Ka, Silvestri, Nj, Sindrup, Sh, Sommer, Cl, Stein, B, Stino, Am, Spyropoulos, A, Srinivasan, J, Suzuki, H, Tankisi, H, Tigner, D, Twydell, Pt, van Damme, P, van der Kooi AJ, van Dijk GW, van der Ree, T, van Koningsveld, R, Varrato, Jd, Vermeij, Fh, Visser, Lh, Vytopil, Mv, Waheed, W, Wilken, M, Wilkerson, C, Wirtz, Pw, Yamagishi, Y, Zhou, L, Zivkovic, S., Doets, A, Verboon, C, van den Berg, B, Harbo, T, Cornblath, D, Willison, H, Islam, Z, Attarian, S, Barroso, F, Bateman, K, Benedetti, L, van den Bergh, P, Casasnovas, C, Cavaletti, G, Chavada, G, Claeys, K, Dardiotis, E, Davidson, A, van Doorn, P, Feasby, T, Galassi, G, Gorson, K, Hartung, H, Hsieh, S, Hughes, R, Illa, I, Islam, B, Kusunoki, S, Kuwabara, S, Lehmann, H, Miller, J, Mohammad, Q, Monges, S, Nobile Orazio, E, Pardo, J, Pereon, Y, Rinaldi, S, Querol, L, Reddel, S, Reisin, R, Shahrizaila, N, Sindrup, S, Waqar, W, Jacobs, B, Neurology, AII - Infectious diseases, AII - Inflammatory diseases, ANS - Neuroinfection & -inflammation, Immunology, UCL - SSS/IONS/NEUR - Clinical Neuroscience, and UCL - (SLuc) Service de neurologie

Subjects
Adult, Male, medicine.medical_specialty, Adolescent, clinical course, Guillain-Barre Syndrome, 03 medical and health sciences, Young Adult, 0302 clinical medicine, axonal degeneration, demyelination, outcome, polyradiculoneuropathy, Internal medicine, Severity of illness, medicine, Humans, 030212 general & internal medicine, Young adult, Child, Geographic difference, Aged, Aged, 80 and over, Guillain-Barre syndrome, Polyradiculoneuropathy, Overlap syndrome, Middle Aged, medicine.disease, Regional variation, Child, Preschool, neurology, Settore MED/26 - Neurologia, Female, Neurology (clinical), 030217 neurology & neurosurgery, Cohort study, polyradiculoneuropathy, demyelination, axonal degeneration, clinical course, outcome
Abstract

Guillain-Barré syndrome is a heterogeneous disorder regarding the clinical presentation, electrophysiological subtype and outcome. Previous single country reports indicate that Guillain-Barré syndrome may differ among regions, but no systematic comparative studies have been conducted. Comparative studies are required to identify factors determining disease susceptibility, variation and prognosis, and to improve diagnostic criteria. The International Guillain-Barré Syndrome Outcome Study is a prospective, observational cohort study including all patients within the diagnostic spectrum, aiming to describe the heterogeneity of Guillain-Barré syndrome worldwide. The current study was based on the first 1000 inclusions with a follow-up of at least 1 year and confirmed the variation in clinical presentation, course and outcome between patients. The full clinical spectrum of Guillain-Barré syndrome was observed in patients from all countries participating in the International Guillain-Barré Syndrome Outcome Study, but the frequency of variants differed between regions. We compared three regions based on geography, income and previous reports of Guillain-Barré syndrome subtypes: 'Europe/Americas', 'Asia' (without Bangladesh), and 'Bangladesh'. We excluded 75 (8%) patients because of alternative diagnoses, protocol violations, or missing data. The predominant clinical variant was sensorimotor in Europe/Americas (n = 387/562, 69%) and Asia (n = 27/63, 43%), and pure motor in Bangladesh (n = 74/107, 69%). Miller Fisher syndrome and Miller Fisher-Guillain-Barré overlap syndrome were more common in Asia (n = 14/63, 22%) than in the other two regions (Europe/Americas: n = 64/562, 11%; Bangladesh: n = 1/107, 1%) (P < 0.001). The predominant electrophysiological subtype was demyelinating in all regions (Europe/Americas: n = 312/573, 55%; Asia: n = 29/65, 45%; Bangladesh: n = 38/94, 40%). The axonal subtype occurred more often in Bangladesh (n = 34/94, 36%) than in Europe/Americas (n = 33/573, 6%) and other Asian countries (n = 4/65, 6%) (P < 0.001). In all regions, patients with the axonal subtype were younger, had fewer sensory deficits, and showed a trend towards poorer recovery compared to patients with the demyelinating subtype. The proportion of patients able to walk unaided after 1 year varied between Asia (n = 31/34, 91%), Europe/Americas (n = 334/404, 83%) and Bangladesh (n = 67/97, 69%) (P = 0.003). A similar variation was seen for mortality, being higher in Bangladesh (n = 19/114, 17%) than in Europe/Americas (n = 23/486, 5%) and Asia (n = 1/45, 2%) (P < 0.001). This study showed that factors related to geography have a major influence on clinical phenotype, disease severity, electrophysiological subtype, and outcome of Guillain-Barré syndrome.

Published
2018

15. 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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16. International Guillain-Barré Syndrome Outcome Study: protocol of a prospective observational cohort study on clinical and biological predictors of disease course and outcome in Guillain-Barré syndrome

