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

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

doi:10.1212/WNL.0000000000207282

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

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

^*Corresponding author for this work

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

16 Citations (Scopus)

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.

Original language	English
Pages (from-to)	e2386-e2397
Journal	Neurology
Volume	100
Issue number	23
DOIs	https://doi.org/10.1212/WNL.0000000000207282
Publication status	Published - 6 Jun 2023

Bibliographical note

Funding Information:
IGOS is funded by the GBS-CIDP Foundation International, gain, Erasmus University Medical Centre, Glasgow University, CSL Behring, Grifols, Annexon, and Hansa. H. Al-Hakem was awarded the Lundbeck Foundation Scholarship from The Danish Neurological Society.

Publisher Copyright:
© American Academy of Neurology.

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10.1212/WNL.0000000000207282

Cite this

IGOS Consortium, 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, S. H., Kusunoki, S., Davidson, A., Casasnovas, C., Bateman, K., Miller, J. A. L., van den Berg, B., Verboon, C., ... Jacobs, B. C. (2023). CSF Findings in Relation to Clinical Characteristics, Subtype, and Disease Course in Patients With Guillain-Barré Syndrome. Neurology, 100(23), e2386-e2397. https://doi.org/10.1212/WNL.0000000000207282

@article{ca184a9d909c4e1da3c503ab06ddd771,

title = "CSF Findings in Relation to Clinical Characteristics, Subtype, and Disease Course in Patients With Guillain-Barr{\'e} Syndrome",

abstract = "Background and ObjectivesTo investigate CSF findings in relation to clinical and electrodiagnostic subtypes, severity, and outcome of Guillain-Barr{\'e} 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.",

author = "{IGOS Consortium} and Helle Al-Hakem and Doets, {Alex Y} and Stino, {Amro Maher} and Zivkovic, {Sasha A} and Henning Andersen and Willison, {Hugh J} and Cornblath, {David R} and Gorson, {Kenneth C} and Zhahirul Islam and Mohammad, {Quazi Deen} and Sindrup, {S{\o}ren Hein} and Susumu Kusunoki and Amy Davidson and Carlos Casasnovas and Kathleen Bateman and Miller, {James A L} and {van den Berg}, Bianca and Christine Verboon and Joyce Roodbol and Leonhard, {Sonja E} and Samuel Arends and Luijten, {Linda W G} and Luana Benedetti and Satoshi Kuwabara and {Van den Bergh}, Peter and Soledad Monges and Marfia, {Girolama A} and Nortina Shahrizaila and Giuliana Galassi and Yann Pereon and Jan B{\"u}rmann and Krista Kuitwaard and Kleyweg, {Ruud P} and Cintia Marchesoni and {Sedano Tous}, {Mar{\'i}a J} and Luis Querol and Lorena Mart{\'i}n-Aguilar and Yuzhong Wang and Eduardo Nobile-Orazio and Simon Rinaldi and Angelo Schenone and Julio Pardo and Vermeij, {Frederique H} and Waqar Waheed and Lehmann, {Helmar C} and Volkan Granit and Beth Stein and Samijn, {Johnny P A} and {van Dijk}, {Gert W} and Jacobs, {Bart C}",

note = "Funding Information: IGOS is funded by the GBS-CIDP Foundation International, gain, Erasmus University Medical Centre, Glasgow University, CSL Behring, Grifols, Annexon, and Hansa. H. Al-Hakem was awarded the Lundbeck Foundation Scholarship from The Danish Neurological Society. Publisher Copyright: {\textcopyright} American Academy of Neurology.",

