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

Neurology. 2023 Jun 6;100(23):e2386-e2397. doi: 10.1212/WNL.0000000000207282. Epub 2023 Apr 19.

Abstract

Background and objectives: To 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.

Methods: Albuminocytologic 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%).

Results: In 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%).

Discussion: ACD 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.

Classification of evidence: This study provides Class IV evidence that CSF ACD (defined by the Brighton Collaboration) is common in patients with GBS.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Cell Count
  • Cerebrospinal Fluid / cytology
  • Cohort Studies
  • Disease Progression
  • Female
  • Guillain-Barre Syndrome* / cerebrospinal fluid
  • Guillain-Barre Syndrome* / diagnosis
  • Guillain-Barre Syndrome* / pathology
  • Guillain-Barre Syndrome* / physiopathology
  • Humans
  • Internationality
  • Male
  • Middle Aged
  • Miller Fisher Syndrome / cerebrospinal fluid
  • Miller Fisher Syndrome / diagnosis
  • Miller Fisher Syndrome / pathology
  • Miller Fisher Syndrome / physiopathology
  • Prognosis
  • Treatment Outcome