Author

Jacobs, B.C. (Bart C.), Berg, B. (Bianca) van den, Verboon, C. (Christine), Chavada, G. (Govindsinh), Cornblath, D.R. (David), Gorson, K.C. (Kenneth), Harbo, T. (Thomas), Hartung, H.P., Hughes, R.A.C. (Richard A. C.), Kusunoki, S. (Susumu), Doorn, P.A. (Pieter) van, Willison, H.J. (Hugh J.), Jacobs, B.C. (Bart), Hughes, R.A.C. (Richard), Cornblath, D.R., Gorson, K.C., Kusunoki, S., Willison, H.J. (Hugh), van Woerkom, M., van den Berg, B., Verboon, C., Roodbol, J. (J.), Reisin, R. (R.), Reddel, S.W., Islam, Z. (Zhahirul), Islam, B., Mohammad, Q.D. (Quazi), Bergh, P.Y.K. (Paul) van den, Feasby, T.E., Wang, Y.Z., Harbo, T., Péréon, Y., Lehmann, H.C., Dardiotis, E., Nobile-Orazio, E. (Eduarde), Shahrizaila, N., Jacobs, B.C., Bateman, K., Illa, I. (Isabel), Querol, L. (Luis), Hsieh, S.T., Willison, H.J., Chavada, G., Davidson, A., Addington, J.M., Ajroud-Driss, S., Andersen, H., Antonini, G., Attarian, S., Badrising, U.A. (Umesh), Barroso, F.A., Benedetti, L., Beronio, A., Bianco, M., Binda, D., Briani, C., Bürmann, J., Bella, I.R., Bertorini, T.E., Bhavaraju-Sanka, R., Brannagan, T.H., Busby, M., Butterworth, S., Campagnolo, M., Casasnovas, C., Cavaletti, G., Chao, C.S., Chen, S., Chetty, S., Claeys, T. (Tine), Cohen, J.A., Conti, M.E., Cosgrove, J.S., Dalakas, M.C. (Marinos), Dimachkie, M.M., Dillmann, U., Domínguez González, C., Doppler, K., Dornonville de la Cour, C., Echaniz-Laguna, A., Eftimov, F. (Filip), Faber, C.G., Fazio, R. (R.), Fokke, M. (Maureen), Fujioka, T., Fulgenzi, E. (E.), Galassi, G., Garcia, T., Garnero, M., Garssen, M.P.J. (Marcel), Gijsbers, C.J., Gilchrist, J.M., Gilhuis, J. (Job), Goldstein, J.M., Goyal, N., Granit, V., Grapperon, A., Gutiérrez Gutiérrez, G., Gutmann, L., Hadden, R.D.M. (Rob), Holbech, J.V., Holt, J.K.L., Homedes Pedret, C., Htut, M., Jellema, K., Jericó Pascual, I., Kaida, K.I. (Ken Ichi), Karafiath, S., Katzberg, H. (Hans), Kiers, H.A.L. (Henk), Kieseier, B.C. (Bernd), Kimpinski, K., Kleyweg, R.P., Kokubun, N., Kolb, N.A., Kuitwaard, K. (Krista), Kuwabara, S., Kwan, J.Y., Ladha, S.S., Landschoff Lassen, L., Lawson, V., Ledingham, D., Léon Cejas, L., Luciano, C.A., Lucy, S.T., Lunn, M.P.T. (Michael P. T.), Magot, A., Manji, H., Marchesoni, A., Marfia, G.A.M., Márquez Infante, C., Martinez Hernandez, E., Mataluni, G., Mattiazi, M., McDermott, C.J., Meekins, G.D., Miller, J., Monges, M.S., Montero, M.C.J., Morís de la Tassa, G., Nascimbene, C., Neumann, C., Nowak, R.J., Orizaola Balaguer, P., Osei-Bonsu, M., Pan, E.B.L., Pardo Fernandez, J., Pasnoor, M., Pulley, M.T., Rajabally, Y.A., Rinaldi, S. (Sabina), Ritter, C., Roberts, R.C., Rojas-Marcos, I., Rudnicki, S.A., Sachs, G.M., Samijn, J.P. (Johnny), Santoro, L., Saperstein, D.S., Savransky, A., Schneider, H., Schenone, A. (Andrea), Sedano Tous, M.J., Sekiguchi, Y., Sheikh, K.A., Silvestri, N.J., Sindrup, S.H., Sommer, C., Stein, B., Stino, A.M., Spyropoulos, A., Srinivasan, J., Suzuki, H., Taylor, S.W., Tankisi, H., Tigner, D., Twydell, P.T., Valzania, F., van Damme, P., Kooj, A.J. (Anneke), Dijk, G.W. (Gert) van, van der Ree, T., Koningsveld, R. (Rinske) van, Varrato, J.D., Vermeij, F.H. (Frederique), Verschuuren, J.J. (Jan), Visser, L.H. (Leendert), Vytopil, M.V., Waheed, W., Wilken, M., Wilkerson, C., Wirtz, P.W., Yamagishi, Y., Yiu, E.M., Zhou, L., Zivkovic, S.A. (Sasa), Jacobs, B.C. (Bart C.), Berg, B. (Bianca) van den, Verboon, C. (Christine), Chavada, G. (Govindsinh), Cornblath, D.R. (David), Gorson, K.C. (Kenneth), Harbo, T. (Thomas), Hartung, H.P., Hughes, R.A.C. (Richard A. C.), Kusunoki, S. (Susumu), Doorn, P.A. (Pieter) van, Willison, H.J. (Hugh J.), Jacobs, B.C. (Bart), Hughes, R.A.C. (Richard), Cornblath, D.R., Gorson, K.C., Kusunoki, S., Willison, H.J. (Hugh), van Woerkom, M., van den Berg, B., Verboon, C., Roodbol, J. (J.), Reisin, R. (R.), Reddel, S.W., Islam, Z. (Zhahirul), Islam, B., Mohammad, Q.D. (Quazi), Bergh, P.Y.K. (Paul) van den, Feasby, T.E., Wang, Y.Z., Harbo, T., Péréon, Y., Lehmann, H.C., Dardiotis, E., Nobile-Orazio, E. (Eduarde), Shahrizaila, N., Jacobs, B.C., Bateman, K., Illa, I. (Isabel), Querol, L. (Luis), Hsieh, S.T., Willison, H.J., Chavada, G., Davidson, A., Addington, J.M., Ajroud-Driss, S., Andersen, H., Antonini, G., Attarian, S., Badrising, U.A. (Umesh), Barroso, F.A., Benedetti, L., Beronio, A., Bianco, M., Binda, D., Briani, C., Bürmann, J., Bella, I.R., Bertorini, T.E., Bhavaraju-Sanka, R., Brannagan, T.H., Busby, M., Butterworth, S., Campagnolo, M., Casasnovas, C., Cavaletti, G., Chao, C.S., Chen, S., Chetty, S., Claeys, T. (Tine), Cohen, J.A., Conti, M.E., Cosgrove, J.S., Dalakas, M.C. (Marinos), Dimachkie, M.M., Dillmann, U., Domínguez González, C., Doppler, K., Dornonville de la Cour, C., Echaniz-Laguna, A., Eftimov, F. (Filip), Faber, C.G., Fazio, R. (R.), Fokke, M. (Maureen), Fujioka, T., Fulgenzi, E. (E.), Galassi, G., Garcia, T., Garnero, M., Garssen, M.P.J. (Marcel), Gijsbers, C.J., Gilchrist, J.M., Gilhuis, J. (Job), Goldstein, J.M., Goyal, N., Granit, V., Grapperon, A., Gutiérrez Gutiérrez, G., Gutmann, L., Hadden, R.D.M. (Rob), Holbech, J.V., Holt, J.K.L., Homedes Pedret, C., Htut, M., Jellema, K., Jericó Pascual, I., Kaida, K.I. (Ken Ichi), Karafiath, S., Katzberg, H. (Hans), Kiers, H.A.L. (Henk), Kieseier, B.C. (Bernd), Kimpinski, K., Kleyweg, R.P., Kokubun, N., Kolb, N.A., Kuitwaard, K. (Krista), Kuwabara, S., Kwan, J.Y., Ladha, S.S., Landschoff Lassen, L., Lawson, V., Ledingham, D., Léon Cejas, L., Luciano, C.A., Lucy, S.T., Lunn, M.P.T. (Michael P. T.), Magot, A., Manji, H., Marchesoni, A., Marfia, G.A.M., Márquez Infante, C., Martinez Hernandez, E., Mataluni, G., Mattiazi, M., McDermott, C.J., Meekins, G.D., Miller, J., Monges, M.S., Montero, M.C.J., Morís de la Tassa, G., Nascimbene, C., Neumann, C., Nowak, R.J., Orizaola Balaguer, P., Osei-Bonsu, M., Pan, E.B.L., Pardo Fernandez, J., Pasnoor, M., Pulley, M.T., Rajabally, Y.A., Rinaldi, S. (Sabina), Ritter, C., Roberts, R.C., Rojas-Marcos, I., Rudnicki, S.A., Sachs, G.M., Samijn, J.P. (Johnny), Santoro, L., Saperstein, D.S., Savransky, A., Schneider, H., Schenone, A. (Andrea), Sedano Tous, M.J., Sekiguchi, Y., Sheikh, K.A., Silvestri, N.J., Sindrup, S.H., Sommer, C., Stein, B., Stino, A.M., Spyropoulos, A., Srinivasan, J., Suzuki, H., Taylor, S.W., Tankisi, H., Tigner, D., Twydell, P.T., Valzania, F., van Damme, P., Kooj, A.J. (Anneke), Dijk, G.W. (Gert) van, van der Ree, T., Koningsveld, R. (Rinske) van, Varrato, J.D., Vermeij, F.H. (Frederique), Verschuuren, J.J. (Jan), Visser, L.H. (Leendert), Vytopil, M.V., Waheed, W., Wilken, M., Wilkerson, C., Wirtz, P.W., Yamagishi, Y., Yiu, E.M., Zhou, L., and Zivkovic, S.A. (Sasa)

