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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

2. Reflectie met beleidsaanbevelingen naar aanleiding van het rapport ‘De Europees -en internationaalrechtelijke status van de Waddenzee’

Author: van Rijswick, Helena, Bastmeijer, C.J. (Kees), Boerema, Luuk, Gilissen, Herman Kasper, Kistenkas , F., Miltenburg, L., Trouwborst, A., Verschuuren, J., Zwier, W, van Rijswick, Helena, Bastmeijer, C.J. (Kees), Boerema, Luuk, Gilissen, Herman Kasper, Kistenkas , F., Miltenburg, L., Trouwborst, A., Verschuuren, J., and Zwier, W
Published: 2024

3. The Dutch Dystrophinopathy Database: A National Registry with Standardized Patient and Clinician Reported Real-World Data

Author: Neurologen, Epi Methoden Team 1, Arts Assistenten Longziekten, van de Velde, N. M., Krom, Y. D., Bongers, J., Hoek, R. J.A., Ikelaar, N. A., van der Holst, M., Naarding, K. J., van den Bergen, J. C., Vroom, E., Horemans, A., Hendriksen, J. G.M., de Groot, I. J.M., Opstal, S. L.S.Houwen Van, Verschuuren, J. J.G.M., van Duyvenvoorde, H. A., Snijder, R. R., Niks, E. H., Neurologen, Epi Methoden Team 1, Arts Assistenten Longziekten, van de Velde, N. M., Krom, Y. D., Bongers, J., Hoek, R. J.A., Ikelaar, N. A., van der Holst, M., Naarding, K. J., van den Bergen, J. C., Vroom, E., Horemans, A., Hendriksen, J. G.M., de Groot, I. J.M., Opstal, S. L.S.Houwen Van, Verschuuren, J. J.G.M., van Duyvenvoorde, H. A., Snijder, R. R., and Niks, E. H.
Published: 2024

4. Reflectie met beleidsaanbevelingen naar aanleiding van het rapport ‘De Europees -en internationaalrechtelijke status van de Waddenzee’

Author: Parel Water en duurzaamheid / UCWOSL, Europees en nationaal waterrecht, van Rijswick, Helena, Bastmeijer, C.J. (Kees), Boerema, Luuk, Gilissen, Herman Kasper, Kistenkas , F., Miltenburg, L., Trouwborst, A., Verschuuren, J., Zwier, W, Parel Water en duurzaamheid / UCWOSL, Europees en nationaal waterrecht, van Rijswick, Helena, Bastmeijer, C.J. (Kees), Boerema, Luuk, Gilissen, Herman Kasper, Kistenkas , F., Miltenburg, L., Trouwborst, A., Verschuuren, J., and Zwier, W
Published: 2024

5. Reflectie met beleidsaanbevelingen : naar aanleiding van het rapport ‘De Europees - en internationaalrechtelijke status van de Waddenzee’

Author: Bastmeijer, K., Boerema, L., Gilissen, H.K., Kistenkas, F., Miltenburg, L., Rijswick, M. van, Trouwborst, A., Verschuuren, J., Zwier, W., Bastmeijer, K., Boerema, L., Gilissen, H.K., Kistenkas, F., Miltenburg, L., Rijswick, M. van, Trouwborst, A., Verschuuren, J., and Zwier, W.
Abstract: Dit rapport bevat een reflectie en vijftien beleidsaanbevelingen naar aanleiding van het rapport ‘De Europees- en internationaalrechtelijke status van de Waddenzee’ (Waddenacademie, december 2023).
Published: 2024

6. Low dystrophin variability between muscles and stable expression over time in Becker muscular dystrophy using capillary Western immunoassay

Author: Koeks, Z., Janson, A. A., Beekman, C., Signorelli, M., van Duyvenvoorde, H. A., van den Bergen, J. C., Hooijmans, M. T., Alleman, I., Hegeman, I. M., Verschuuren, J. J. G. M., v. Deutekom, J. C., Spitali, P., Datson, N. A., and Niks, E. H.
Published: 2021
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7. CSF Findings in Relation to Clinical Characteristics, Subtype, and Disease Course in Patients With Guillain-Barré Syndrome

Author: Al-Hakem, H, Doets, A, Stino, A, Zivkovic, S, Andersen, H, Willison, H, Cornblath, D, Gorson, K, Islam, Z, Mohammad, Q, Sindrup, S, Kusunoki, S, Davidson, A, Casasnovas, C, Bateman, K, Miller, J, Van Den Berg, B, Verboon, C, Roodbol, J, Leonhard, S, Arends, S, Luijten, L, Benedetti, L, Kuwabara, S, Van Den Bergh, P, Monges, S, Marfia, G, Shahrizaila, N, Galassi, G, Pereon, Y, Burmann, J, Kuitwaard, K, Kleyweg, R, Marchesoni, C, Tous, M, Querol, L, Martin-Aguilar, L, Wang, Y, Nobile-Orazio, E, Rinaldi, S, Schenone, A, Pardo, J, Vermeij, F, Waheed, W, Lehmann, H, Granit, V, Stein, B, Cavaletti, G, Gutierrez-Gutierrez, G, Barroso, F, Visser, L, Katzberg, H, Dardiotis, E, Attarian, S, Van Der Kooi, A, Eftimov, F, Wirtz, P, Samijn, J, Jacobus Gilhuis, H, Hadden, R, Holt, J, Sheikh, K, Kolb, N, Karafiath, S, Vytopil, M, Antonini, G, Feasby, T, Faber, C, Kramers, H, Busby, M, Roberts, R, Silvestri, N, Fazio, R, Van Dijk, G, Garssen, M, Verschuuren, J, Harbo, T, Jacobs, B, Al-Hakem H., Doets A. Y., Stino A. M., Zivkovic S. A., Andersen H., Willison H. J., Cornblath D. R., Gorson K. C., Islam Z., Mohammad Q. D., Sindrup So. H., Kusunoki S., Davidson A., Casasnovas C., Bateman K., Miller J. A. L., Van Den Berg B., Verboon C., Roodbol J., Leonhard S. E., Arends S., Luijten L. W. G., Benedetti L., Kuwabara S., Van Den Bergh P., Monges S., Marfia G. A., Shahrizaila N., Galassi G., Pereon Y., Burmann J., Kuitwaard K., Kleyweg R. P., Marchesoni C., Tous M. J. S., Querol L., Martin-Aguilar L., Wang Y., Nobile-Orazio E., Rinaldi S., Schenone A., Pardo J., Vermeij F. H., Waheed W., Lehmann H. C., Granit V., Stein B., Cavaletti G., Gutierrez-Gutierrez G., Barroso F. A., Visser L. H., Katzberg H. D., Dardiotis E., Attarian S., Van Der Kooi A. J., Eftimov F., Wirtz P. W., Samijn J. P. A., Jacobus Gilhuis H., Hadden R. D. M., Holt J. K. L., Sheikh K. A., Kolb N., Karafiath S., Vytopil M., Antonini G., Feasby T. E., Faber C., Kramers H., Busby M., Roberts R. C., Silvestri N. J., Fazio R., Van Dijk G. W., Garssen M. P. J., Verschuuren J., Harbo T., Jacobs B. C., Al-Hakem, H, Doets, A, Stino, A, Zivkovic, S, Andersen, H, Willison, H, Cornblath, D, Gorson, K, Islam, Z, Mohammad, Q, Sindrup, S, Kusunoki, S, Davidson, A, Casasnovas, C, Bateman, K, Miller, J, Van Den Berg, B, Verboon, C, Roodbol, J, Leonhard, S, Arends, S, Luijten, L, Benedetti, L, Kuwabara, S, Van Den Bergh, P, Monges, S, Marfia, G, Shahrizaila, N, Galassi, G, Pereon, Y, Burmann, J, Kuitwaard, K, Kleyweg, R, Marchesoni, C, Tous, M, Querol, L, Martin-Aguilar, L, Wang, Y, Nobile-Orazio, E, Rinaldi, S, Schenone, A, Pardo, J, Vermeij, F, Waheed, W, Lehmann, H, Granit, V, Stein, B, Cavaletti, G, Gutierrez-Gutierrez, G, Barroso, F, Visser, L, Katzberg, H, Dardiotis, E, Attarian, S, Van Der Kooi, A, Eftimov, F, Wirtz, P, Samijn, J, Jacobus Gilhuis, H, Hadden, R, Holt, J, Sheikh, K, Kolb, N, Karafiath, S, Vytopil, M, Antonini, G, Feasby, T, Faber, C, Kramers, H, Busby, M, Roberts, R, Silvestri, N, Fazio, R, Van Dijk, G, Garssen, M, Verschuuren, J, Harbo, T, Jacobs, B, Al-Hakem H., Doets A. Y., Stino A. M., Zivkovic S. A., Andersen H., Willison H. J., Cornblath D. R., Gorson K. C., Islam Z., Mohammad Q. D., Sindrup So. H., Kusunoki S., Davidson A., Casasnovas C., Bateman K., Miller J. A. L., Van Den Berg B., Verboon C., Roodbol J., Leonhard S. E., Arends S., Luijten L. W. G., Benedetti L., Kuwabara S., Van Den Bergh P., Monges S., Marfia G. A., Shahrizaila N., Galassi G., Pereon Y., Burmann J., Kuitwaard K., Kleyweg R. P., Marchesoni C., Tous M. J. S., Querol L., Martin-Aguilar L., Wang Y., Nobile-Orazio E., Rinaldi S., Schenone A., Pardo J., Vermeij F. H., Waheed W., Lehmann H. C., Granit V., Stein B., Cavaletti G., Gutierrez-Gutierrez G., Barroso F. A., Visser L. H., Katzberg H. D., Dardiotis E., Attarian S., Van Der Kooi A. J., Eftimov F., Wirtz P. W., Samijn J. P. A., Jacobus Gilhuis H., Hadden R. D. M., Holt J. K. L., Sheikh K. A., Kolb N., Karafiath S., Vytopil M., Antonini G., Feasby T. E., Faber C., Kramers H., Busby M., Roberts R. C., Silvestri N. J., Fazio R., Van Dijk G. W., Garssen M. P. J., Verschuuren J., Harbo T., and Jacobs B. C.
Abstract: Background and ObjectivesTo investigate CSF findings in relation to clinical and electrodiagnostic subtypes, severity, and outcome of Guillain-Barré syndrome (GBS) based on 1,500 patients in the International GBS Outcome Study.MethodsAlbuminocytologic dissociation (ACD) was defined as an increased protein level (>0.45 g/L) in the absence of elevated white cell count (<50 cells/L). We excluded 124 (8%) patients because of other diagnoses, protocol violation, or insufficient data. The CSF was examined in 1,231 patients (89%).ResultsIn 846 (70%) patients, CSF examination showed ACD, which increased with time from weakness onset: ≤4 days 57%, >4 days 84%. High CSF protein levels were associated with a demyelinating subtype, proximal or global muscle weakness, and a reduced likelihood of being able to run at week 2 (odds ratio [OR] 0.42, 95% CI 0.25-0.70; p = 0.001) and week 4 (OR 0.44, 95% CI 0.27-0.72; p = 0.001). Patients with the Miller Fisher syndrome, distal predominant weakness, and normal or equivocal nerve conduction studies were more likely to have lower CSF protein levels. CSF cell count was <5 cells/L in 1,005 patients (83%), 5-49 cells/L in 200 patients (16%), and ≥50 cells/L in 13 patients (1%).DiscussionACD is a common finding in GBS, but normal protein levels do not exclude this diagnosis. High CSF protein level is associated with an early severe disease course and a demyelinating subtype. Elevated CSF cell count, rarely ≥50 cells/L, is compatible with GBS after a thorough exclusion of alternative diagnoses.Classification of EvidenceThis study provides Class IV evidence that CSF ACD (defined by the Brighton Collaboration) is common in patients with GBS.
Published: 2023