year = "2023",

month = jun,

day = "6",

doi = "10.1212/WNL.0000000000207282",

language = "English",

volume = "100",

pages = "e2386--e2397",

journal = "Neurology",

issn = "0028-3878",

publisher = "Lippincott Williams & Wilkins",

number = "23",

}

IGOS Consortium, Al-Hakem, H, Doets, AY, Stino, AM, Zivkovic, SA, Andersen, H, Willison, HJ, Cornblath, DR, Gorson, KC, Islam, Z, Mohammad, QD, Sindrup, SH, Kusunoki, S, Davidson, A, Casasnovas, C, Bateman, K, Miller, JAL, van den Berg, B, Verboon, C, Roodbol, J , Leonhard, SE , Arends, S , Luijten, LWG, Benedetti, L, Kuwabara, S, Van den Bergh, P, Monges, S, Marfia, GA, Shahrizaila, N, Galassi, G, Pereon, Y, Bürmann, J, Kuitwaard, K, Kleyweg, RP, Marchesoni, C, Sedano Tous, MJ, Querol, L, Martín-Aguilar, L, Wang, Y, Nobile-Orazio, E, Rinaldi, S, Schenone, A, Pardo, J, Vermeij, FH, Waheed, W, Lehmann, HC, Granit, V, Stein, B, Samijn, JPA, van Dijk, GW & Jacobs, BC 2023, 'CSF Findings in Relation to Clinical Characteristics, Subtype, and Disease Course in Patients With Guillain-Barré Syndrome', Neurology, vol. 100, no. 23, pp. e2386-e2397. https://doi.org/10.1212/WNL.0000000000207282

TY - JOUR

T1 - CSF Findings in Relation to Clinical Characteristics, Subtype, and Disease Course in Patients With Guillain-Barré Syndrome

AU - IGOS Consortium

AU - Al-Hakem, Helle

AU - Doets, Alex Y

AU - Stino, Amro Maher

AU - Zivkovic, Sasha A

AU - Andersen, Henning

AU - Willison, Hugh J

AU - Cornblath, David R

AU - Gorson, Kenneth C

AU - Islam, Zhahirul

AU - Mohammad, Quazi Deen

AU - Sindrup, Søren Hein

AU - Kusunoki, Susumu

AU - Davidson, Amy

AU - Casasnovas, Carlos

AU - Bateman, Kathleen

AU - Miller, James A L

AU - van den Berg, Bianca

AU - Verboon, Christine

AU - Roodbol, Joyce

AU - Leonhard, Sonja E

AU - Arends, Samuel

AU - Luijten, Linda W G

AU - Benedetti, Luana

AU - Kuwabara, Satoshi

AU - Van den Bergh, Peter

AU - Monges, Soledad

AU - Marfia, Girolama A

AU - Shahrizaila, Nortina

AU - Galassi, Giuliana

AU - Pereon, Yann

AU - Bürmann, Jan

AU - Kuitwaard, Krista

AU - Kleyweg, Ruud P

AU - Marchesoni, Cintia

AU - Sedano Tous, María J

AU - Querol, Luis

AU - Martín-Aguilar, Lorena

AU - Wang, Yuzhong

AU - Nobile-Orazio, Eduardo

AU - Rinaldi, Simon

AU - Schenone, Angelo

AU - Pardo, Julio

AU - Vermeij, Frederique H

AU - Waheed, Waqar

AU - Lehmann, Helmar C

AU - Granit, Volkan

AU - Stein, Beth

AU - Samijn, Johnny P A

AU - van Dijk, Gert W

AU - Jacobs, Bart C

N1 - Funding Information: IGOS is funded by the GBS-CIDP Foundation International, gain, Erasmus University Medical Centre, Glasgow University, CSL Behring, Grifols, Annexon, and Hansa. H. Al-Hakem was awarded the Lundbeck Foundation Scholarship from The Danish Neurological Society. Publisher Copyright: © American Academy of Neurology.

PY - 2023/6/6

Y1 - 2023/6/6

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=85162175839&partnerID=8YFLogxK

U2 - 10.1212/WNL.0000000000207282

DO - 10.1212/WNL.0000000000207282

M3 - Article

C2 - 37076309

SN - 0028-3878

VL - 100

SP - e2386-e2397

JO - Neurology

JF - Neurology

IS - 23

ER -

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

Abstract

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