Abstract

Guillain-Barré syndrome (GBS) is an acute polyradiculoneuropathy with a highly variable clinical presentation, course, and outcome. The factors that determine the clinical variation of GBS are poorly understood which complicates the care and treatment of individual patients. The protocol of the ongoing International GBS Outcome Study (IGOS), a prospective, observational, multicenter cohort study that aims to identify the clinical and biological determinants and predictors of disease onset, subtype, course and outcome of GBS is presented here. Patients fulfilling the diagnostic criteria for GBS, regardless of age, disease severity, variant forms, or treatment, can participate if included within 2 weeks after onset of weakness. Information about demography, preceding infections, clinical features, diagnostic findings, treatment, course, and outcome is collected. In addition, cerebrospinal fluid and serial blood samples for serum and DNA is collected at standard time points. The original aim was to include at least 1,000 patients with a follow-up of 1–3 years. Data are collected via a web-based data entry system and stored anonymously. IGOS started in May 2012 and by January 2017 included more than 1,400 participants from 143 active centers in 19 countries across 5 continents. The IGOS data/biobank is available for research projects conducted by expertise groups focusing on specific topics including epidemiology, diagnostic criteria, clinimetrics, electrophysiology, antecedent events, antibodies, genetics, prognostic modeling, treatment effects, and long-term outcome of GBS. The IGOS will help to standardize the international collection of data and biosamples for future research of GBS.

Published
2017
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17. International Guillain-Barré Syndrome Outcome Study: protocol of a prospective observational cohort study on clinical and biological predictors of disease course and outcome in Guillain-Barré syndrome

Author

Jacobs, B, van den Berg, B, Verboon, C, Chavada, G, Cornblath, D, Gorson, K, Harbo, T, Hartung, H, Hughes, R, Kusunoki, S, van Doorn, P, Willison, H, van Woerkom, M, Roodbol, J, Reisin, R, Reddel, S, Islam, Z, Islam, B, Mohammad, Q, van den Bergh, P, Feasby, T, Wang, Y, Pã©rã©on, Y, Lehmann, H, Dardiotis, E, Nobile Orazio, E, Shahrizaila, N, Bateman, K, Illa, I, Querol, L, Hsieh, S, Davidson, A, Addington, J, Ajroud Driss, S, Andersen, H, Antonini, G, Attarian, S, Badrising, U, Barroso, F, Benedetti, L, Beronio, A, Bianco, M, Binda, D, Briani, C, Bã1⁄4rmann, J, Bella, I, Bertorini, T, Bhavaraju Sanka, R, Brannagan, T, Busby, M, Butterworth, S, Campagnolo, M, Casasnovas, C, Cavaletti, G, Chao, C, Chen, S, Chetty, S, Claeys, K, Cohen, J, Conti, M, Cosgrove, J, Dalakas, M, Dimachkie, M, Dillmann, U, Domínguez González, C, Doppler, K, Dornonville de la Cour, C, Echaniz Laguna, A, Eftimov, F, Faber, C, Fazio, R, Fokke, C, Fujioka, T, Fulgenzi, E, Galassi, G, Garcia, T, Garnero, M, Garssen, M, Gijsbers, C, Gilchrist, J, Gilhuis, H, Goldstein, J, Goyal, N, Granit, V, Grapperon, A, Gutiérrez Gutiérrez, G, Gutmann, L, Hadden, R, Holbech, J, Holt, J, Homedes Pedret, C, Htut, M, Jellema, K, JericÃ3 Pascual, I, Kaida, K, Karafiath, S, Katzberg, H, Kiers, L, Kieseier, B, Kimpinski, K, Kleyweg, R, Kokubun, N, Kolb, N, Kuitwaard, K, Kuwabara, S, Kwan, J, Ladha, S, Landschoff Lassen, L, Lawson, V, Ledingham, D, Léon Cejas, L, Luciano, C, Lucy, S, Lunn, M, Magot, A, Manji, H, Marchesoni, C, Marfia, G, Márquez Infante, C, Martinez Hernandez, E, Mataluni, G, Mattiazi, M, Mcdermott, C, Meekins, G, Miller, J, Monges, M, Montero, M, Morís de la Tassa, G, Nascimbene, C, Neumann, C, Nowak, R, Orizaola Balaguer, P, Osei Bonsu, M, Pan, E, Pardo Fernandez, J, Pasnoor, M, Pulley, M, Rajabally, Y, Rinaldi, S, Ritter, C, Roberts, R, Rojas Marcos, I, Rudnicki, S, Sachs, G, Samijn, J, Santoro, L, Saperstein, D, Savransky, A, Schneider, H, Schenone, A, Sedano Tous, M, Sekiguchi, Y, Sheikh, K, Silvestri, N, Sindrup, S, Sommer, C, Stein, B, Stino, A, Spyropoulos, A, Srinivasan, J, Suzuki, H, Taylor, S, Tankisi, H, Tigner, D, Twydell, P, Valzania, F, van Damme, P, van der Kooi, A, van Dijk, G, van der Ree, T, van Koningsveld, R, Varrato, J, Vermeij, F, Verschuuren, J, Visser, L, Vytopil, M, Waheed, W, Wilken, M, Wilkerson, C, Wirtz, P, Yamagishi, Y, Yiu, E, Zhou, L, Zivkovic, S, Zivkovic, S., BINDA, DAVIDE, CAVALETTI, GUIDO ANGELO, Jacobs, B, van den Berg, B, Verboon, C, Chavada, G, Cornblath, D, Gorson, K, Harbo, T, Hartung, H, Hughes, R, Kusunoki, S, van Doorn, P, Willison, H, van Woerkom, M, Roodbol, J, Reisin, R, Reddel, S, Islam, Z, Islam, B, Mohammad, Q, van den Bergh, P, Feasby, T, Wang, Y, Pã©rã©on, Y, Lehmann, H, Dardiotis, E, Nobile Orazio, E, Shahrizaila, N, Bateman, K, Illa, I, Querol, L, Hsieh, S, Davidson, A, Addington, J, Ajroud Driss, S, Andersen, H, Antonini, G, Attarian, S, Badrising, U, Barroso, F, Benedetti, L, Beronio, A, Bianco, M, Binda, D, Briani, C, Bã1⁄4rmann, J, Bella, I, Bertorini, T, Bhavaraju Sanka, R, Brannagan, T, Busby, M, Butterworth, S, Campagnolo, M, Casasnovas, C, Cavaletti, G, Chao, C, Chen, S, Chetty, S, Claeys, K, Cohen, J, Conti, M, Cosgrove, J, Dalakas, M, Dimachkie, M, Dillmann, U, Domínguez González, C, Doppler, K, Dornonville de la Cour, C, Echaniz Laguna, A, Eftimov, F, Faber, C, Fazio, R, Fokke, C, Fujioka, T, Fulgenzi, E, Galassi, G, Garcia, T, Garnero, M, Garssen, M, Gijsbers, C, Gilchrist, J, Gilhuis, H, Goldstein, J, Goyal, N, Granit, V, Grapperon, A, Gutiérrez Gutiérrez, G, Gutmann, L, Hadden, R, Holbech, J, Holt, J, Homedes Pedret, C, Htut, M, Jellema, K, JericÃ3 Pascual, I, Kaida, K, Karafiath, S, Katzberg, H, Kiers, L, Kieseier, B, Kimpinski, K, Kleyweg, R, Kokubun, N, Kolb, N, Kuitwaard, K, Kuwabara, S, Kwan, J, Ladha, S, Landschoff Lassen, L, Lawson, V, Ledingham, D, Léon Cejas, L, Luciano, C, Lucy, S, Lunn, M, Magot, A, Manji, H, Marchesoni, C, Marfia, G, Márquez Infante, C, Martinez Hernandez, E, Mataluni, G, Mattiazi, M, Mcdermott, C, Meekins, G, Miller, J, Monges, M, Montero, M, Morís de la Tassa, G, Nascimbene, C, Neumann, C, Nowak, R, Orizaola Balaguer, P, Osei Bonsu, M, Pan, E, Pardo Fernandez, J, Pasnoor, M, Pulley, M, Rajabally, Y, Rinaldi, S, Ritter, C, Roberts, R, Rojas Marcos, I, Rudnicki, S, Sachs, G, Samijn, J, Santoro, L, Saperstein, D, Savransky, A, Schneider, H, Schenone, A, Sedano Tous, M, Sekiguchi, Y, Sheikh, K, Silvestri, N, Sindrup, S, Sommer, C, Stein, B, Stino, A, Spyropoulos, A, Srinivasan, J, Suzuki, H, Taylor, S, Tankisi, H, Tigner, D, Twydell, P, Valzania, F, van Damme, P, van der Kooi, A, van Dijk, G, van der Ree, T, van Koningsveld, R, Varrato, J, Vermeij, F, Verschuuren, J, Visser, L, Vytopil, M, Waheed, W, Wilken, M, Wilkerson, C, Wirtz, P, Yamagishi, Y, Yiu, E, Zhou, L, Zivkovic, S, Zivkovic, S., BINDA, DAVIDE, and CAVALETTI, GUIDO ANGELO

