Current treatment practice of Guillain-Barré syndrome

Christine Verboon; Alex Y Doets; IGOS Consortium; Giuliana Galassi; Amy Davidson; Waqar Waheed; Yann Péréon; Nortina Shahrizaila; Susumu Kusunoki; Helmar C Lehmann; Thomas Harbo; Soledad Monges; Peter Van den Bergh; Hugh J Willison; David R Cornblath; Bart C Jacobs; Krista Kuitwaard

doi:10.1212/WNL.0000000000007719

Current treatment practice of Guillain-Barré syndrome

Christine Verboon, Alex Y Doets, IGOS Consortium, Giuliana Galassi, Amy Davidson, Waqar Waheed, Yann Péréon, Nortina Shahrizaila, Susumu Kusunoki, Helmar C Lehmann, Thomas Harbo, Soledad Monges, Peter Van den Bergh, Hugh J Willison, David R Cornblath, Bart C Jacobs^*, Krista Kuitwaard

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

72 Citations (Scopus)

Abstract

OBJECTIVE: To define the current treatment practice of Guillain-Barré 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.

Original language	English
Pages (from-to)	E59-E76
Journal	Neurology
Volume	93
Issue number	1
DOIs	https://doi.org/10.1212/WNL.0000000000007719
Publication status	Published - 2 Jul 2019

Bibliographical note

Study funding:
This study is funded by the GBS-CIDP Foundation International, Gain, Erasmus MC University Medical Centre
Rotterdam, Glasgow University, CSL Behring, Grifols, and
Annexon.

© 2019 American Academy of Neurology.

Research programs

EMC MM-02-72-02

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10.1212/WNL.0000000000007719

http://hdl.handle.net/1765/119468

Cite this

Verboon, C., Doets, A. Y., IGOS Consortium, Galassi, G., Davidson, A., Waheed, W., Péréon, 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., & Kuitwaard, K. (2019). Current treatment practice of Guillain-Barré syndrome. Neurology, 93(1), E59-E76. https://doi.org/10.1212/WNL.0000000000007719

@article{b2123fe250a744c1b6863f538709ef60,

title = "Current treatment practice of Guillain-Barr{\'e} syndrome",

abstract = "OBJECTIVE: To define the current treatment practice of Guillain-Barr{\'e} 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.",

author = "Christine Verboon and Doets, \{Alex Y\} and \{IGOS Consortium\} and Giuliana Galassi and Amy Davidson and Waqar Waheed and Yann P{\'e}r{\'e}on and Nortina Shahrizaila and Susumu Kusunoki and Lehmann, \{Helmar C\} and Thomas Harbo and Soledad Monges and \{Van den Bergh\}, Peter and Willison, \{Hugh J\} and Cornblath, \{David R\} and Jacobs, \{Bart C\} and Krista Kuitwaard",

note = "Study funding: This study is funded by the GBS-CIDP Foundation International, Gain, Erasmus MC University Medical Centre Rotterdam, Glasgow University, CSL Behring, Grifols, and Annexon. {\textcopyright} 2019 American Academy of Neurology.",

year = "2019",

month = jul,

day = "2",

doi = "10.1212/WNL.0000000000007719",

language = "English",

volume = "93",

pages = "E59--E76",

journal = "Neurology",

issn = "0028-3878",

publisher = "Lippincott Williams \& Wilkins",

number = "1",

}

Verboon, C, Doets, AY, IGOS Consortium, Galassi, G, Davidson, A, Waheed, W, Péréon, Y, Shahrizaila, N, Kusunoki, S, Lehmann, HC, Harbo, T, Monges, S, Van den Bergh, P, Willison, HJ, Cornblath, DR, Jacobs, BC & Kuitwaard, K 2019, 'Current treatment practice of Guillain-Barré syndrome', Neurology, vol. 93, no. 1, pp. E59-E76. https://doi.org/10.1212/WNL.0000000000007719

TY - JOUR

T1 - Current treatment practice of Guillain-Barré syndrome

AU - Verboon, Christine

AU - Doets, Alex Y

AU - IGOS Consortium

AU - Galassi, Giuliana

AU - Davidson, Amy

AU - Waheed, Waqar

AU - Péréon, Yann

AU - Shahrizaila, Nortina

AU - Kusunoki, Susumu

AU - Lehmann, Helmar C

AU - Harbo, Thomas

AU - Monges, Soledad

AU - Van den Bergh, Peter

AU - Willison, Hugh J

AU - Cornblath, David R

AU - Jacobs, Bart C

AU - Kuitwaard, Krista

N1 - Study funding: This study is funded by the GBS-CIDP Foundation International, Gain, Erasmus MC University Medical Centre Rotterdam, Glasgow University, CSL Behring, Grifols, and Annexon. © 2019 American Academy of Neurology.

PY - 2019/7/2

Y1 - 2019/7/2

N2 - OBJECTIVE: To define the current treatment practice of Guillain-Barré 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.

AB - OBJECTIVE: To define the current treatment practice of Guillain-Barré 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.

U2 - 10.1212/WNL.0000000000007719

DO - 10.1212/WNL.0000000000007719

M3 - Article

C2 - 31175208

SN - 0028-3878

VL - 93

SP - E59-E76

JO - Neurology

JF - Neurology

IS - 1

ER -

Current treatment practice of Guillain-Barré syndrome

Abstract

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