What are the diagnostic criteria and treatment options for Guillain-Barré Syndrome (GBS)?

From the Guidelines

Guillain-Barré Syndrome (GBS) should be diagnosed and treated promptly with intravenous immunoglobulin (IVIG) at 0.4 g/kg/day for 5 days or plasma exchange (PLEX) with 5 exchanges over 2 weeks, as these are equally effective first-line treatments, as recommended by the most recent and highest quality study 1.

Diagnostic Criteria

The diagnostic criteria for GBS include:

• Progressive, relatively symmetric muscle weakness with diminished or absent deep tendon reflexes
• Progression of symptoms over days to weeks
• Exclusion of other causes
• Cerebrospinal fluid (CSF) analysis typically shows albuminocytologic dissociation (elevated protein with normal cell count)
• Nerve conduction studies demonstrate demyelination or axonal damage

Treatment Options

Treatment should begin as soon as GBS is suspected, with immunotherapy being the mainstay.

• IVIG at 0.4 g/kg/day for 5 days or PLEX with 5 exchanges over 2 weeks are equally effective first-line treatments, as shown in a study published in Nature Reviews Neurology 1 and further supported by another study 1.
• IVIG is often preferred due to convenience and fewer complications.
• Supportive care is crucial, including:
  • Respiratory monitoring (with mechanical ventilation if needed)
  • Thromboprophylaxis
  • Pain management
  • Early rehabilitation
• Approximately 25% of patients require mechanical ventilation, so close monitoring of respiratory function is essential.
• Corticosteroids are not recommended as they have not shown benefit, except in cases of immune-related adverse events, where a trial of corticosteroids may be reasonable, as suggested by a study published in the Journal of Clinical Oncology 1.

Outcome

Early treatment improves outcomes, with about 80% of patients achieving full recovery within 6-12 months, though some may have residual weakness or sensory symptoms, as reported in a study published in Nature Reviews Neurology 1.

From the Research

Diagnostic Criteria for Guillain-Barré Syndrome (GBS)

• The diagnosis of GBS is based on a combination of clinical, laboratory, and electrodiagnostic findings 2, 3, 4
• The principal diagnostic criteria include:
  • A history of recent diarrhoea or respiratory infection 2
  • Progressive weakness and universal areflexia or hyporeflexia 5, 3
  • Symmetric weakness 3, 6
  • Loss of tendon reflexes 3
  • Elevated cerebrospinal fluid protein 3
  • Reduced conduction velocity in motor nerves and absent F-waves 3
• Electrodiagnostic testing, such as electromyography (EMG) and nerve conduction studies (NCS), can support the diagnosis 2, 3
• Testing for anti-ganglioside antibodies may be useful in some cases, particularly when Miller Fisher syndrome (MFS) is suspected 2

Treatment Options for GBS

• The treatment of GBS typically involves immunomodulatory therapies, such as:
  • Intravenous immunoglobulin (IVIg) 0.4 g/kg for 5 days 2
  • Plasma exchange (PE) 12-15 L in four to five exchanges over 1-2 weeks 2
• The choice of treatment depends on the severity of the disease and the patient's ability to walk unaided 2
• Corticosteroids are not recommended for the treatment of GBS 2, 3
• Pain management may involve the use of gabapentinoids, tricyclic antidepressants, or carbamazepine 2

Clinical Heterogeneity and Diagnostic Challenges

• GBS can present with a range of clinical features, including:
  • Pure sensory symptoms 5
  • Nonspecific generalized weakness 5
  • Atypical pattern of weakness 5
  • Preserved or increased deep tendon reflexes (DTRs) 5
• Delay in diagnosis can occur due to the clinical heterogeneity of GBS and the presence of atypical features 5, 6
• Rheumatologists should be aware of the potential for GBS to present with rheumatic symptoms, such as nociceptive-like prodromal pain 6