Abstract

Guillain-Barré syndrome (GBS) is an acute polyradiculoneuropathy with a highly variable clinical presentation, course, and outcome. The factors that determine the clinical variation of GBS are poorly understood which complicates the care and treatment of individual patients. The protocol of the ongoing International GBS Outcome Study (IGOS), a prospective, observational, multicenter cohort study that aims to identify the clinical and biological determinants and predictors of disease onset, subtype, course and outcome of GBS is presented here. Patients fulfilling the diagnostic criteria for GBS, regardless of age, disease severity, variant forms, or treatment, can participate if included within 2 weeks after onset of weakness. Information about demography, preceding infections, clinical features, diagnostic findings, treatment, course, and outcome is collected. In addition, cerebrospinal fluid and serial blood samples for serum and DNA is collected at standard time points. The original aim was to include at least 1,000 patients with a follow-up of 1–3 years. Data are collected via a web-based data entry system and stored anonymously. IGOS started in May 2012 and by January 2017 included more than 1,400 participants from 143 active centers in 19 countries across 5 continents. The IGOS data/biobank is available for research projects conducted by expertise groups focusing on specific topics including epidemiology, diagnostic criteria, clinimetrics, electrophysiology, antecedent events, antibodies, genetics, prognostic modeling, treatment effects, and long-term outcome of GBS. The IGOS will help to standardize the international collection of data and biosamples for future research of GBS.

Published
2017

19. Diagnostic value of the activity of mitochondrial respiratory chain complex for mitochondrial myopathies

Author

Lebrato-Hernandez, L., primary, Rivas-Infante, E., additional, Cortes-Rodriguez, A., additional, Carbonell-Corvillo, P., additional, Villareal-Perez, L., additional, Marquez-Infante, C., additional, Rojas-Marcos, I., additional, Martinez-Fernandez, E., additional, Carvajal-Hernandez, A., additional, Cascajo-Almenara, M., additional, Martin-Casanueva, M., additional, Rodriguez-Aguilera, J., additional, and Paradas-Lopez, C., additional

Published
2016
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20. Late onset riboflavin responsive lipid myopathy with multiple acyl-CoA dehydrogenase deficiency: Report of four patients

Author

Rojas-Marcos, I., primary, Rubí, J., additional, Blázquez, A., additional, Carbonell, P., additional, De Torres, R., additional, Domínguez-Mayoral, A., additional, Ávilla, R., additional, Martín-Casanueva, M., additional, Gutiérrez, C., additional, Márquez-Infante, C., additional, Rivas, E., additional, Paradas, C., additional, and Domínguez-González, C., additional

Published
2016
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22. Ischemic stroke in patients with glioblastoma multiforme

Author

Rojas-Marcos, I., Martin-Duverneuil, N., Laigle-Donadey, F., Taillibert, S., and Delattre, Y.J.

Subjects
Glioblastoma multiforme -- Diagnosis, Glioblastoma multiforme -- Complications and side effects, Glioblastoma multiforme -- Case studies, Stroke (Disease) -- Diagnosis, Stroke (Disease) -- Case studies, Health
Published
2005

25. [Paraneoplastic syndromes in otoneuro-ophthalmology]

Author

Rojas-Marcos I, Reñé R, and Francesc Graus

Subjects
Optic Neuritis, Eye Diseases, Hearing Loss, Sensorineural, Vision Disorders, Paraneoplastic Cerebellar Degeneration, Uveitis, Lambert-Eaton Myasthenic Syndrome, Autoimmune Diseases of the Nervous System, Retinal Diseases, Neoplasms, Sensation Disorders, Humans, Neoplasms, Unknown Primary, Paraneoplastic Polyneuropathy, Paraneoplastic Syndromes, Nervous System
Abstract

The so-called neurological paraneoplastic syndromes (NPNS) are a group of diseases of the central nervous system of unknown etiology which are seen almost exclusively in patients with cancer. We review the main NPNS paying particular attention to those with ophthalmological and otological features.Certain neuro-ophthalmological findings may constitute, at least partly, some paraneoplastic syndromes. There are alterations of vision in paraneoplastic retinopathy and in optic neuritis of paraneoplastic origin. The latter, unlike the retinopathy, usually coexists with involvement of other structures of the nervous system. Oculomotor function is affected in the opsoclonus-myoclonus syndrome. Diplopia and/or ophthalmoplegia may be a predominant or initial symptom, in patients with paraneoplastic neurological degeneration or brainstem encephalitis. In the Lambert-Eaton syndrome and in paraneoplastic encephalomyelitis, may have blurred vision and alterations of the pupil. Cases of paraneoplastic uveitis have also been described. Paraneoplastic otological involvement is less frequent. Patients with sensorineural deafness in the context of a paraneoplastic encephalomyelitis have been reported. In the NPNS vertigo is caused by cerebellar or brainstem lesions and not by lesions of peripheral organs. When nystagmus occurs in a NPNS it may be of various types and is due to involvement of structures in the brain stem or cerebellum.The diagnosis of NPNS in patients with no known cancer is important because it may lead to the detection of an occult cancer which is localized or scarcely extended, and therefore is still potentially treatable. Oto-neuro-ophthalmological manifestations may be the first or only symptom of presentation of a paraneoplastic neurological clinical picture.