8. Towards a framework for evaluating the effectiveness of cybersecurity awareness programs

Author: Verschuuren , J., Verschuuren , J., Verschuuren , J., and Verschuuren , J.
Abstract: The full text of this thesis is not available due to privacy or embargo reasons.
Published: 2023

9. De Europees- en internationaalrechtelijke status van de Waddenzee: Een analyse van de relevantie van EU-richtlijnen en internationale verdragen voor de bescherming en het beheer van de Waddenzee met een doorkijk naar de Nederlandse implementatie

Author: van Rijswick, Helena, Bastmeijer, C.J. (Kees), Boerema, Luuk, Gilissen, Herman Kasper, Kistenkas , F., Miltenburg, L., Trouwborst, A., Verschuuren, J., Zwier, W., van Rijswick, Helena, Bastmeijer, C.J. (Kees), Boerema, Luuk, Gilissen, Herman Kasper, Kistenkas , F., Miltenburg, L., Trouwborst, A., Verschuuren, J., and Zwier, W.
Abstract: In nauwe afstemming en samenwerking met de ministeries van LNV en IenW heeft Rijkswaterstaat de Waddenacademie gevraagd een onderzoek uit te voeren naar voor de Waddenzee geldende internationale verdragen en Europese richtlijnen op het gebied van natuur- en waterbescherming. Doelstelling van het onderzoek is om een gedegen overzicht te krijgen van de inhoud en concrete consequenties van de internationale en Europese beschermingsregimes voor de Waddenzee. Wat zijn de doelen die bereikt moeten worden, welke verboden en verplichtingen vloeien er uit verdragen en EU-richtlijnen voort en besteden de beschermingsregimes ook aandacht aan onderwerpen zoals klimaatverandering, cumulatieve effecten, actief natuurherstel en monitoring? Deze en andere vragen worden voor 13 regimes in afzonderlijke hoofdstukken door juridische experts beantwoord en de bevindingen zijn samengebracht in een uitvoerig samenvattend hoofdstuk.
Published: 2023

10. De Europees- en internationaalrechtelijke status van de Waddenzee: Een analyse van de relevantie van EU-richtlijnen en internationale verdragen voor de bescherming en het beheer van de Waddenzee met een doorkijk naar de Nederlandse implementatie

Author: Parel Water en duurzaamheid / UCWOSL, Europees en nationaal waterrecht, van Rijswick, Helena, Bastmeijer, C.J. (Kees), Boerema, Luuk, Gilissen, Herman Kasper, Kistenkas , F., Miltenburg, L., Trouwborst, A., Verschuuren, J., Zwier, W., Parel Water en duurzaamheid / UCWOSL, Europees en nationaal waterrecht, van Rijswick, Helena, Bastmeijer, C.J. (Kees), Boerema, Luuk, Gilissen, Herman Kasper, Kistenkas , F., Miltenburg, L., Trouwborst, A., Verschuuren, J., and Zwier, W.
Published: 2023

11. Europees - en internationaalrechtelijke status van de Waddenzee : Een analyse van de relevantie van EU-richtlijnen en internationale verdragen voor de bescherming en het beheer van de Waddenzee met een doorkijk naar de Nederlandse implementatie

Author: Bastmeijer, K., Boerema, L., Gilissen, H.K., Kistenkas, F., Miltenburg, L., Rijswick, M. van, Trouwborst, A., Verschuuren, J., Zwier, W., Bastmeijer, K., Boerema, L., Gilissen, H.K., Kistenkas, F., Miltenburg, L., Rijswick, M. van, Trouwborst, A., Verschuuren, J., and Zwier, W.
Abstract: In nauwe afstemming en samenwerking met de ministeries van LNV en IenW heeft Rijkswaterstaat de Waddenacademie gevraagd een onderzoek uit te voeren naar voor de Waddenzee geldende internationale verdragen en Europese richtlijnen op het gebied van natuur- en waterbescherming. Doelstelling van het onderzoek is om een gedegen overzicht te krijgen van de inhoud en concrete consequenties van de internationale en Europese beschermingsregimes voor de Waddenzee.
Published: 2023

12. Assessment of left atrial function and diastolic dysfunction in patients with Becker muscular dystrophy

Author: Gegenava, T, primary, Van De Velde, N, additional, Koeks, Z, additional, Spitali, P, additional, Verschuuren, J J G M, additional, Delgado, V, additional, Bax, J, additional, Niks, E H, additional, and Ajmone-Marsan, N, additional
Published: 2022
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13. Breakthrough SARS-CoV-2 infections with the delta (B.1.617.2) variant in vaccinated patients with immune-mediated inflammatory diseases using immunosuppressants: a substudy of two prospective cohort studies

Author: Boekel, L., Stalman, E.W., Wieske, L., Hooijberg, F., Dam, K.P.J. van, Besten, Y.R., Kummer, L.Y., Steenhuis, M., Kempen, Z.L.E. van, Killestein, J., Volkers, A.G., Tas, S.W., Kooi, A.J. van der, Raaphorst, J., Löwenberg, M., Takkenberg, R.B., d'Haens, G., Spuls, P.I., Bekkenk, M.W., Musters, A.H., Post, N.F., Bosma, A.L., Hilhorst, M.L., Vegting, Y., Bemelman, F.J., Voskuyl, A.E., Broens, B., Sanchez, A., Els, C. van, Wit, J. de, Rutgers, A., Leeuw, K. de, Horváth, B., Verschuuren, J., Ruiter, A.M. de, Ouwerkerk, L., Woude, D. van der, Allaart, C.F., Teng, Y.K.O., Paassen, P. van, Busch, M.H., Jallah, P.B.P., Brusse, E., Doorn, P.A. van, Baars, A.E., Hijnen, D.J., Schreurs, C.R.G., Pol, W.Ludo van der, Goedee, H.S., Vogelzang, E.H., Leeuw, M. de, Atiqi, S., Vollenhoven, R. van, Gerritsen, M, Horst-Bruinsma, I.E. van der, Lems, W.F., Nurmohamed, M.T., Boers, Maarten, Keijzer, Sofie, Keijser, J. de, Sandt, C., Boogaard, A., Cristianawati, O., Brinke, A. Ten, Verstegen, N.J., Zwinderman, K.A.H., Ham, S.M. van, Rispens, T., Kuijpers, T.W., Wolbink, G., Eftimov, F., Boekel, L., Stalman, E.W., Wieske, L., Hooijberg, F., Dam, K.P.J. van, Besten, Y.R., Kummer, L.Y., Steenhuis, M., Kempen, Z.L.E. van, Killestein, J., Volkers, A.G., Tas, S.W., Kooi, A.J. van der, Raaphorst, J., Löwenberg, M., Takkenberg, R.B., d'Haens, G., Spuls, P.I., Bekkenk, M.W., Musters, A.H., Post, N.F., Bosma, A.L., Hilhorst, M.L., Vegting, Y., Bemelman, F.J., Voskuyl, A.E., Broens, B., Sanchez, A., Els, C. van, Wit, J. de, Rutgers, A., Leeuw, K. de, Horváth, B., Verschuuren, J., Ruiter, A.M. de, Ouwerkerk, L., Woude, D. van der, Allaart, C.F., Teng, Y.K.O., Paassen, P. van, Busch, M.H., Jallah, P.B.P., Brusse, E., Doorn, P.A. van, Baars, A.E., Hijnen, D.J., Schreurs, C.R.G., Pol, W.Ludo van der, Goedee, H.S., Vogelzang, E.H., Leeuw, M. de, Atiqi, S., Vollenhoven, R. van, Gerritsen, M, Horst-Bruinsma, I.E. van der, Lems, W.F., Nurmohamed, M.T., Boers, Maarten, Keijzer, Sofie, Keijser, J. de, Sandt, C., Boogaard, A., Cristianawati, O., Brinke, A. Ten, Verstegen, N.J., Zwinderman, K.A.H., Ham, S.M. van, Rispens, T., Kuijpers, T.W., Wolbink, G., and Eftimov, F.
Abstract: Item does not contain fulltext, BACKGROUND: Concerns have been raised regarding the risks of SARS-CoV-2 breakthrough infections in vaccinated patients with immune-mediated inflammatory diseases treated with immunosuppressants, but clinical data on breakthrough infections are still scarce. The primary objective of this study was to compare the incidence and severity of SARS-CoV-2 breakthrough infections between patients with immune-mediated inflammatory diseases using immunosuppressants, and controls (patients with immune-mediated inflammatory diseases not taking immunosuppressants and healthy controls) who had received full COVID-19 vaccinations. The secondary objective was to explore determinants of breakthrough infections of the delta (B.1.617.2) variant of SARS-CoV-2, including humoral immune responses after vaccination. METHODS: In this substudy, we pooled data collected in two large ongoing prospective multicentre cohort studies conducted in the Netherlands (Target to-B! [T2B!] study and Amsterdam Rheumatology Center COVID [ARC-COVID] study). Both studies recruited adult patients (age ≥18 years) with immune-mediated inflammatory diseases and healthy controls. We sourced clinical data from standardised electronic case record forms, digital questionnaires, and medical files. We only included individuals who were vaccinated against SARS-CoV-2. For T2B!, participants were recruited between Feb 2 and Aug 1, 2021, and for ARC-COVID, participants were recruited between April 26, 2020, and March 1, 2021. In this study we assessed data on breakthrough infections collected between July 1 and Dec 15, 2021, a period in which the delta SARS-CoV-2 variant was the dominant variant in the Netherlands. We defined a SARS-CoV-2 breakthrough infection as a PCR-confirmed or antigen test-confirmed SARS-CoV-2 infection that occurred at least 14 days after vaccination. All breakthrough infections during this period were assumed to be due to the delta variant due to its dominance during the study period. We analysed
Published: 2022

