What are the implications of repeated Guillain-Barré Syndrome (GBS) events?

From the Guidelines

Repeated Guillain-Barré Syndrome (GBS) events are rare, affecting 2-5% of patients, and can have significant implications for patient management and outcomes.

Implications of Repeated GBS Events

• The risk of recurrent GBS episodes is higher than the lifetime risk of GBS in the general population (0.1%) 1.
• Patients with a history of GBS may need to weigh the benefits of vaccination against the small risk of a recurrent GBS episode, particularly if they have a planned vaccination or have previously developed GBS shortly after receiving the same vaccination 1.
• The management of repeated GBS events may involve repeat treatment with intravenous immunoglobulin or plasma exchange, although the efficacy of repeat treatment is uncertain 1.

Clinical Considerations

• The diagnosis and management of GBS can be complicated by its heterogeneous clinical presentation and disease course, and the lack of highly sensitive and specific diagnostic tools or biomarkers 1.
• Patients with GBS are at risk of developing respiratory failure, cardiac arrhythmias, and blood pressure instability, which can contribute to mortality 1.
• The prognosis for patients with GBS is generally positive, with about 80% of patients regaining the ability to walk independently at 6 months after disease onset, although long-term residual complaints are common 1.

Treatment and Prevention

• Intravenous immunoglobulin and plasma exchange are equally effective in treating GBS, although the efficacy of repeat treatment is uncertain 1.
• Vaccines and immunobiologicals may be associated with an increased risk of GBS, although the epidemiological evidence is limited and the risk is generally considered to be small 1.

From the Research

Clinical Characteristics of Recurrent Guillain-Barré Syndrome

• Recurrent Guillain-Barré Syndrome (RGBS) occurs in a subset of patients, with studies suggesting a recurrence rate of up to 6% 2, 3.
• Clinical symptoms in subsequent episodes are often similar, but the severity of symptoms and preceding infections can vary 2, 4.
• Patients with recurrent GBS tend to be younger, with a mean age of 34.2 years, and are more likely to have milder symptoms or Miller Fisher syndrome (MFS) 2.
• The majority of patients with RGBS are male, with a male to female ratio of 3:1 3.

Triggers and Infections

• Triggering infections occur in most patients with RGBS, with few examples of etiological promiscuity 5.
• The nature of preceding infections can vary between episodes, suggesting that genetic or immunological host factors may play a role in recurrent GBS 2.
• Identical triggering events were noted in some patients, but not all 4.

Outcome and Prognosis

• The severity of different attacks is not related to the number of recurrences, but there may be a tendency to accumulate neurological deficits with increasing frequency of attacks 4.
• Most patients with RGBS recover fully or have moderate sequelae after each episode, but some may experience persistent deficits 4, 5.
• Episodes in RGBS tend to be shorter than in monophasic GBS, and an initial episode duration of less than 45 days may be predictive of recurrence 5.

Treatment and Management

• Treatment for RGBS is generally the same as for monophasic GBS, focusing on supportive care, intravenous immunoglobulin administration, and plasmapheresis, with steroids having no impact on prognosis 6.
• Rapid deterioration can occur in severe cases of recurrent GBS, highlighting the need for prompt recognition and treatment 6.