Published
2001

29. Patterns of care and outcome for patients with glioblastoma diagnosed during 2008-2010 in Spain

Author

Graus, F., primary, Bruna, J., additional, Pardo, J., additional, Escudero, D., additional, Vilas, D., additional, Barcelo, I., additional, Brell, M., additional, Pascual, C., additional, Crespo, J. A., additional, Erro, E., additional, Garcia-Romero, J. C., additional, Estela, J., additional, Martino, J., additional, Garcia-Castano, A., additional, Mata, E., additional, Lema, M., additional, Gelabert, M., additional, Fuentes, R., additional, Perez, P., additional, Manzano, A., additional, Aguas, J., additional, Belenguer, A., additional, Simon, A., additional, Henriquez, I., additional, Murcia, M., additional, Vivanco, R., additional, Rojas-Marcos, I., additional, Munoz-Carmona, D., additional, Navas, I., additional, de Andres, P., additional, Mas, G., additional, Gil, M., additional, and Verger, E., additional

Published
2013
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31. CpG island hypermethylation of the DNA repair enzyme methyltransferase predicts response to temozolomide in primary gliomas

Author

Yaya-Tur, R., primary, Paz, M.F., additional, Rojas-Marcos, I., additional, Reynes, G., additional, Pollan, M., additional, Aguirre-Cruz, L., additional, Garcia-Lopez, J.L., additional, Piquer, J., additional, Safont, M.J., additional, Balana, C., additional, Sanchez-Cespedes, M., additional, Garcia-Villanueva, M., additional, Arribas, L., additional, and Esteller, M., additional

Published
2005
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32. Análisis descriptivo de la demanda de asistencia neurológica ambulatoria en un área sanitaria de Huelva

Author

Robledo-Strauss A, Sanz-Fernández G, López-Domínguez Jm, Díaz-Espejo C, Blanco-Ollero A, and Rojas-Marcos I

Subjects
Neurology (clinical), General Medicine
Abstract

Introduccion. En los ultimos anos se ha incrementado la demanda de asistencia neurologica ambulatoria. Su estudio puede contribuir a mejorar la planificacion sanitaria y la calidad de las derivaciones desde atencion primaria. Objetivo. Analizar diversas variables de las primeras visitas remitidas desde atencion primaria a las consultas de neurologia. Pacientes y metodos. Estudio prospectivo descriptivo en el que se recogieron de forma consecutiva los datos de 500 pacientes nuevos procedentes de atencion primaria citados en una consulta de neurologia general en el area sanitaria de la provincia de Huelva. Se analizaron las caracteristicas demograficas, el motivo de derivacion, los diagnosticos iniciales y el indice de resolucion de los casos. Resultados. La media de edad fue de 51 anos, con un predominio femenino (63,4%). Los diagnosticos mas frecuentes fueron las cefaleas (42,8%) y el deterioro cognitivo (12%). El 8,2% de los pacientes remitidos no tenia ninguna patologia neurologica. El 40,2% fue dado de alta tras la primera visita. Conclusiones. Las caracteristicas demograficas y los motivos de consulta son similares a los publicados previamente en otras comunidades espanolas. El elevado porcentaje de altas directas traduce una escasa seleccion de los pacientes remitidos desde atencion primaria. La mejora en la calidad de las derivaciones permitiria mejorar la eficiencia de la asistencia neurologica ambulatoria

Published
2007

34. Infarto cortical frontal y temblor postural e intencional contralateral.

Author

Domínguez, J. M. López, Rojas-Marcos, I., Fernández, G. Sanz, and Strauss, A. Robledo

Subjects
INFARCTION, TREMOR, CEREBROVASCULAR disease, FRONTAL lobe, PATHOLOGICAL physiology
Abstract

Copyright of Neurologia (Grupo ARS XXI de Comunicacion, S.A.) is the property of Grupo ARS XXI de Comunicacion, S.A. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Published
2008

35. Human epidermal growth factor receptor 2 overexpression in breast cancer of patients with anti-Yo--associated paraneoplastic cerebellar degeneration

Author

Dimitri Psimaras, Bruno Giometto, David Chinchón, Jérôme Honnorat, Iñigo Rojas-Marcos, Ellen Gelpi, Francesc Graus, Géraldine Picard, J.-Y. Delattre, Rojas-Marcos, I, Picard, G, Chinchon, D, Gelpi, E, Psimaras, D, Giometto, B, Delattre, Jy, Honnorat, J, and Graus, F

Subjects
Adult, Cancer Research, Pathology, medicine.medical_specialty, Receptor, ErbB-2, Clinical Investigations, Breast Neoplasms, Nerve Tissue Proteins, Paraneoplastic Cerebellar Degeneration, Breast cancer, Antigen, Antigens, Neoplasm, Neuro-Oncological Ventral Antigen, medicine, Humans, Receptor, skin and connective tissue diseases, Human Epidermal Growth Factor Receptor 2, neoplasms, Aged, Retrospective Studies, biology, business.industry, Case-control study, RNA-Binding Proteins, Retrospective cohort study, Middle Aged, Paraneoplastic cerebellar degeneration, medicine.disease, Oncology, Case-Control Studies, biology.protein, Female, Neurology (clinical), Antibody, business, Paraneoplastic Syndromes, Nervous System
Abstract

Isolated case reports suggest that breast tumors from patients with paraneoplastic cerebellar degeneration (PCD) and Yo antibodies overexpress human epidermal growth factor receptor 2 (HER2). HER2 overexpression is present in 15%-25% of breast cancers and is associated with poor prognosis. We retrospectively analyzed the status of HER2 in breast tumors of 27 patients with anti-Yo associated PCD to evaluate whether HER2 overexpression in this group of patients is higher than expected. In addition, we analyzed HER2 status of 19 breast tumors from patients with paraneoplastic neurological syndromes and Ri antibodies to see whether HER2 was specifically related to anti-Yo associated PCD. We also assessed cdr2 expression (the onconeural antigen recognized by Yo antibodies) in 21 HER2-positive breast tumors from patients without paraneoplastic neurological syndromes. HER2 was overexpressed in 26 patients (96.3%) with anti-Yo associated PCD but only in 2 patients (10.5%) with paraneoplastic neurological syndromes associated with Ri antibodies (P < .0001). Only 5 (23.8%) of the 21 HER2-positive breast tumors showed cdr2 immunoreactivity. This study shows a very high frequency of HER2 overexpression in breast cancers in patients with anti-Yo associated PCD but not in those from patients with Ri antibodies. Although the expression of cdr2 onconeural antigen is not high in HER2-positive breast cancers, HER2 overexpression seems to be an important requirement to develop an anti-Yo associated PCD.

Published
2012

36. Ablation of the carboxy-terminal end of MAMDC2 causes a distinct muscular dystrophy.

Author

Mavillard F, Servian-Morilla E, Dofash L, Rojas-Marcos I, Folland C, Monahan G, Gutierrez-Gutierrez G, Rivas E, Hernández-Lain A, Valladares A, Cantero G, Morales JM, Laing NG, Paradas C, Ravenscroft G, and Cabrera-Serrano M

Subjects
Adult, Humans, Muscle, Skeletal metabolism, Extracellular Matrix Proteins, Muscular Dystrophies genetics
Abstract

The extracellular matrix (ECM) has an important role in the development and maintenance of skeletal muscle, and several muscle diseases are associated with the dysfunction of ECM elements. MAMDC2 is a putative ECM protein and its role in cell proliferation has been investigated in certain cancer types. However, its participation in skeletal muscle physiology has not been previously studied. We describe 17 individuals with an autosomal dominant muscular dystrophy belonging to two unrelated families in which different heterozygous truncating variants in the last exon of MAMDC2 co-segregate correctly with the disease. The radiological aspect of muscle involvement resembles that of COL6 myopathies with fat replacement at the peripheral rim of vastii muscles. In this cohort, a subfascial and peri-tendinous pattern is observed in upper and lower limb muscles. Here we show that MAMDC2 is expressed in adult skeletal muscle and differentiating muscle cells, where it appears to localize to the sarcoplasm and myonuclei. In addition, we show it is secreted by myoblasts and differentiating myotubes into to the extracellular compartment. The last exon encodes a disordered region with a polar residue compositional bias loss of which likely induces a toxic effect of the mutant protein. The precise mechanisms by which the altered MAMDC2 proteins cause disease remains to be determined. MAMDC2 is a skeletal muscle disease-associated protein. Its role in muscle development and ECM-muscle communication remains to be fully elucidated. Screening of the last exon of MAMDC2 should be considered in patients presenting with autosomal dominant muscular dystrophy, particularly in those with a subfascial radiological pattern of muscle involvement., (© The Author(s) 2023. Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Published
2023
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37. Autoantibody screening in Guillain-Barré syndrome.

