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

  • 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 Burmann, Krista Kuitwaard, Ruud P. Kleyweg, Cintia Marchesoni, Maria J.Sedano Tous, Luis Querol, Lorena Martin-Aguilar, Yuzhong Wang, Eduardo Nobile-Orazio, Simon Rinaldi, Angelo Schenone, Julio Pardo, Frederique H. Vermeij, Waqar Waheed, Helmar C. Lehmann, Volkan Granit, Beth Stein, Guido Cavaletti, Gerardo Gutierrez-Gutierrez, Fabio A. Barroso, Leo H. Visser, Hans D. 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 K.L. Holt, Kazim A. Sheikh, Noah Kolb, Summer Karafiath, Michal Vytopil, Giovanni Antonini, Thomas E. Feasby, Catharina Faber, Hans Kramers, Mark Busby, Rhys C. Roberts, Nicholas J. Silvestri, Raffaella Fazio, Gert W. Van Dijk, Marcel P.J. Garssen, Jan Verschuuren, Thomas Harbo, Bart C. Jacobs
  • Aarhus University
  • Erasmus University Rotterdam
  • University of Michigan, Ann Arbor
  • University of Pittsburgh
  • University of Glasgow
  • Johns Hopkins University
  • Tufts University
  • Laboratory of Gut-Brain Signaling
  • Laboratory Sciences and Services Division
  • National Institute of Neurosciences and Hospital
  • University of Southern Denmark
  • Kindai University
  • L'Hospitalet Del Llobregat
  • University of Cape Town
  • Newcastle upon Tyne Hospitals NHS Foundation Trust
  • Vlietland Hospital
  • Haga Ziekenhuis
  • ETZ Elisabeth
  • Ospedale Policlino San Martino -University of Genoa
  • Chiba University
  • Université catholique de Louvain
  • Hospital de Pediatría Prof. Dr. Juan P. Garrahan
  • University of Rome Tor Vergata
  • University of Malaya
  • University of Modena and Reggio Emilia
  • Nantes University Hospital
  • Saarland University
  • Mvz Pfalzklinikum
  • Albert Schweitzer Ziekenhuis
  • Hospital Británico de Buenos Aires
  • Hospital Universitario Marques de Valdecilla
  • Autonomous University of Barcelona
  • Jining Medical College
  • University of Oxford
  • University of Milan
  • Ophthalmology
  • University of Genoa
  • Complejo Hospitalario Universitario de Santiago
  • University of Vermont
  • University of Cologne
  • Albert Einstein College of Medicine
  • University of Milan - Bicocca
  • Hospital Universitario Infanta Sofía
  • Fundación para la Lucha contra las Enfermedades Neurológicas de la Infancia
  • Toronto Hospital
  • University Hospital of Larissa
  • Assistance publique - Hôpitaux de Marseille
  • University of Amsterdam
  • Maasstad Hospital
  • Reinier de Graaf Groep
  • King's College Hospital
  • The Walton Centre NHS Foundation Trust
  • University of Texas Health Science Center at Houston
  • Utah Valley University
  • Lahey Hospital and Medical Center
  • University of Rome "sapienza,"
  • University of Calgary
  • Maastricht University
  • Leeds Teaching Hospitals NHS Trust
  • Cambridge University Hospitals NHS Foundation Trust
  • San Raffaele Scientific Institute
  • Canisius Wilhelmina Hospital
  • Jeroen Bosch Ziekenhuis
  • Leiden University

Research output: Contribution to journalArticlepeer-review

42 Scopus citations

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 languageEnglish
Pages (from-to)E2386-E2397
JournalNeurology
Volume100
Issue number23
DOIs
StatePublished - Jun 6 2023

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