From the Guidelines
Guillain-Barré syndrome (GBS) is a rare, but potentially fatal, immune-mediated disease of the peripheral nerves and nerve roots that is usually triggered by infections, and its diagnosis and management can be complicated due to its heterogeneous clinical presentation and disease course. The condition typically begins with weakness and sensory signs in the legs that progress to the arms and cranial muscles, although several distinct clinical variants exist 1. GBS is thought to be caused by an aberrant immune response to infections that results in damage to peripheral nerves, and its incidence can increase during outbreaks of infectious diseases 1.
Key Characteristics of GBS
- Annual global incidence of approximately 1–2 per 100,000 person-years 1
- Occurs more frequently in males than in females and the incidence increases with age 1
- Patients typically present with weakness and sensory signs in the legs that progress to the arms and cranial muscles 1
- Diagnosis of GBS is based on the patient history and neurological, electrophysiological and cerebrospinal fluid (CSF) examinations 1
Treatment and Management
Treatment of GBS focuses on immunotherapy, primarily intravenous immunoglobulin (IVIG) given at 0.4 g/kg for 5 days, or plasma exchange (PLEX) performed 4-6 times over 1-2 weeks, and these treatments are most effective when started within the first two weeks of symptom onset. Supportive care is crucial, including monitoring of respiratory function, as up to 30% of patients may require mechanical ventilation 1. Physical therapy should begin early to prevent complications. While most patients recover, recovery can take months to years, and about 20% of patients may have residual disability. The condition is considered a medical emergency, and patients showing symptoms should seek immediate medical attention.
Recent Developments in Treatment
Recent studies have explored the use of small volume plasma exchange (SVPE) as a relatively low-cost and simple technique for selective removal of plasma, which has been shown to be a safe and feasible treatment for GBS in resource-limited settings 1. Additionally, complement inhibitors such as eculizumab are being studied as a potential new treatment for GBS in high-income countries 1.
From the Research
Definition and Overview of Guillain-Barré Syndrome
- Guillain-Barré syndrome (GBS) is a potentially devastating yet treatable disorder, characterized as a classically postinfectious, immune-mediated, monophasic polyradiculoneuropathy 2.
- It is the leading global cause of acquired neuromuscular paralysis, with the immunopathological process driving nerve injury being ill-defined in most cases.
- Diagnosis of GBS relies on clinical features, supported by laboratory findings and electrophysiology.
Treatment Options for Guillain-Barré Syndrome
- Intravenous immunoglobulin and plasma exchange remain the primary modalities of treatment, regardless of the electrophysiological subtype 2.
- Studies have compared the effectiveness of plasma exchange (PE) and intravenous immunoglobulin (IVIG) in treating GBS, with some showing that PE has a more significant curative effect 3.
- However, other studies have found that both PE and IVIG have a high response as therapy and are reasonable therapeutic options for GBS, with no significant difference in outcome at one year 4.
- The long-term outcomes of PE and IVIG have been found to be equivalent, although the effect size showed measurable differences between the two groups across different measures at one-year follow-up 4.
Effectiveness and Cost-Efficiency of Treatment Options
- A study found that IVIG followed by PE was not more effective than IVIG alone for patients with GBS, and was associated with an increase in cost and duration of hospitalization 5.
- Another study found that PE and IVIG had similar outcomes during 48 weeks of follow-up, with no significant difference in outcome between the two groups 6.
- The cost/benefit ratio of using PE after IVIG is still unclear, and prospective studies are needed to clarify this issue 5.