Author

Lleixà C, Martín-Aguilar L, Pascual-Goñi E, Franco T, Caballero M, de Luna N, Gallardo E, Suárez-Calvet X, Martínez-Martínez L, Diaz-Manera J, Rojas-García R, Cortés-Vicente E, Turón J, Casasnovas C, Homedes C, Gutiérrez-Gutiérrez G, Jimeno-Montero MC, Berciano J, Sedano-Tous MJ, García-Sobrino T, Pardo-Fernández J, Márquez-Infante C, Rojas-Marcos I, Jericó-Pascual I, Martínez-Hernández E, Morís de la Tassa G, Domínguez-González C, Juárez C, Illa I, and Querol L

Subjects
Aged, Animals, Cell Line, Tumor, Cohort Studies, Female, Ganglia, Spinal metabolism, Ganglia, Spinal pathology, Guillain-Barre Syndrome epidemiology, Humans, Macaca, Male, Mass Screening methods, Middle Aged, Prospective Studies, Rats, Spain epidemiology, Autoantibodies blood, Guillain-Barre Syndrome blood, Guillain-Barre Syndrome diagnosis
Abstract

Background: Guillain-Barré syndrome (GBS) is an acute inflammatory neuropathy with a heterogeneous presentation. Although some evidences support the role of autoantibodies in its pathogenesis, the target antigens remain unknown in a substantial proportion of GBS patients. The objective of this study is to screen for autoantibodies targeting peripheral nerve components in Guillain-Barré syndrome., Methods: Autoantibody screening was performed in serum samples from all GBS patients included in the International GBS Outcome study by 11 different Spanish centres. The screening included testing for anti-ganglioside antibodies, anti-nodo/paranodal antibodies, immunocytochemistry on neuroblastoma-derived human motor neurons and murine dorsal root ganglia (DRG) neurons, and immunohistochemistry on monkey peripheral nerve sections. We analysed the staining patterns of patients and controls. The prognostic value of anti-ganglioside antibodies was also analysed., Results: None of the GBS patients (n = 100) reacted against the nodo/paranodal proteins tested, and 61 (61%) were positive for, at least, one anti-ganglioside antibody. GBS sera reacted strongly against DRG neurons more frequently than controls both with IgG (6% vs 0%; p = 0.03) and IgM (11% vs 2.2%; p = 0.02) immunodetection. No differences were observed in the proportion of patients reacting against neuroblastoma-derived human motor neurons. Reactivity against monkey nerve tissue was frequently detected both in patients and controls, but specific patterns were only detected in GBS patients: IgG from 13 (13%) patients reacted strongly against Schwann cells. Finally, we confirmed that IgG anti-GM1 antibodies are associated with poorer outcomes independently of other known prognostic factors., Conclusion: Our study confirms that (1) GBS patients display a heterogeneous repertoire of autoantibodies targeting nerve cells and structures; (2) gangliosides are the most frequent antigens in GBS patients and have a prognostic value; (3) further antigen-discovery experiments may elucidate other potential antigens in GBS., (© 2021. The Author(s).)

Published
2021
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38. Serum neurofilament light chain predicts long-term prognosis in Guillain-Barré syndrome patients.

Author

Martín-Aguilar L, Camps-Renom P, Lleixà C, Pascual-Goñi E, Díaz-Manera J, Rojas-García R, De Luna N, Gallardo E, Cortés-Vicente E, Muñoz L, Alcolea D, Lleó A, Casasnovas C, Homedes C, Gutiérrez-Gutiérrez G, Jimeno-Montero MC, Berciano J, Sedano-Tous MJ, García-Sobrino T, Pardo-Fernández J, Márquez-Infante C, Rojas-Marcos I, Jericó-Pascual I, Martínez-Hernández E, Morís de la Tassa G, Domínguez-González C, Illa I, and Querol L

Abstract

Objective: To study baseline serum neurofilament light chain (sNfL) levels as a prognostic biomarker in Guillain-Barré syndrome (GBS)., Methods: We measured NfL in serum (98 samples) and cerebrospinal fluid (CSF) (24 samples) of patients with GBS prospectively included in the International GBS Outcome Study (IGOS) in Spain using single-molecule array (SiMoA) and compared them with 53 healthy controls (HCs). We performed multivariable regression to analyse the association between sNfL levels and functional outcome at 1 year., Results: Patients with GBS had higher NfL levels than HC in serum (55.49 pg/mL vs 9.83 pg/mL, p<0.0001) and CSF (1308.5 pg/mL vs 440.24 pg/mL, p=0.034). Patients with preceding diarrhoea had higher sNfL than patients with respiratory symptoms or no preceding infection (134.90 pg/mL vs 47.86 pg/mL vs 38.02 pg/mL, p=0.016). sNfL levels correlated with Guillain-Barré Syndrome Disability Score and Inflammatory Rasch-built Overall Disability Scale (I-RODS) at every timepoint. Patients with pure motor variant and Miller Fisher syndrome showed higher sNfL levels than patients with sensorimotor GBS (162.18 pg/mL vs 95.50 pg/mL vs 38.02 pg/mL, p=0.025). Patients with acute motor axonal neuropathy cute motor axonal neuropathy had higher sNfL levels than other variants (190.55 pg/mL vs 46.79 pg/mL, p=0.013). sNfL returned to normal levels at 1 year. High baseline sNfL levels were associated with inability to run (OR=1.65, 95% CI 1.14 to 2.40, p=0.009) and lower I-RODS (β -2.60, 95% CI -4.66 to -0.54, p=0.014) at 1 year. Cut-off points predicting clinically relevant outcomes at 1 year with high specificity were calculated: inability to walk independently (>319 pg/mL), inability to run (>248 pg/mL) and ability to run (<34 pg/mL)., Conclusion: Baseline sNfL levels are increased in patients with GBS, are associated with disease severity and axonal variants and have an independent prognostic value in patients with GBS., Competing Interests: Competing interests: LAQ has provided expert testimony for Grifols, Sanofi-Genzyme, Novartis, UCB, Roche and CSL Behring and received research funds from Novartis Spain, Sanofi-Genzyme and Grifols. LM-A has received speaking honoraria from Roche. EP-G has received speaking honoraria from Roche and Biogen. JD-M has provided expert testimony for PTC and Sanofi-Genzyme, has been external advisor for Sanofi, Sarepta and Audentes and received research funds from Sanofi-Genzyme and Boehringer. DA participated in advisory boards from Fujirebio-Europe and Roche Diagnostics and received speaker honoraria from Fujirebio-Europe, Nutricia and from Krka Farmacéutica S.L. GG-G has received speaking honoraria from Sanofi-Genzyme, Takeda and has provided expert testimony for Biogen and CSL Behring. The other authors report no disclosures., (© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Published
2020
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39. [Reversible posterior leukoencephalopathy syndrome associated to bevacizumab].