14. 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

15. 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
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16. International Consensus Guidance for Management of Myasthenia Gravis 2020 Update

Author: Narayanaswami, P, Sanders, DB, Wolfe, G, Benatar, M, Cea, G, Evoli, A, Gilhus, NE, Illa, I, Kuntz, NL, Massey, J, Melms, A, Murai, H, Nicolle, M, Palace, J, Richman, D, and Verschuuren, J
Subjects: education
Abstract: Objective To update the 2016 formal consensus-based guidance for the management of myasthenia gravis (MG) based on the latest evidence in the literature. Methods In October 2013, the Myasthenia Gravis Foundation of America appointed a Task Force to develop treatment guidance for MG, and a panel of 15 international experts was convened. The RAND/UCLA appropriateness method was used to develop consensus recommendations pertaining to 7 treatment topics. In February 2019, the international panel was reconvened with the addition of one member to represent South America. All previous recommendations were reviewed for currency, and new consensus recommendations were developed on topics that required inclusion or updates based on the recent literature. Up to 3 rounds of anonymous e-mail votes were used to reach consensus, with modifications to recommendations between rounds based on the panel input. A simple majority vote (80% of panel members voting "yes") was used to approve minor changes in grammar and syntax to improve clarity. Results The previous recommendations for thymectomy were updated. New recommendations were developed for the use of rituximab, eculizumab, and methotrexate as well as for the following topics: early immunosuppression in ocular MG and MG associated with immune checkpoint inhibitor treatment. Conclusion This updated formal consensus guidance of international MG experts, based on new evidence, provides recommendations to clinicians caring for patients with MG worldwide.
Published: 2021

17. Occurrence of symptoms in different stages of Duchenne muscular dystrophy and their impact on social participation

Author: Houwen, S.L.S., Heutinck, L.B.M., Jansen, M., Krom, Y.D., Cup, E.H.C., Hendriksen, J.G.M., Willemsen, M.A.A.P., Verschuuren, J., Niks, E.H., Groot, I.J.M. de, Houwen, S.L.S., Heutinck, L.B.M., Jansen, M., Krom, Y.D., Cup, E.H.C., Hendriksen, J.G.M., Willemsen, M.A.A.P., Verschuuren, J., Niks, E.H., and Groot, I.J.M. de
Abstract: Item does not contain fulltext, INTRODUCTION/AIMS: As life expectancy improves for patients with Duchenne muscular dystrophy (DMD), new symptoms are likely to arise. This aims of this study are: (1) to explore the prevalence of a broad variety of symptoms in the various stages of DMD (with and without steroid use); (2) to explore the prevalence of common secondary diagnoses; and (3) to evaluate the social participation level of patients with DMD older than 16 y of age; and to explore correlations between social participation and symptoms. METHODS: A cross-sectional self-report questionnaire, including questions on functional level and health status, as well as a standardized participation scale was distributed among Dutch patients with DMD. RESULTS: Eighty-four male patients with a mean age of 22.0 (SD = 10.0) y were enrolled. The most prevalent and limiting symptoms were difficulty coughing (58%), coldness of hands (57%), contractures (51%), stiffness (49%), fatigue (40%), myalgia (38%), and low speech volume (33%). Prevalent secondary diagnoses included cardiac disease (14%), neurobehavioral diagnosis (13%), low blood pressure (13%), and arthrosis (5%). Social participation correlated negatively with coldness of hands (r = - .29; P < .03), decreased intelligibility (r = - .40; P < .003), and chewing problems (r = - .33; P < .02). DISCUSSION: The prevalence of a broad spectrum of symptoms and secondary diagnoses is high in patients with DMD, and some of these symptoms are correlated with social participation. Growing awareness of new symptoms and secondary diagnoses among patients, caregivers, and professionals can enhance their recognition, possibly facilitating prevention and early treatment.
Published: 2021

18. Verdroging in Brabant : Een Europeesrechtelijk Perspectief

Author: Bastmeijer, K., Rijswick, M. van, Verschuuren, J., Bastmeijer, K., Rijswick, M. van, and Verschuuren, J.
Abstract: Verdroging is een probleem dat in Nederland en Noord-Brabant al meer dan 30 jaar wordt waargenomen en ervaren. Een recent onderzoek door Deltares e.a. (hierna: ‘Onderzoek watervraag natuur 2020’ maakt duidelijk dat deze problematiek in Noord-Brabant extra zorgelijk is vanwege een aanzienlijke onbalans tussen aanvulling en onttrekking van grondwater. Met dat onderzoek is ook duidelijk geworden dat momenteel aan de ecologische ‘watervraag’ van de Brabantse natuur niet wordt voldaan. Tegen deze achtergrond geeft dit onderzoek – uitgevoerd in opdracht van Brabantse samenwerkende natuurorganisaties - inzicht in de Europeesrechtelijke verplichtingen die relevant zijn voor verdrogingsbestrijding. Het gaat daarbij primair om het instrumentarium op grond van de EU Vogelrichtlijn en Habitatrichtlijn (het Natura 2000-regime voor gebieden en soortenbescherming), de Kaderrichtlijn Water en het EU klimaatadaptatierecht. Op basis van dit inzicht in verplichtingen wordt vervolgens een grove inschatting gemaakt van de mate waarin de huidige inzet van provincie en waterschappen aan die EU-rechtelijke verplichtingen en verboden voldoet en wat de consequenties zijn van mogelijk strijdigheden of spanningen voor bestaande en nieuwe grondwateronttrekkingen. Tot slot wordt een korte reflectie gegeven op het ontwerp Regionaal Water en Bodem Programma 2022-2027 van de Provincie Noord-Brabant.
Published: 2021

19. International Consensus Guidance for Management of Myasthenia Gravis: 2020 Update

Author: Narayanaswami, P., Sanders, D. B., Wolfe, G., Benatar, M., Cea, G., Evoli Stampanoni-B, Amelia, Gilhus, N. E., Illa, I., Kuntz, N. L., Massey, J., Melms, A., Murai, H., Nicolle, M., Palace, J., Richman, D., Verschuuren, J., Evoli A. (ORCID:0000-0003-0282-8787), Narayanaswami, P., Sanders, D. B., Wolfe, G., Benatar, M., Cea, G., Evoli Stampanoni-B, Amelia, Gilhus, N. E., Illa, I., Kuntz, N. L., Massey, J., Melms, A., Murai, H., Nicolle, M., Palace, J., Richman, D., Verschuuren, J., and Evoli A. (ORCID:0000-0003-0282-8787)
Abstract: OBJECTIVE: To update the 2016 formal consensus-based guidance for the management of myasthenia gravis (MG) based on the latest evidence in the literature. METHODS: In October 2013, the Myasthenia Gravis Foundation of America appointed a Task Force to develop treatment guidance for MG, and a panel of 15 international experts was convened. The RAND/UCLA appropriateness method was used to develop consensus recommendations pertaining to 7 treatment topics. In February 2019, the international panel was reconvened with the addition of one member to represent South America. All previous recommendations were reviewed for currency, and new consensus recommendations were developed on topics that required inclusion or updates based on the recent literature. Up to 3 rounds of anonymous e-mail votes were used to reach consensus, with modifications to recommendations between rounds based on the panel input. A simple majority vote (80% of panel members voting "yes") was used to approve minor changes in grammar and syntax to improve clarity. RESULTS: The previous recommendations for thymectomy were updated. New recommendations were developed for the use of rituximab, eculizumab, and methotrexate as well as for the following topics: early immunosuppression in ocular MG and MG associated with immune checkpoint inhibitor treatment. CONCLUSION: This updated formal consensus guidance of international MG experts, based on new evidence, provides recommendations to clinicians caring for patients with MG worldwide.
Published: 2021

20. 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, DomÃnguez GonzÃ¡lez, 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, GutiÃ©rrez GutiÃ©rrez, 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, LÃ©on Cejas, L, Luciano, C, Lucy, S, Lunn, M, Magot, A, Manji, H, Marchesoni, C, Marfia, G, MÃ¡rquez Infante, C, Martinez Hernandez, E, Mataluni, G, Mattiazi, M, Mcdermott, C, Meekins, G, Miller, J, Monges, M, Montero, M, MorÃs 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-BarrÃ© 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
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21. Pathogenic immune mechanisms at the neuromuscular synapse: the role of specific antibody-binding epitopes in myasthenia gravis

