CSF findings in relation to clinical characteristics, subtype, and disease course in patients with Guillain-Barre syndrome

Al-Hakem, H.; Doets, A.Y.; Stino, A.M.; Zivkovic, S.A.; Andersen, H.; Willison, H.J.; ... ; IGOS Consortium (2023)

Article / Letter to editor

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/μL). 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/μL in 1,005 patients (83%), 5–49 cells/μL in 200 patients (16%), and ≥50 cells/μL 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/μL, is compatible with GBS after a thorough exclusion of alternative diagnoses.

• All authors: 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.; Berg, B. van den; Verboon, C.; Roodbol, J.; Leonhard, S.E.; Arends, S.; Luijten, L.W.G.; Benedetti, L.; Kuwabara, S.; Bergh, P. van den; 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.Z.; 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.; Kooi, A.J. van der; Eftimov, F.; Wirtz, P.W.; Samijn, J.P.A.; Gilhuis, H.J.; 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.; Dijk, G.W. van; Garssen, M.P.J.; Verschuuren, J.; Harbo, T.; Jacobs, B.C.; IGOS Consortium
• Date: 2023-06-06
• Journal: Neurology
• Volume: 100
• Issue: 23
• Pages: e2386 - e2397
• DOI: doi:10.1212/WNL.0000000000207282
• Link: https://www.neurology.org/doi/10.1212/WNL.0000000000207282