Author

Ortiz-Lopez EM, Gonzalez-Nieto JA, Rojas-Marcos I, and Blanco-Ollero A

Subjects
Aged, Angiogenesis Inhibitors therapeutic use, Antibodies, Monoclonal therapeutic use, Antibodies, Monoclonal, Humanized, Bevacizumab, Colonic Neoplasms pathology, Humans, Lung Neoplasms drug therapy, Lung Neoplasms secondary, Magnetic Resonance Imaging, Male, Posterior Leukoencephalopathy Syndrome pathology, Angiogenesis Inhibitors adverse effects, Antibodies, Monoclonal adverse effects, Posterior Leukoencephalopathy Syndrome chemically induced
Published
2010

40. [Primary sciatic nerve lymphoma].

Author

Rojas-Marcos I, Montero-Perea E, Salinas-Martín MV, Encinas V, Pujol M, and Martino M

Subjects
Aged, 80 and over, Biopsy, Fatal Outcome, Humans, Lymphoma diagnosis, Male, Sciatic Neuropathy diagnosis, Lymphoma pathology, Sciatic Nerve pathology, Sciatic Neuropathy pathology
Published
2010

41. [Frontal cortical infarction and contralateral postural and international tremor].

Author

López Domínguez JM, Rojas-Marcos I, Sanz Fernández G, and Robledo Strauss A

Subjects
Female, Humans, Magnetic Resonance Imaging, Middle Aged, Cerebral Infarction complications, Cerebral Infarction pathology, Cerebral Infarction physiopathology, Tremor etiology, Tremor physiopathology
Abstract

Introduction: Movement disorders, when caused by ischemic stroke, may appear as initial manifestation or after a variable interval of time. Among postictal movement disorders tremor is an uncommon manifestation. Holmes' tremor, which is a mixed tremor, is the most frequent type of tremor after stroke. It is associated to infarcts of various localizations like mesencephalon, pons, cerebellum, and thalamus. Cortical infarct is exceptional as a cause of tremor., Case Report: A sixty-three-year-old woman, with hypertension, who, 2 weeks after a cortical infarct located in the precentral circunvolution of the left frontal lobe, presented with a postural and intention tremor of low frequency (4 Hz) and high amplitude at the right arm. Tremor is still present 2 years after stroke and it has improved partially after treatment with clonazepam., Discussion: Frontal cortical infarcts may cause a contralateral postural and intentional tremor. It usually appears after a time interval. Tremor physiopathology may be related, in these cases, to interruption of the inhibitory fronto-subcortical motor circuit.

Published
2008

42. [A descriptive analysis of the demand for ambulatory neurological care in the health district of Huelva].

Author

López-Domínguez JM, Rojas-Marcos I, Sanz-Fernández G, Blanco-Ollero A, Robledo-Strauss A, and Díaz-Espejo C

Subjects
Adolescent, Adult, Aged, Aged, 80 and over, Ambulatory Care, Ambulatory Care Facilities, Female, Humans, Male, Middle Aged, Neurology, Prospective Studies, Spain, Cognition Disorders diagnosis, Cognition Disorders physiopathology, Nervous System Diseases diagnosis, Nervous System Diseases physiopathology
Abstract

Introduction: In recent years the demand for ambulatory neurological care has risen. Studying this situation can help to improve health care planning and the quality of the referrals from primary care., Aim: To analyse a number of variables involved in the first visits referred from primary care to neurology services., Patients and Methods: We conducted a prospective, descriptive study in which data was consecutively collected about 500 new patients from primary care who had appointments for a visit to general neurology services in the health district of the province of Huelva. Demographic features, the reason for referral, initial diagnoses and the case resolution index were analysed., Results: Mean age was 51 years old, and patients were predominantly female (63.4%). The most frequent diagnoses were headaches (42.8%) and cognitive impairment (12%). In 8.2% of cases the referred patients had no neurological pathology. Data showed that 40.2% were discharged after the first visit., Conclusions: The demographic characteristics and reasons for the visit were similar to those previously published on other regions in Spain. The high percentage of direct discharges translates into a poor selection of the patients referred from primary care. Improving the quality of referrals would make it possible to enhance the efficiency of ambulatory neurological care.

Published
2007

43. Frequent hypermethylation of the DNA repair gene MGMT in long-term survivors of glioblastoma multiforme.

Author

Martinez R, Schackert G, Yaya-Tur R, Rojas-Marcos I, Herman JG, and Esteller M

Subjects
Adult, Aged, Central Nervous System Neoplasms physiopathology, Glioblastoma physiopathology, Humans, Middle Aged, Survival Analysis, Central Nervous System Neoplasms genetics, DNA Methylation, DNA Modification Methylases genetics, DNA Repair Enzymes genetics, Glioblastoma genetics, Tumor Suppressor Proteins genetics
Abstract

We have performed a methylation-specific PCR approach to comparatively analyze the MGMT promoter methylation status in 186 glioblastomas (GBM) from patients with classic survival and nine from patients with long-term survival (LTS GBM). The methylation rate in LTS GBM was significantly higher (77.8% vs. 39.2%, P = 0.033) which suggests that MGMT hypermethylation is a frequent hallmark of LTS GBM and contributes to characterize this intriguing GBM subtype.

Published
2007
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44. Gene symbol: NOTCH3.

Author

Rojas-Marcos I, Garcfa-Lozano R, Lozano P, Gil-NTciga E, Gil-Peralta A, and Bautista J

Subjects
Amino Acid Substitution, Codon, Nonsense, Humans, Mutation, Missense, Receptor, Notch3, CADASIL genetics, Receptors, Notch genetics
Published
2007

45. [Room tilt illusion: a rare symptom of migraine aura].

Author

López Domínguez JM, Rojas-Marcos I, Sanz Fernández G, and Díaz Espejo C

Subjects
Female, Humans, Middle Aged, Illusions etiology, Migraine with Aura complications
Abstract

Introduction: Room tilt illusion is a distorted perception of the spatial distribution of objects due to complex rotations in different planes. This distortion usually occurs in the vertical plane and is usually associated to vertebrobasilar ischemia. The etiology of the phenomenon varies greatly and only rarely corresponds to migraine aura., Case Report: Since youth, a sixty-year-old woman had frequent episodes of room tilt illusion in the horizontal plane. These lasted from 30 minutes to 2 hours and were always followed by frontal headache fulfilling criteria for migraine (computerized tomography and magnetic resonance imaging) were normal. Visual symptoms and headache disappeared following treatment with flunarizine., Discussion: Room tilt illusion might be a rare symptom of migraine aura. In this setting a functional depression of neurons from the posterior parietal cortex may occur, possibly mediated by chemical factors.

Published
2007

46. Hypersomnia as presenting symptom of anti-Ma2-associated encephalitis: case study.

Author

Rojas-Marcos I, Graus F, Sanz G, Robledo A, and Diaz-Espejo C

Subjects
Aged, Antigens, Neoplasm immunology, Encephalitis immunology, Encephalitis therapy, Humans, Male, Nerve Tissue Proteins immunology, Antigens, Neoplasm metabolism, Disorders of Excessive Somnolence diagnosis, Encephalitis diagnosis, Nerve Tissue Proteins metabolism
Abstract

We describe a patient who presented with excessive daytime sleepiness (EDS) and was eventually diagnosed with anti-Ma2 encephalitis. Neurological examination disclosed somnolence, left palpebral ptosis, and vertical gaze paresis. A brain MRI showed high signal intensity in the hypothalamus and each hippocampus. Ma2 antibodies were found in the patient's serum, and fiberbronchoscopy disclosed a lung carcinoma. After three months of steroid treatment, the results of the patient's neurological exam became normal. We conclude that anti-Ma2 encephalitis may present with mostly isolated EDS and that it may respond to steroids despite old age and the presence of an untreated lung cancer.

Published
2007
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47. Meningeal lymphomatosis as the first manifestation of splenic marginal zone lymphoma.