Author: Huijbers, M. G., Lipka, A. F., Plomp, J. J., Niks, E. H., van der Maarel, S. M., and Verschuuren, J. J.
Published: 2014
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22. 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
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23. Novel free-circulating and extracellular vesicle-derived miRNAs dysregulated in Duchenne muscular dystrophy

Author: Catapano, F., Scaglioni, D., Maresh, K., Ala, P., Domingos, J., Selby, V., Ricotti, V., Phillips, L., Servais, L., Seferian, A., Groot, I.J. de, Krom, Y.D., Voit, T., Verschuuren, J., Niks, E.H., Straub, V., Morgan, J., Muntoni, F., Catapano, F., Scaglioni, D., Maresh, K., Ala, P., Domingos, J., Selby, V., Ricotti, V., Phillips, L., Servais, L., Seferian, A., Groot, I.J. de, Krom, Y.D., Voit, T., Verschuuren, J., Niks, E.H., Straub, V., Morgan, J., and Muntoni, F.
Abstract: Item does not contain fulltext, Aim: To perform cross-sectional and longitudinal miRNA profiling in plasma from Duchenne muscular dystrophy (DMD) subjects and find non-invasive biomarkers in DMD. Subjects/materials & methods: Plasma was collected from 14 age and sex matched controls and 46 DMD subjects. Free-circulating and extracellular vesicle (EV)-derived miRNA expression was measured by RT-qPCR. Results: Free-circulating and EVs derived miR-29c-3p and miR-133a-3p are dysregulated in DMD subjects. Free-circulating and EV-derived miR-29c-3p are reduced in DMD subjects undergoing daily corticosteroid treatment. Free-circulating miR-1-3p and miR-122-5p are longitudinally upregulated in ambulant DMD subjects. Conclusion: We detected novel free-circulating and EV-derived dysregulated miRNAs in plasma from DMD subjects and characterized the longitudinal profile of free-circulating miRNA on plasma from DMD subjects.
Published: 2020

24. Lowering the cutoff value for increment increases the sensitivity for the diagnosis of Lambert-Eaton myasthenic syndrome

Author: Lipka, AF, Titulaer, Maarten, Tannemaat, MR, Verschuuren, J, Lipka, AF, Titulaer, Maarten, Tannemaat, MR, and Verschuuren, J
Published: 2020

25. Minimal manifestation status and prednisone withdrawal in the MGTX trial

Author: Lee, I., Kuo, H. -C., Aban, I. B., Cutter, G. R., McPherson, T., Kaminski, H. J., Sussman, J., Strobel, P., Oger, J., Cea, G., Heckmann, J. M., Evoli, A., Nix, W., Ciafaloni, E., Antonini, G., Witoonpanich, R., King, J. O., Beydoun, S. R., Chalk, C. H., Barboi, A. C., Amato, A. A., Shaibani, A. I., Katirji, B., Lecky, B. R. F., Buckley, C., Vincent, A., Dias-Tosta, E., Yoshikawa, H., Waddington-Cruz, M., Pulley, M. T., Rivner, M. H., Kostera-Pruszczyk, A., Pascuzzi, R. M., Jackson, C. E., Verschuuren, J. J. G., Massey, J. M., Kissel, J. T., Werneck, L. C., Benatar, M., Barohn, R. J., Tandan, R., Mozaffar, T., Conwit, R., Minisman, G., Sonett, J. R., Wolfe, G. I., Evoli A. (ORCID:0000-0003-0282-8787), Lee, I., Kuo, H. -C., Aban, I. B., Cutter, G. R., McPherson, T., Kaminski, H. J., Sussman, J., Strobel, P., Oger, J., Cea, G., Heckmann, J. M., Evoli, A., Nix, W., Ciafaloni, E., Antonini, G., Witoonpanich, R., King, J. O., Beydoun, S. R., Chalk, C. H., Barboi, A. C., Amato, A. A., Shaibani, A. I., Katirji, B., Lecky, B. R. F., Buckley, C., Vincent, A., Dias-Tosta, E., Yoshikawa, H., Waddington-Cruz, M., Pulley, M. T., Rivner, M. H., Kostera-Pruszczyk, A., Pascuzzi, R. M., Jackson, C. E., Verschuuren, J. J. G., Massey, J. M., Kissel, J. T., Werneck, L. C., Benatar, M., Barohn, R. J., Tandan, R., Mozaffar, T., Conwit, R., Minisman, G., Sonett, J. R., Wolfe, G. I., and Evoli A. (ORCID:0000-0003-0282-8787)
Abstract: OBJECTIVE: To examine whether sustained minimal manifestation status (MMS) with complete withdrawal of prednisone is better achieved in thymectomized patients with myasthenia gravis (MG). METHODS: This study is a post hoc analysis of data from a randomized trial of thymectomy in MG (Thymectomy Trial in Non-Thymomatous Myasthenia Gravis Patients Receiving Prednisone Therapy [MGTX]). MGTX was a multicenter, randomized, rater-blinded 3-year trial that was followed by a voluntary 2-year extension for patients with acetylcholine receptor (AChR) antibody-positive MG without thymoma. Patients were randomized 1:1 to thymectomy plus prednisone vs prednisone alone. Participants were age 18-65 years at enrollment with disease duration less than 5 years. All patients received oral prednisone titrated up to 100 mg on alternate days until they achieved MMS, which prompted a standardized prednisone taper as long as MMS was maintained. The achievement rate of sustained MMS (no symptoms of MG for 6 months) with complete withdrawal of prednisone was compared between the thymectomy plus prednisone and prednisone alone groups. RESULTS: Patients with MG in the thymectomy plus prednisone group achieved sustained MMS with complete withdrawal of prednisone more frequently (64% vs 38%) and quickly compared to the prednisone alone group (median time 30 months vs no median time achieved, p < 0.001) over the 5-year study period. Prednisone-associated adverse symptoms were more frequent in the prednisone alone group and distress level increased with higher doses of prednisone. CONCLUSIONS: Thymectomy benefits patients with MG by increasing the likelihood of achieving sustained MMS with complete withdrawal of prednisone. CLINICALTRIALSGOV IDENTIFIER: NCT00294658. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that for patients with generalized MG with AChR antibody, those receiving thymectomy plus prednisone are more likely to attain sustained MMS and complete prednisone wi
Published: 2020

26. Duchenne/Becker muscular dystrophy in the family: have potential carriers been tested at a molecular level?

Author: Helderman-van den Enden, A TJM, van den Bergen, J C, Breuning, M H, Verschuuren, J JGM, Tibben, A, Bakker, E, and Ginjaar, H B
Published: 2011
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27. LATE-ONSET EPILEPSY, MILD COGNITIVE IMPAIRMENT AND ANTI-VOLTAGE-GATED POTASSIUM (VGKC) ANTIBODIES: AN OBSERVATIONAL STUDY ON THE EFFECTS OF AN ORAL PREDNISOLONE COURSE: 041

Author: Thijs, R D, Verschuuren, J J, and Sander, J W
Published: 2010

28. Second IVIg course in Guillain-Barre syndrome patients with poor prognosis (SID-GBS trial): Protocol for a double-blind randomized, placebo-controlled clinical trial

Author: Walgaard, Christa, Jacobs, Bart C., Lingsma, Hester F., Steyerberg, Ewout W., Cornblath, David R., van Doorn, Pieter A., de Wit, M. C. Y., van den Berg, B., Doets, A. Y., Leonhard, S. E., Verboon, J. C., van Woerkom, M., Tio-Gillen, A. P., van Rijs, W., Huizinga, H., Badrising, U. A., Bienfait, H. M. E., Blom, R. J., van Boheemen, C. J. M., Breukelman, A. J., Bronner, I. M., Dieks, H. J. G., van Dijk, G. W., van Engelen, B. G. M., Faber, C. G., Feenstra, B., Fokke, C., Garssen, M. P. J., Gijsbers, C. J., Gilhuis, H. J., van der Graaff, M. M., Groen, R. J., Hoogendoorn, T. A., Hovestad, A., Jansen, P. J. H. W., Jellema, K., Keuter, E., Kleyweg, R. P., van Koningsveld, R., van der Kooi, A. J., van der Kooi, E. L., Krudde, J., Kuks, J. B. M., Kuitwaard, K., Linssen, W. H. J. P., Lion, J., Lovenich, H., Manschot, S. M., Mellema, S. J., Merkies, I. S. J., van der Meulen, M. F. G., van der Meulen, W. D. M., Molenaar, D. S. M., Oenema, D. G., van Oosten, B. W., van Oostrom, J. C. H., van Orshoven, N. P., van der Ploeg, R. J. O., van der Pol, W. L., Polman, S., van der Ree, T. C., de Rijk, M. C., Ruitenberg, A., Ruts, L., Samijn, J. P. A., Schyns-Soeterboek, A. J. G. M., Stevens, M., Vermeij, F. H., Verschuuren, J. J. G. M., Visser, L. H., Wirtz, P. W., Wohlgemuth, M., Zwetsloot, C. P., Dippel, D. W. J., Hintzen, R. Q., Anesthesiology, Neurology, Amsterdam Neuroscience - Neuroinfection & -inflammation, AII - Infectious diseases, ANS - Neuroinfection & -inflammation, Immunology, and Public Health
Subjects: Male, IVIg, Disease, Guillain-Barre syndrome, Disability Evaluation, 0302 clinical medicine, hemic and lymphatic diseases, 030212 general & internal medicine, Hospital Mortality, Netherlands, Randomized Controlled Trials as Topic, treatment, General Neuroscience, Standard treatment, Immunoglobulins, Intravenous, Middle Aged, trial, Disorders of movement Donders Center for Medical Neuroscience [Radboudumc 3], Prognosis, Intensive Care Units, Treatment Outcome, Research Design, Female, Adult, medicine.medical_specialty, Poor prognosis, Randomization, Adolescent, Placebo, 03 medical and health sciences, Young Adult, All institutes and research themes of the Radboud University Medical Center, Double-Blind Method, Internal medicine, medicine, Humans, Muscle Strength, protocol, Adverse effect, Aged, business.industry, Patient Selection, Recovery of Function, Length of Stay, medicine.disease, Respiration, Artificial, Clinical trial, Immunoglobulin G, Neurology (clinical), business, 030217 neurology & neurosurgery
Abstract: One course of intravenous immunoglobulins (IVIg) of 2 g/kg is standard treatment in Guillain-Barré syndrome (GBS) patients unable to walk independently. Despite treatment some patients recover poorly, in part related to rapid consumption of IVIg, indicating that they may benefit from a second course of IVIg. The aim of the study is to determine whether a second course of IVIg, administered 1 week after start of the first course in patients with GBS and predicted poor outcome improves functional outcome on the GBS disability scale after 4 weeks. Secondary outcome measures include adverse events (AEs), Medical Research Council sumscore and GBS disability score after 8, 12, and 26 weeks, length of hospital and ICU admission, mortality, and changes in serum IgG levels. GBS patients of 12 years and older with a poor prognosis, based on the modified Erasmus GBS outcome score (mEGOS) at 1 week after start of the first IVIg course are eligible for randomization in this double-blind, placebo-controlled (IVIg or albumin) clinical trial. This study will determine if a second course of IVIg administered in the acute phase of the disease is safe, feasible, and effective in patients with GBS and a poor prognosis. This Dutch trial is registered prospectively as NTR 2224 in the Netherlands National Trial Register (NTR) which is the Primary Registry in the WHO Registry Network for the Netherlands.
Published: 2018
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29. Late onset axonal Charcot-Marie-Tooth phenotype caused by a novel myelin protein zero mutation

