What is the recurrence rate of Guillain-Barré Syndrome (GBS)?

From the Guidelines

The recurrence rate of Guillain-Barré Syndrome (GBS) is approximately 2-5% of patients experiencing a recurrence after their initial episode. This estimate is based on the most recent and highest quality study available, which was published in 2019 in the journal Nature Reviews Neurology 1. The study highlights that despite the generally positive prospects for patients with GBS, recurrent episodes can occur, although they are rare.

Key Points to Consider

• Most cases of GBS are monophasic, meaning they occur as a single episode with recovery and no further recurrences.
• When recurrences do happen, they may occur months or even years after the initial episode.
• Patients who experience recurrent GBS are sometimes diagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP) instead, which is a related but distinct condition characterized by a more chronic, relapsing course.
• The risk factors for GBS recurrence are not well established, but some evidence suggests that certain variants of GBS, such as Miller Fisher syndrome, may have slightly higher recurrence rates.

Clinical Implications

• Patients who have had GBS should be aware of the possibility of recurrence and seek immediate medical attention if they experience symptoms similar to their initial presentation, such as progressive weakness, numbness, or tingling in the extremities.
• Early treatment with intravenous immunoglobulin (IVIG) or plasma exchange remains the standard approach for recurrent episodes, just as with the initial occurrence, as supported by the study published in 2021 in Nature Reviews Neurology 1.
• It is essential to weigh the benefits of vaccination against the small risk of a recurrent GBS episode in patients who have previously developed GBS, particularly in the context of specific illnesses like influenza in elderly individuals.

From the Research

GBS Recurrence Rate

• The recurrence rate of Guillain-Barré Syndrome (GBS) is estimated to be around 2-3% of patients with a prior GBS episode 2.
• A study published in the Journal of Neurology, Neurosurgery, and Psychiatry found that recurrences occur more frequently in patients under 30, with milder symptoms, and in Miller Fisher syndrome (MFS) 3.
• Another study published in Rinsho Shinkeigaku found that the recurrence rate of FS was 10.8%, and all patients with recurrence showed good prognosis and increased anti-GQ1b glycolipid antibody levels both at the initial episode and at recurrence 4.
• The clinical characteristics of recurrent GBS patients were compared with those of non-recurrent patients, and it was found that recurrent patients were younger and more often had MFS or milder symptoms 3.
• A review of 12 trials found that intravenous immunoglobulin (IVIg) started within two weeks from onset hastens recovery as much as plasma exchange (PE) in severe disease, but there is no significant difference in the recurrence rate between IVIg and PE 5.
• A study published in Arquivos de Neuro-Psiquiatria found that treatment-related fluctuations (TRFs) in GBS were more frequently triggered by infectious mononucleosis, and patients with TRF were initially treated with plasmapheresis more frequently than those without TRF 6.

Factors Associated with Recurrence

• Age: Recurrences occur more frequently in patients under 30 3.
• Severity of symptoms: Patients with milder symptoms are more likely to experience recurrences 3.
• Type of GBS: Miller Fisher syndrome (MFS) is more likely to recur than other types of GBS 3, 4.
• Infectious triggers: Infectious mononucleosis is a common trigger for TRFs in GBS 6.
• Treatment: Plasmapheresis and combined treatment may be associated with a higher risk of TRFs, but the evidence is limited 6.