Author

Bruna J, Martínez-Yelamos S, Alonso E, Romagosa V, Arruga J, Arruga J, Domingo A, Rojas-Marcos I, Petit J, and Rubio F

Subjects
Adrenal Cortex Hormones therapeutic use, Antigens, CD analysis, Fatal Outcome, Humans, Lymphoproliferative Disorders immunology, Male, Meninges immunology, Middle Aged, Lymphoma complications, Lymphoproliferative Disorders etiology, Meninges pathology, Splenic Neoplasms complications
Abstract

Meningeal lymphomatosis (ML) as the first manifestation of a splenic marginal zone lymphoma (SMZL) is rare. The descriptions of only 2 cases with this complication, one of which had ML as the first manifestation, have been published to date. We describe a 53-year-old man, an ex-smoker, who presented with transitory episodes of bilateral loss of visual acuity. On examination, only papilledema and splenomegalia were observed. The hemogram showed a predominance of lymphocytes with a villous morphology. Cytochemical staining and an immunophenotypic analysis revealed a positive reaction to tartrate-sensitive acid phosphatase and B-lineage markers (CD19+, CD20+, CD79b+, surface immunoglobulin 3 expression, immunoglobulin D+, CD5-, CD23-, CD10-, CD25-, CD103-, and CD11c-). Magnetic resonance imaging of the brain showed tumoral infiltration in both optic nerves and in the cervicodorsal meninges. The cerebrospinal fluid examination revealed significant pleocytosis, and all lymphocytes had a phenotype identical to that of the peripheral blood, confirming the presence of ML. The bone marrow section also showed lymphocytes with an immunophenotype identical to that of the peripheral blood.A splenectomy confirmed the SMZL diagnosis. Treatment with corticosteroids and intrathecal chemotherapy was administrated; however, the response was not good, and the patient died. In this report, we discuss the other 2 cases and ML in B-cell chronic lymphoproliferative disorders.

Published
2005
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48. CpG island hypermethylation of the DNA repair enzyme methyltransferase predicts response to temozolomide in primary gliomas.

Author

Paz MF, Yaya-Tur R, Rojas-Marcos I, Reynes G, Pollan M, Aguirre-Cruz L, García-Lopez JL, Piquer J, Safont MJ, Balaña C, Sanchez-Cespedes M, García-Villanueva M, Arribas L, and Esteller M

Subjects
Adult, Aged, Alkylating Agents pharmacology, Brain Neoplasms therapy, Carmustine pharmacology, DNA metabolism, Female, Humans, Male, Middle Aged, Polymerase Chain Reaction, Temozolomide, Brain Neoplasms genetics, CpG Islands, DNA Methylation, DNA Repair, Dacarbazine analogs & derivatives, Dacarbazine pharmacology, Glioma genetics, O(6)-Methylguanine-DNA Methyltransferase genetics, Promoter Regions, Genetic, Treatment Outcome
Abstract

Purpose: The DNA repair enzyme O(6)-methylguanine DNA methyltransferase (MGMT) inhibits the killing of tumor cells by alkylating agents, and its loss in cancer cells is associated with hypermethylation of the MGMT CpG island. Thus, methylation of MGMT has been correlated with the clinical response to 1,3-bis(2-chloroethyl)-1-nitrosourea (BCNU) in primary gliomas. Here, we investigate whether the presence of MGMT methylation in gliomas is also a good predictor of response to another emergent alkylating agent, temozolomide., Experimental Design: Using a methylation-specific PCR approach, we assessed the methylation status of the CpG island of MGMT in 92 glioma patients who received temozolomide as first-line chemotherapy or as treatment for relapses., Results: Methylation of the MGMT promoter positively correlated with the clinical response in the glioma patients receiving temozolomide as first-line chemotherapy (n = 40). Eight of 12 patients with MGMT-methylated tumors (66.7%) had a partial or complete response, compared with 7 of 28 patients with unmethylated tumors (25.0%; P = 0.030). We also found a positive association between MGMT methylation and clinical response in those patients receiving BCNU (n = 35, P = 0.041) or procarbazine/1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea (n = 17, P = 0.043) as first-line chemotherapy. Overall, if we analyze the clinical response of all of the first-line chemotherapy treatments with temozolomide, BCNU, and procarbazine/1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea as a group in relation to the MGMT methylation status, MGMT hypermethylation was strongly associated with the presence of partial or complete clinical response (P < 0.001). Finally, the MGMT methylation status determined in the initial glioma tumor did not correlate with the clinical response to temozolomide when this drug was administered as treatment for relapses (P = 0.729)., Conclusions: MGMT methylation predicts the clinical response of primary gliomas to first-line chemotherapy with the alkylating agent temozolomide. These results may open up possibilities for more customized treatments of human brain tumors.

Published
2004
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49. Gene symbol: NOTCH3. Disease: CADASIL.

Author

Rojas-Marcos I, Encarnacion M, Martinez-Yelamos S, Ferrer I, Arbizu T, Gil-Peralta A, and Garcia-Lozano JR

Subjects
Humans, Receptor, Notch3, Receptors, Notch, Dementia, Multi-Infarct genetics, Mutation, Missense, Proto-Oncogene Proteins genetics, Receptors, Cell Surface genetics
Published
2004

50. Spectrum of paraneoplastic neurologic disorders in women with breast and gynecologic cancer.

Author

Rojas-Marcos I, Rousseau A, Keime-Guibert F, Reñé R, Cartalat-Carel S, Delattre JY, and Graus F

Subjects
Aged, Antibodies, Neoplasm immunology, Antigens, Neoplasm immunology, Female, Humans, Middle Aged, Paraneoplastic Polyneuropathy immunology, Retrospective Studies, Breast Neoplasms complications, Genital Neoplasms, Female complications, Paraneoplastic Polyneuropathy complications
Abstract

We conducted the current review of the paraneoplastic neurologic syndromes (PNSs) associated with gynecologic and breast carcinomas to describe their clinical and immunologic characteristics and their relative frequency. We retrospectively reviewed 92 patients whose serum was sent to our laboratories to detect onconeural antibodies and who were diagnosed as having PNSs associated with breast or gynecologic tumors. PNSs were defined as "definitive" and "possible" (atypical PNS, no onconeural antibodies, and no improvement after tumor treatment). Forty-nine patients had breast and 43 had gynecologic cancer. Sixty-three patients had onconeural antibodies (50 Yo-ab, 5 Hu-ab, 5 Ri-ab, and 3 amphiphysin-ab). Cerebellar ataxia represented 57 (62%) of all PNSs and was associated with anti-Yo in 88%. All Yo-abnegative patients had breast cancer; 4 of them had a mild cerebellar syndrome that improved after tumor treatment. Sensorypredominant neuropathies were present in 17 (18%) patients. Seven of them had Hu-ab (5) or amphiphysin-ab (2). Other PNSs were opsoclonus-myoclonus syndrome (4 cases, Ri-ab in 2), sensorimotor neuropathy (4 cases), paraneoplastic encephalomyelitis (4 cases, Ri-ab in 3), paraneoplastic retinopathy (2 cases), amyotrophic lateral sclerosis (2 cases), stiff-person syndrome (1 with amphiphysin-ab), and limbic encephalitis (1 case). All patients with gynecologic cancer presented definitive PNS, and onconeural antibodies were diagnosed in 93% of them. In contrast, 20% of PNSs associated with breast cancer were defined as possible and the incidence of onconeural antibodies was 51%, excluding the 2 patients with paraneoplastic retinopathy in whom antiretinal antibodies were not analyzed. In patients with possible PNS, a coincidental association between the tumor and the neurologic disorder cannot be excluded.

Published
2003
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