Author: Bienfait, H M E, Faber, C G, Baas, F, Gabreëls-Festen, A A W M, Koelman, J H T M, Hoogendijk, J E, Verschuuren, J J, Wokke, J H J, and de Visser, M
Published: 2006

30. De val van het Programma Aanpak Stikstof. Van ontwikkelingsruimte naar ruim baan voor bron- en herstelmaatregelen?

Author: Verschuuren, J., Frins, R.H.W., Verschuuren, J., and Frins, R.H.W.
Abstract: Item does not contain fulltext
Published: 2019

31. Respiratory and upper limb function as outcome measures in ambulant and non-ambulant subjects with Duchenne muscular dystrophy: A prospective multicentre study

Author: Ricotti, V., Selby, V., Ridout, D., Domingos, J., Decostre, V., Mayhew, A., Eagle, M., Butler, J., Guglieri, M., Holst, M., Jansen, M., Verschuuren, J., Groot, I.J.M. de, Niks, E.H., Servais, L., Straub, V., Voit, T., Hogrel, J.Y., Muntoni, F., Ricotti, V., Selby, V., Ridout, D., Domingos, J., Decostre, V., Mayhew, A., Eagle, M., Butler, J., Guglieri, M., Holst, M., Jansen, M., Verschuuren, J., Groot, I.J.M. de, Niks, E.H., Servais, L., Straub, V., Voit, T., Hogrel, J.Y., and Muntoni, F.
Abstract: Item does not contain fulltext, The field of translational research in Duchenne muscular dystrophy (DMD) has been transformed in the last decade by a number of therapeutic targets, mostly studied in ambulant patients. A paucity of studies focus on measures that capture the non-ambulant stage of the disease, and the transition between the ambulant and non-ambulant phase. In this prospective natural history study, we report the results of a comprehensive assessment of respiratory, upper limb function and upper limb muscle strength in a group of 89 DMD boys followed in 3 European countries, 81 receiving corticosteroids, spanning a wide age range (5-18 years) and functional abilities, from ambulant (n=60) to non-ambulant (n=29). Respiratory decline could be detected in the early ambulatory phase using Peak Expiratory Flow percentage predicted (PEF%), despite glucocorticoid use (mean annual decline: 4.08, 95% CI [-7.44,-0.72], p=0.02 in ambulant; 4.81, 95% CI [-6.79,-2.82], p < 0.001 in non-ambulant). FVC% captured disease progression in non-ambulant DMD subjects, with an annual loss of 5.47% (95% CI [-6.48,-4.45], p < 0.001). Upper limb function measured with the Performance of Upper Limb (PUL 1.2) showed an annual loss of 4.13 points (95% CI [-4.79,3.47], p < 0.001) in the non-ambulant cohort. Measures of upper limb strength (MyoGrip and MyoPinch) showed a continuous decline independent of the ambulatory status, when reported as percentage predicted (grip force -5.51%, 95% CI [-6.54,-4.48], p < 0.001 in ambulant and a slower decline -2.86%; 95% CI -3.29,-2.43, p < 0.001, in non-ambulant; pinch force: -2.66%, 95% CI [-3.82,-1.51], p < 0.001 in ambulant and -2.23%, 95% CI [-2.92,-1.53], p < 0.001 in non-ambulant). Furthermore, we also explored the novel concept of a composite endpoint by combining respiratory, upper limb function and force domains: we were able to identify clear clinical progression in patients in whom an isolated measurement of only one of these domains failed to appreciate the yearly
Published: 2019

32. Current treatment practice of Guillain-Barre syndrome

Author: Verboon, Christine, Doets, Alex Y., Galassi, Giuliana, Davidson, Amy, Waheed, Waqar, Pereon, Yann, Shahrizaila, Nortina, Kusunoki, Susumu, Lehmann, Helmar C., Harbo, Thomas, Monges, Soledad, Van den Bergh, Peter, Willison, Hugh J., Cornblath, David R., Jacobs, Bart C., 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, Christine, Doets, Alex Y., Galassi, Giuliana, Davidson, Amy, Waheed, Waqar, Pereon, Yann, Shahrizaila, Nortina, Kusunoki, Susumu, Lehmann, Helmar C., Harbo, Thomas, Monges, Soledad, Van den Bergh, Peter, Willison, Hugh J., Cornblath, David R., Jacobs, Bart C., 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: Objective To define the current treatment practice of Guillain-Barre syndrome (GBS). Methods The 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. Results We 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. Conclusions In 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

33. The expanded clinical spectrum of anti-GABA(B)R encephalitis and added value of KCTD16 autoantibodies

Author: Hameete, Marleen, de Bruijn, Marienke, Graaff, E, Bastiaansen, D, Schreurs, Marco, Demmers, Jeroen, Ramberger, M, Hulsenboom, Esther, Nagtzaam, Mariska, Boukhrissi, S, Veldink, JH, Verschuuren, J, Hoogenraad, CC, Sillevis Smitt, Peter, Titulaer, Maarten, Hameete, Marleen, de Bruijn, Marienke, Graaff, E, Bastiaansen, D, Schreurs, Marco, Demmers, Jeroen, Ramberger, M, Hulsenboom, Esther, Nagtzaam, Mariska, Boukhrissi, S, Veldink, JH, Verschuuren, J, Hoogenraad, CC, Sillevis Smitt, Peter, and Titulaer, Maarten
Abstract: In this study we report the clinical features of 32 patients with gamma aminobutyric acid B receptor (GABABR) antibodies, identify additional autoantibodies in patients with anti-GABABR encephalitis that mark the presence of an underlying small cell lung carcinoma and optimize laboratory methods for the detection of GABABR antibodies. Patients (n = 3225) were tested for the presence of GABABR antibodies using cell-based assay, immunohistochemistry and live hippocampal neurons. Clinical data were obtained retrospectively. Potassium channel tetramerization domain-containing (KCTD)16 antibodies were identified by immunoprecipitation, mass spectrometry analysis and cell-based assays. KCTD16 antibodies were identified in 23/32 patients with anti-GABABR encephalitis, and in 1/26 patients with small cell lung carcinoma and Hu antibodies, but not in 329 healthy subjects and disease controls. Of the anti-GABABR encephalitis patients that were screened sufficiently, 18/19 (95%) patients with KCTD16 antibodies had a tumour versus 3/9 (33%) anti-GABABR encephalitis patients without KCTD16 antibodies (P = 0.001). In most cases this was a small cell lung carcinoma. Patients had cognitive or behavioural changes (97%) and prominent seizures (90%). Thirteen patients developed a refractory status epilepticus with intensive care unit admittance (42%). Strikingly, 4/32 patients had a rapidly progressive dementia. The addition of KCTD16 to the GABABR cell-based assay improved sensitivity of the in-house fixed cell-based assay, without loss of specificity. Twenty-two of 26 patients improved (partially) to immunotherapy or chemotherapy. Anti-GABABR encephalitis is a limbic encephalitis with prominent, severe seizures, but patients can also present with rapidly progressive dementia. The co-occurrence of KCTD16 antibodies points towards a paraneoplastic origin. The addition of KCTD16 improves the sensitivity of the cell-based assay.
Published: 2019

34. Recommended diagnostic criteria for paraneoplastic neurological syndromes

Author: Graus, F, Delattre, J Y, Antoine, J C, Dalmau, J, Giometto, B, Grisold, W, Honnorat, J, Smitt, P Sillevis, Vedeler, Ch, Verschuuren, J J G M, Vincent, A, and Voltz, R
Published: 2004

35. Difference in distribution of muscle weakness between myasthenia gravis and the Lambert-Eaton myasthenic syndrome

Author: Wirtz, P W, Sotodeh, M, Nijnuis, M, van Doorn, P A, van Engelen, B G M, Hintzen, R Q, de Kort, P L M, Kuks, J B, Twijnstra, A, de Visser, M, Visser, L H, Wokke, J H, Wintzen, A R, and Verschuuren, J J
Published: 2002

36. CNS involvement in systemic lupus erythematosus: a case with remarkable histopathological findings

Author: Rood, M J, Haverman, J F, van Duinen, S G, Breedveld, F C, Verschuuren, J J G M, and Huizinga, T W J
Published: 2001

37. Facilitating orphan drug development: Proceedings of the TREAT-NMD International Conference, December 2015, Washington, DC, USA

