CSF Findings in Relation to Clinical Characteristics, Subtype, and Disease Course in Patients With Guillain-Barré Syndrome
Helle Al-Hakem, Alex Y. Doets, Amro Stino, Sasha Živković, Henning Andersen, Hugh J. Willison, David R. Cornblath, Kenneth C. Gorson, Zhahirul Islam, Quazi Deen Mohammad, Søren H. Sindrup, Susumu Kusunoki, Amy Davidson, Carlos Casasnovas, Kathleen Bateman, James 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 Alessandra Marfia, Nortina Shahrizaila, Giuliana Galassi, Yann Péréon, J. Bürmann, Krista Kuitwaard, R. P. Kleyweg, Cintia Marchesoni, María J. Sedano Tous, Luís Querol, L. Aguilar, Yuzhong Wang, Eduardo Nobile‐Orazio, Simon Rinaldi, Angelo Schenone, J. Marín Pardo, Frédérique H Vermeij, Waqar Waheed, Helmar C. Lehmann, Volkan Granit, Beth Stein, Guido Cavaletti, Gerardo Gutiérrez‐Gutiérrez, Fábio Barroso, Leo H. Visser, Hans Katzberg, Efthimios Dardiotis, Shahram Attarian, Anneke J. van der Kooi, Filip Eftimov, Paul W. Wirtz, Johnny P.A. Samijn, H. Jacobus Gilhuis, Robert D. M. Hadden, James Holt, Kazim A. Sheikh, Noah Kolb, Summer Karafiath, Michal Vytopil, Giovanni Antonini, Thomas E. Feasby, Catharina G. Faber, Hans Kramers, Mark Busby, Rhys Roberts, Nicholas J. Silvestri, Raffaella Fazio, Gert W. van Dijk, Marcel P.J. Garssen, Jan J.G.M. Verschuuren, Thomas Harbo, Bart C. Jacobs, Richard AC Hughes, H.‐P. Hartung, L.C. de Koning, Melissa R. Mandarakas, M. van Woerkom, Ricardo Reisin, Stephen Reddel, Sung‐Tsang Hsieh, Jean Addington, Senda Ajroud‐Driss, Lucas Alessandro, Umesh A. Badrising, G. Balloy, I.R. Bella, T. E. Bertoríni, R. Bhavaraju-Sanka, Mariangela Bianco, Thomas H. Brannagan, Kirsty Brennan, Chiara Briani, S. Butterworth, Chi‐Chao Chao
Abstract
<h3>Objective:</h3> To investigate cerebrospinal fluid findings in relation to clinical and electrodiagnostic subtypes, severity, and outcome of Guillain-Barré syndrome based on 1500 patients in the International GBS Outcome Study. <h3>Methods:</h3> Albuminocytological dissociation was defined as an increased protein level (>0.45 g/L) in absence of elevated white cell count (<50 cells/µL). We excluded 124 (8%) patients due to other diagnoses, protocol violation or insufficient data. CSF was examined in 1231 patients (89%). <h3>Results:</h3> In 846 (70%) patients CSF examination showed albuminocytological dissociation, 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 (OR: 0.42 (95% CI 0.25-0.70; <i>p</i> = 0.001) and week 4 (OR: 0.44 (95% CI 0.27-0.72; <i>p</i> = 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/µL in 1005 patients (83%), 5-49 cells/µL in 200 patients (16%) and ≥50 cells/µL in 13 patients (1%). <h3>Interpretation:</h3> Albuminocytological dissociation is a common finding in Guillain-Barré syndrome, 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/µL, is compatible with GBS after thorough exclusion of alternative diagnoses. <h3>Classification of evidence:</h3> This study provides Class IV evidence that CSF albuminocytologic dissociation (defined by the Brighton Collaboration) is common in patients with GBS.