Author: Hoffman, E.P., Bonnemann, C., Boutin, M., Brais, B., Buccella, F., Burghes, A., Coffey, C., Dasgupta, N., Dawkins, H., Luca, A. de, Dowd, C., Duong, T., Eagle, M., Finkel, R., Furlong, P., Gagnon, C., Goemans, N., Guglieri, M., Hathout, Y., Johnson, N., Kakkis, E., Kaufmann, P., Kimmelman, J., Korngut, L., Kullman, J., Lochmuller, H., Marini, S., McDonald, C., Mohan, C., Morgenroth, L., Morizono, H., Nagaraju, K., Porter, J., Reilly, L., Ruegg, M., Schneider, J., Spitali, P., Straub, V., Sweeney, L., Tasca, G., Turner, C., Veldhuizen, O., Verschuuren, J., Ward, S., Willmann, R., and TREAT-NMD Alliance
Subjects: 0301 basic medicine, medicine.medical_specialty, Orphan Drug Production, business.industry, MEDLINE, Neuromuscular Diseases, Congresses as Topic, Orphan drug, 03 medical and health sciences, 030104 developmental biology, 0302 clinical medicine, Neurology, Family medicine, Pediatrics, Perinatology and Child Health, District of Columbia, Medicine, Humans, Neurology (clinical), business, 030217 neurology & neurosurgery, Genetics (clinical)
Published: 2017

38. Evaluation of serum MMP-9 as predictive biomarker for antisense therapy in Duchenne

Author: Lourbakos, A., primary, Yau, N., additional, de Bruijn, P., additional, Hiller, M., additional, Kozaczynska, K., additional, Jean-Baptiste, R., additional, Reza, M., additional, Wolterbeek, R., additional, Koeks, Z., additional, Ayoglu, B., additional, de Klerk, D., additional, Campion, G., additional, Zaharieva, I., additional, Nadarajah, V. D., additional, Nilsson, P., additional, Al-Khalili Szigyarto, C., additional, Muntoni, F., additional, Lochmüller, H., additional, Verschuuren, J. J., additional, Goemans, N., additional, Tulinius, M., additional, Niks, E. H., additional, de Kimpe, S., additional, Aartsma-Rus, A., additional, ’t Hoen, Peter A. C., additional, and Spitali, P., additional
Published: 2017
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39. Evaluation of serum MMP-9 as predictive biomarker for antisense therapy in Duchenne

Author: Lourbakos, A., Yau, N., de Bruijn, P., Hiller, M., Kozaczynska, K., Jean-Baptiste, R., Reza, M., Wolterbeek, R., Koeks, Z., Ayoglu, Burcu, de Klerk, D., Campion, G., Zaharieva, I., Nadarajah, V. D., Nilsson, P., Al-Khalili Szigyarto, Cristina, Muntoni, F., Lochmuller, H., Verschuuren, J. J., Goemans, N., Tulinius, M., Niks, E. H., de Kimpe, S., Aartsma-Rus, A., 't Hoen, Peter A. C., Spitali, P., Lourbakos, A., Yau, N., de Bruijn, P., Hiller, M., Kozaczynska, K., Jean-Baptiste, R., Reza, M., Wolterbeek, R., Koeks, Z., Ayoglu, Burcu, de Klerk, D., Campion, G., Zaharieva, I., Nadarajah, V. D., Nilsson, P., Al-Khalili Szigyarto, Cristina, Muntoni, F., Lochmuller, H., Verschuuren, J. J., Goemans, N., Tulinius, M., Niks, E. H., de Kimpe, S., Aartsma-Rus, A., 't Hoen, Peter A. C., and Spitali, P.
Abstract: Duchenne Muscular Dystrophy (DMD) is a severe muscle disorder caused by lack of dystrophin. Predictive biomarkers able to anticipate response to the therapeutic treatments aiming at dystrophin re-expression are lacking. The objective of this study is to investigate Matrix Metalloproteinase-9 (MMP-9) as predictive biomarker for Duchenne. Two natural history cohorts were studied including 168 longitudinal samples belonging to 66 patients. We further studied 1536 samples obtained from 3 independent clinical trials with drisapersen, an antisense oligonucleotide targeting exon 51: an open label study including 12 patients; a phase 3 randomized, double blind, placebo controlled study involving 186 patients; an open label extension study performed after the phase 3. Analysis of natural history cohorts showed elevated MMP-9 levels in patients and a significant increase over time in longitudinal samples. MMP-9 decreased in parallel to clinical stabilization in the 12 patients involved in the open label study. The phase 3 study and subsequent extension study clarified that the decrease in MMP-9 levels was not predictive of treatment response. These data do not support the inclusion of serum MMP-9 as predictive biomarker for DMD patients., QC 20180111
Published: 2017
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40. Antibodies to TRIM46 are associated with paraneoplastic neurological syndromes

Author: Hameete, Marleen, van Beuningen, SFB, Perrenoud, M, Will, LM, Hulsenboom, Esther, Demonet, JF, Sabater, L, Kros, J.M., Verschuuren, J, Titulaer, Maarten, Graaff, E, Sillevis Smitt, Peter, Hoogenraad, CC, Hameete, Marleen, van Beuningen, SFB, Perrenoud, M, Will, LM, Hulsenboom, Esther, Demonet, JF, Sabater, L, Kros, J.M., Verschuuren, J, Titulaer, Maarten, Graaff, E, Sillevis Smitt, Peter, and Hoogenraad, CC
Published: 2017

41. 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, DomÃnguez GonzÃ¡lez, 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, GutiÃ©rrez GutiÃ©rrez, 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, LÃ©on Cejas, L, Luciano, C, Lucy, S, Lunn, M, Magot, A, Manji, H, Marchesoni, C, Marfia, G, MÃ¡rquez Infante, C, Martinez Hernandez, E, Mataluni, G, Mattiazi, M, Mcdermott, C, Meekins, G, Miller, J, Monges, M, Montero, M, MorÃs 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, DomÃnguez GonzÃ¡lez, 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, GutiÃ©rrez GutiÃ©rrez, 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, LÃ©on Cejas, L, Luciano, C, Lucy, S, Lunn, M, Magot, A, Manji, H, Marchesoni, C, Marfia, G, MÃ¡rquez Infante, C, Martinez Hernandez, E, Mataluni, G, Mattiazi, M, Mcdermott, C, Meekins, G, Miller, J, Monges, M, Montero, M, MorÃs 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

42. Randomized Trial of Thymectomy in Myasthenia Gravis

Author: Wolfe, Gi, Kaminski, Hj, Aban, Ib, Minisman, G, Kuo, Hc, Marx, A, Ströbel, P, Mazia, C, Oger, J, Cea, Jg, Heckmann, Jm, Evoli, A, Nix, W, Ciafaloni, E, Antonini, G, Witoonpanich, R, King, Jo, Beydoun, Sr, Chalk, Ch, Barboi, Ac, Amato, Aa, Shaibani, Ai, Katirji, B, Lecky, Br, Buckley, C, Vincent, A, Dias Tosta, E, Yoshikawa, H, Waddington Cruz, M, Pulley, Mt, Rivner, Mh, Kostera Pruszczyk, A, Pascuzzi, Rm, Jackson, Ce, Garcia Ramos GS, Verschuuren, Jj, Massey, Jm, Kissel, Jt, Werneck, Lc, Benatar, M, Barohn, Rj, Tandan, R, Mozaffar, T, Conwit, R, Odenkirchen, J, Sonett, Jr, 3rd, Jaretzki A., Newsom Davis, J, Cutter, Gr, MGTX study group including Cutter GR, Feese, M, Saluto, V, Rosenberg, M, Alvarez, V, Rey, L, King, J, Butzkueven, H, Goldblatt, J, Carey, J, Pollard, J, Reddel, S, Handel, N, Mccaughan, B, Pallot, L, Novis, R, Boasquevisque, C, Morato Fernandez, R, Ximenes, M, Werneck, L, Scola, R, Soltoski, P, Chalk, C, Moore, F, Mulder, D, Wadup, L, Mezei, M, Evans, K, Jiwa, T, Schaffar, A, White, C, Toth, C, Gelfand, G, Wood, S, Pringle, E, Zwicker, J, Maziak, D, Shamji, F, Sundaresan, S, Seely, A, Cea, G, Verdugo, R, Aguayo, A, Jander, S, Zickler, P, Klein, M, Weis, Ca, Melms, A, Bischof, F, Aebert, H, Ziemer, G, Thümler, B, Wilhem Schwenkmezger, T, Mayer, E, Schalke, B, Pöschel, P, Hieber, G, Wiebe, K, Clemenzi, A, Ceschin, V, Rendina, E, Venuta, F, Morino, S, Bucci, E, Durelli, Luca, Tavella, A, Clerico, Marinella, Contessa, G, Borasio, P, Servidei, S, Granone, P, Mantegazza, R, Berta, E, Novellino, L, Spinelli, L, Motomura, M, Matsuo, H, Nagayasu, T, Takamori, M, Oda, M, Matsumoto, I, Furukawa, Y, Noto, D, Motozaki, Y, Iwasa, K, Yanase, D, Ramos, Gg, Cacho, B, de la Garza, L, Lipowska, M, Kwiecinski, H, Potulska Chromik, A, Orlowski, T, Silva, A, Feijo, M, Freitas, A, Heckmann, J, Frost, A, Pan, El, Tucker, L, Rossouw, J, Drummond, F, Illa, I, Diaz, J, Leon, C, Yeh, Jh, Chiu, Hc, Hsieh, Ys, Tunlayadechanont, S, Attanavanich, S, Verschuuren, J, Straathof, C, Titulaer, M, Versteegh, M, Pels, A, Krum, Y, Leite, M, Hilton Jones, D, Ratnatunga, C, Farrugia, Me, Petty, R, Overell, J, Kirk, A, Gibson, A, Mcdermott, C, Hopkinson, D, Lecky, B, Watling, D, Marshall, D, Saminaden, S, Davies, D, Dougan, C, Sathasivam, S, Page, R, Sussman, J, Ealing, J, Krysiak, P, Amato, A, Salajegheh, M, Jaklitsch, M, Roe, K, Ashizawa, T, Smith, Rg, Zwischenberg, J, Stanton, P, Barboi, A, Jaradeh, S, Tisol, W, Gasparri, M, Haasler, G, Yellick, M, Dennis, C, Barohn, R, Pasnoor, M, Dimachkie, M, Mcvey, A, Gronseth, G, Dick, A, Kramer, J, Currence, M, Herbelin, L, Belsh, J, Li, G, Langenfeld, J, Mertz, Ma, Harrison, T, Force, S, Usher, S, Beydoun, S, Lin, F, Demeester, S, Akhter, S, Malekniazi, A, Avenido, G, Crum, B, Milone, M, Cassivi, S, Fisher, J, Heatwole, C, Watson, T, Hilbert, J, Smirnow, A, Distad, B, Weiss, M, Wood, D, Haug, J, Ernstoff, R, Cao, J, Chmielewski, G, Welsh, R, Duris, R, Gutmann, L, Pawar, G, Graeber, Gm, Altemus, P, Nance, C, Jackson, C, Grogan, P, Calhoon, J, Kittrell, P, Myers, D, Kaminski, H, Hayat, G, Naunheim, K, Eller, S, Holzemer, E, Alshekhlee, A, Robke, J, Karlinchak, B, Katz, J, Miller, R, Roan, R, Forshew, D, Kissel, J, Elsheikh, B, Ross, P, Chelnick, S, Lewis, R, Acsadi, A, Baciewicz, F, Masse, S, Massey, J, Juel, V, Onaitis, M, Lowe, J, Lipscomb, B, Thai, G, Milliken, J, Martin, V, Karayan, R, Muley, S, Parry, G, Shumway, S, Oh, S, Claussen, G, Lu, L, Cerfolio, R, Young, A, Morgan, M, Pascuzzi, R, Kincaid, J, Kesler, K, Guingrich, S, Michaels, A, Phillips, L, Burns, T, Jones, D, Fischer, C, Pulley, M, Berger, A, D'Agostino, H, Smith, L, Rivner, M, Pruitt, J, Landolfo, K, Hillman, D, Shaibani, A, Sermas, A, Ruel, R, Ismail, F, Sivak, M, Goldstein, M, Camunas, J, Bratton, J, Panitch, H, Leavitt, B, Jones, M, Wolfe, G, Muppidi, S, Vernino, S, Nations, S, Meyer, D, and Gorham, N.
Subjects: Male, medicine, medicine.medical_treatment, 030204 cardiovascular system & hematology, Medical and Health Sciences, Severity of Illness Index, law.invention, 0302 clinical medicine, Randomized controlled trial, law, Prednisone, Adolescent, Adult, Aged, Combined Modality Therapy, Female, Glucocorticoids, Hospitalization, Humans, Middle Aged, Myasthenia Gravis, Single-Blind Method, Treatment Outcome, Young Adult, Thymectomy, Medicine (all), Young adult, MGTX Study Group, General Medicine, Settore MED/26 - NEUROLOGIA, 6.1 Pharmaceuticals, medicine.drug, medicine.medical_specialty, Clinical Trials and Supportive Activities, Autoimmune Disease, 03 medical and health sciences, Rare Diseases, Clinical Research, General & Internal Medicine, Internal medicine, Severity of illness, business.industry, Neurosciences, Evaluation of treatments and therapeutic interventions, Retrospective cohort study, medicine.disease, Myasthenia gravis, Surgery, Clinical research, adolescent, adult, aged, combined modality therapy, female, glucocorticoids, hospitalization, humans, male, middle aged, myasthenia gravis, prednisone, severity of Illness index, single-blind method, treatment outcome, young adult, thymectomy, business, 030217 neurology & neurosurgery
Abstract: BackgroundThymectomy has been a mainstay in the treatment of myasthenia gravis, but there is no conclusive evidence of its benefit. We conducted a multicenter, randomized trial comparing thymectomy plus prednisone with prednisone alone.MethodsWe compared extended transsternal thymectomy plus alternate-day prednisone with alternate-day prednisone alone. Patients 18 to 65 years of age who had generalized nonthymomatous myasthenia gravis with a disease duration of less than 5 years were included if they had Myasthenia Gravis Foundation of America clinical class II to IV disease (on a scale from I to V, with higher classes indicating more severe disease) and elevated circulating concentrations of acetylcholine-receptor antibody. The primary outcomes were the time-weighted average Quantitative Myasthenia Gravis score (on a scale from 0 to 39, with higher scores indicating more severe disease) over a 3-year period, as assessed by means of blinded rating, and the time-weighted average required dose of prednisone over a 3-year period.ResultsA total of 126 patients underwent randomization between 2006 and 2012 at 36 sites. Patients who underwent thymectomy had a lower time-weighted average Quantitative Myasthenia Gravis score over a 3-year period than those who received prednisone alone (6.15 vs. 8.99, P
Published: 2016
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43. Sustainable development and the nature of environmental legal principles

Author: Verschuuren, J, primary
Published: 2017
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44. Spatially localized phosphorous metabolism of skeletal muscle in Duchenne muscular dystrophy patients: 24–month follow-up

Author: Hooijmans, M. T., primary, Doorenweerd, N., additional, Baligand, C., additional, Verschuuren, J. J. G. M., additional, Ronen, I., additional, Niks, E. H., additional, Webb, A. G., additional, and Kan, H. E., additional
Published: 2017
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45. G.P.147 - Outcome measures for Duchenne muscular dystrophy from ambulant to non-ambulant: implications for clinical trials

Author: Ricotti, V, Eagle, M, Butler, J, Decostre, V, Deborah, R, Moraux, A, Anthony, Karen, Sleby, V, Guglieri, M, Van der Holst, M, Jansen, M, Morgan, J, de Groot, I, Niks, E, Verschuuren, J, Servais, L, Hogrel, J Y, Voit, T, Straub, V, and Muntoni, F
Subjects: musculoskeletal diseases, congenital, hereditary, and neonatal diseases and abnormalities
Abstract: Novel emerging therapies for Duchenne muscular dystrophy (DMD), such as antisense oligomer (AO) mediated exon skipping, have generated the need of understanding the natural history study of the targeted genotype subgroups. Most natural history studies are focused on ambulant subjects; therefore very little data exists on non-ambulant DMD. Specifically targeting skippable deletions, we aim to assess the natural history of DMD through a composite assessment tool capable of capturing disease progression beyond loss of ambulation.
Published: 2015

46. The TREAT-NMD DMD global database: Analysis of more than 7,000 duchenne muscular dystrophy mutations

Author: Bladen, C.L. Salgado, D. Monges, S. Foncuberta, M.E. Kekou, K. Kosma, K. Dawkins, H. Lamont, L. Roy, A.J. Chamova, T. Guergueltcheva, V. Chan, S. Korngut, L. Campbell, C. Dai, Y. Wang, J. Barišić, N. Brabec, P. Lahdetie, J. Walter, M.C. Schreiber-Katz, O. Karcagi, V. Garami, M. Viswanathan, V. Bayat, F. Buccella, F. Kimura, E. Koeks, Z. van den Bergen, J.C. Rodrigues, M. Roxburgh, R. Lusakowska, A. Kostera-Pruszczyk, A. Zimowski, J. Santos, R. Neagu, E. Artemieva, S. Rasic, V.M. Vojinovic, D. Posada, M. Bloetzer, C. Jeannet, P.-Y. Joncourt, F. Díaz-Manera, J. Gallardo, E. Karaduman, A.A. Topaloğlu, H. El Sherif, R. Stringer, A. Shatillo, A.V. Martin, A.S. Peay, H.L. Bellgard, M.I. Kirschner, J. Flanigan, K.M. Straub, V. Bushby, K. Verschuuren, J. Aartsma-Rus, A. Béroud, C. Lochmüller, H.
Abstract: Analyzing the type and frequency of patient-specific mutations that give rise to Duchenne muscular dystrophy (DMD) is an invaluable tool for diagnostics, basic scientific research, trial planning, and improved clinical care. Locus-specific databases allow for the collection, organization, storage, and analysis of genetic variants of disease. Here, we describe the development and analysis of the TREAT-NMD DMD Global database (http://umd.be/TREAT_DMD/). We analyzed genetic data for 7,149 DMD mutations held within the database. A total of 5,682 large mutations were observed (80% of total mutations), of which 4,894 (86%) were deletions (1 exon or larger) and 784 (14%) were duplications (1 exon or larger). There were 1,445 small mutations (smaller than 1 exon, 20% of all mutations), of which 358 (25%) were small deletions and 132 (9%) small insertions and 199 (14%) affected the splice sites. Point mutations totalled 756 (52% of small mutations) with 726 (50%) nonsense mutations and 30 (2%) missense mutations. Finally, 22 (0.3%) mid-intronic mutations were observed. In addition, mutations were identified within the database that would potentially benefit from novel genetic therapies for DMD including stop codon read-through therapies (10% of total mutations) and exon skipping therapy (80% of deletions and 55% of total mutations). © 2015 The Authors.
Published: 2015

47. Randomized trial of thymectomy in myasthenia gravis

Author: Wolfe, G. I., Kaminski, H. J., Aban, I. B., Minisman, G., Kuo, H. -C., Marx, A., Ströbel, P., Mazia, C., Oger, J., Cea, J. G., Heckmann, J. M., Evoli Stampanoni-B, Amelia, Nix, W., Ciafaloni, E., Antonini, G., Witoonpanich, R., King, J. O., Beydoun, S. R., Chalk, C. H., Barboi, A. C., Amato, A. A., Shaibani, A. I., Katirji, B., Lecky, B. R. F., Buckley, C., Vincent, A., Dias-Tosta, E., Yoshikawa, H., Waddington-Cruz, M., Pulley, M. T., Rivner, M. H., Kostera-Pruszczyk, A., Pascuzzi, R. M., Jackson, C. E., Garcia Ramos, G. S., Verschuuren, J. J. G. M., Massey, J. M., Kissel, J. T., Werneck, L. C., Benatar, M., Barohn, R. J., Tandan, R., Mozaffar, T., Conwit, R., Odenkirchen, J., Sonett, J. R., Jaretzki, A., Newsom-Davis, J., Cutter, G. R., Evoli, A. (ORCID:0000-0003-0282-8787), Wolfe, G. I., Kaminski, H. J., Aban, I. B., Minisman, G., Kuo, H. -C., Marx, A., Ströbel, P., Mazia, C., Oger, J., Cea, J. G., Heckmann, J. M., Evoli Stampanoni-B, Amelia, Nix, W., Ciafaloni, E., Antonini, G., Witoonpanich, R., King, J. O., Beydoun, S. R., Chalk, C. H., Barboi, A. C., Amato, A. A., Shaibani, A. I., Katirji, B., Lecky, B. R. F., Buckley, C., Vincent, A., Dias-Tosta, E., Yoshikawa, H., Waddington-Cruz, M., Pulley, M. T., Rivner, M. H., Kostera-Pruszczyk, A., Pascuzzi, R. M., Jackson, C. E., Garcia Ramos, G. S., Verschuuren, J. J. G. M., Massey, J. M., Kissel, J. T., Werneck, L. C., Benatar, M., Barohn, R. J., Tandan, R., Mozaffar, T., Conwit, R., Odenkirchen, J., Sonett, J. R., Jaretzki, A., Newsom-Davis, J., Cutter, G. R., and Evoli, A. (ORCID:0000-0003-0282-8787)
Abstract: BACKGROUND: Thymectomy has been a mainstay in the treatment of myasthenia gravis, but there is no conclusive evidence of its benefit. We conducted a multicenter, randomized trial comparing thymectomy plus prednisone with prednisone alone. METHODS: We compared extended transsternal thymectomy plus alternate-day prednisone with alternate-day prednisone alone. Patients 18 to 65 years of age who had generalized nonthymomatous myasthenia gravis with a disease duration of less than 5 years were included if they had Myasthenia Gravis Foundation of America clinical class II to IV disease (on a scale from I to V, with higher classes indicating more severe disease) and elevated circulating concentrations of acetylcholine-receptor antibody. The primary outcomes were the time-weighted average Quantitative Myasthenia Gravis score (on a scale from 0 to 39, with higher scores indicating more severe disease) over a 3-year period, as assessed by means of blinded rating, and the time-weighted average required dose of prednisone over a 3-year period. RESULTS: A total of 126 patients underwent randomization between 2006 and 2012 at 36 sites. Patients who underwent thymectomy had a lower time-weighted average Quantitative Myasthenia Gravis score over a 3-year period than those who received prednisone alone (6.15 vs. 8.99, P<0.001); patients in the thymectomy group also had a lower average requirement for alternate-day prednisone (44 mg vs. 60 mg, P<0.001). Fewer patients in the thymectomy group than in the prednisone-only group required immunosuppression with azathioprine (17% vs. 48%, P<0.001) or were hospitalized for exacerbations (9% vs. 37%, P<0.001). The number of patients with treatment-associated complications did not differ significantly between groups (P=0.73), but patients in the thymectomy group had fewer treatment-associated symptoms related to immunosuppressive medications (P<0.001) and lower distress levels related to symptoms (P=0.003). CONCLUSIONS: Thymectomy improved cl
Published: 2016

48. Randomized trial of thymectomy in myasthenia gravis

Author: Wolfe, G. I, Kaminski, H. J., Aban, I. B., Minisman, G., Kuo, H. C., Marx, A., Ströbel, P., Mazia, C., Oger, J., Cea, J. G., Heckmann, J. M., Evoli Stampanoni-B, Amelia, Nix, W., Ciafaloni, E., Antonini, G., Witoonpanich, R., King, J. O., Beydoun, S. R., Chalk, C. H., Barboi, A. C., Amato, A. A., Shaibani, A. I., Katirji, B., Lecky, B. R. F., Buckley, C., Vincent, A., Dias Tosta, E., Yoshikawa, H., Waddington Cruz, M., Pulley, M. T., Rivner, M. H., Kostera Pruszczyk, A., Pascuzzi, R. M., Jackson, C. E., Garcia Ramos, G. S., Verschuuren, J. J. G. M., Massey, J. M., Kissel, J. T., Werneck, L. C., Benatar, M., Barohn, R. J., Tandan, R., Mozaffar, T., Conwit, R., Odenkirchen, J., Sonett, J. R., Jaretzki, A., Newsom Davis, J., Cutter, G. R., Evoli, Amelia (ORCID:0000-0003-0282-8787), Wolfe, G. I, Kaminski, H. J., Aban, I. B., Minisman, G., Kuo, H. C., Marx, A., Ströbel, P., Mazia, C., Oger, J., Cea, J. G., Heckmann, J. M., Evoli Stampanoni-B, Amelia, Nix, W., Ciafaloni, E., Antonini, G., Witoonpanich, R., King, J. O., Beydoun, S. R., Chalk, C. H., Barboi, A. C., Amato, A. A., Shaibani, A. I., Katirji, B., Lecky, B. R. F., Buckley, C., Vincent, A., Dias Tosta, E., Yoshikawa, H., Waddington Cruz, M., Pulley, M. T., Rivner, M. H., Kostera Pruszczyk, A., Pascuzzi, R. M., Jackson, C. E., Garcia Ramos, G. S., Verschuuren, J. J. G. M., Massey, J. M., Kissel, J. T., Werneck, L. C., Benatar, M., Barohn, R. J., Tandan, R., Mozaffar, T., Conwit, R., Odenkirchen, J., Sonett, J. R., Jaretzki, A., Newsom Davis, J., Cutter, G. R., and Evoli, Amelia (ORCID:0000-0003-0282-8787)
Abstract: BACKGROUND: Thymectomy has been a mainstay in the treatment of myasthenia gravis, but there is no conclusive evidence of its benefit. We conducted a multicenter, randomized trial comparing thymectomy plus prednisone with prednisone alone. METHODS: We compared extended transsternal thymectomy plus alternate-day prednisone with alternate-day prednisone alone. Patients 18 to 65 years of age who had generalized nonthymomatous myasthenia gravis with a disease duration of less than 5 years were included if they had Myasthenia Gravis Foundation of America clinical class II to IV disease (on a scale from I to V, with higher classes indicating more severe disease) and elevated circulating concentrations of acetylcholine-receptor antibody. The primary outcomes were the time-weighted average Quantitative Myasthenia Gravis score (on a scale from 0 to 39, with higher scores indicating more severe disease) over a 3-year period, as assessed by means of blinded rating, and the time-weighted average required dose of prednisone over a 3-year period. RESULTS: A total of 126 patients underwent randomization between 2006 and 2012 at 36 sites. Patients who underwent thymectomy had a lower time-weighted average Quantitative Myasthenia Gravis score over a 3-year period than those who received prednisone alone (6.15 vs. 8.99, P<0.001); patients in the thymectomy group also had a lower average requirement for alternate-day prednisone (44 mg vs. 60 mg, P<0.001). Fewer patients in the thymectomy group than in the prednisone-only group required immunosuppression with azathioprine (17% vs. 48%, P<0.001) or were hospitalized for exacerbations (9% vs. 37%, P<0.001). The number of patients with treatment-associated complications did not differ significantly between groups (P=0.73), but patients in the thymectomy group had fewer treatment-associated symptoms related to immunosuppressive medications (P<0.001) and lower distress levels related to symptoms (P=0.003). CONCLUSIONS: Thymectomy improved cl
Published: 2016

49. Presentation of a possible functional scale for paraneoplastic neurological syndromes

Author: Vigliani MC, Graus F, Honnorat J, Antoine JC, Sillevis-Smitt P, Hart I, Carpentier A, Verschuuren J, Voltz R, Grisold W, Giometto B, Vigliani, Mc, Graus, F, Honnorat, J, Antoine, Jc, Sillevis-Smitt, P, Hart, I, Carpentier, A, Verschuuren, J, Voltz, R, Grisold, W, and Giometto, B
Published: 2006

50. Guidelines for pre-clinical animal and cellular models of MuSK-myasthenia gravis

Author: Phillips, W D, Christadoss, P, Losen, M, Punga, Anna Rostedt, Shigemoto, K, Verschuuren, J, Vincent, A, Phillips, W D, Christadoss, P, Losen, M, Punga, Anna Rostedt, Shigemoto, K, Verschuuren, J, and Vincent, A
Abstract: Muscle-specific tyrosine kinase (MuSK) autoantibodies are the hallmark of a form of myasthenia gravis (MG) that can challenge the neurologist and the experimentalist. The clinical disease can be difficult to treat effectively. MuSK autoantibodies affect the neuromuscular junction in several ways. When added to muscle cells in culture, MuSK antibodies disperse acetylcholine receptor clusters. Experimental animals actively immunized with MuSK develop MuSK autoantibodies and muscle weakness. Weakness is associated with reduced postsynaptic acetylcholine receptor numbers, reduced amplitudes of miniature endplate potentials and endplate potentials, and failure of neuromuscular transmission. Similar impairments have been found in mice injected with IgG from MG patients positive for MuSK autoantibody (MuSK-MG). The active and passive models have begun to reveal the mechanisms by which MuSK antibodies disrupt synaptic function at the neuromuscular junction, and should be valuable in developing therapies for MuSK-MG. However, translation into new and improved treatments for patients requires procedures that are not too cumbersome but suitable for examining different aspects of MuSK function and the effects of potential therapies. Study design, conduct and analysis should be carefully considered and transparently reported. Here we review what has been learnt from animal and culture models of MuSK-MG, and offer guidelines for experimental design and conduct of studies, including sample size determination, randomization, outcome parameters and precautions for objective data analysis. These principles may also be relevant to the increasing number of other antibody-mediated diseases that are now recognized.
Published: 2015
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