What is Guillain-Barré Syndrome (GBS)?

From the Guidelines

Guillain-Barré syndrome (GBS) should be treated with intravenous immunoglobulin (IVIG) at 0.4g/kg daily for 5 days or plasma exchange (5 exchanges over 2 weeks) as first-line options, as these treatments are most effective when started within 2-4 weeks of symptom onset, according to the most recent and highest quality study 1.

Diagnosis and Management

GBS is a rare autoimmune disorder where the body's immune system attacks the peripheral nerves, causing rapid-onset muscle weakness that can progress to paralysis. It typically develops after a respiratory or gastrointestinal infection, with symptoms starting in the legs and ascending upward.

• The diagnosis of GBS is based on the patient history and neurological, electrophysiological, and cerebrospinal fluid (CSF) examinations 1.
• Electrophysiological studies provide evidence of PNS dysfunction and can distinguish between the subtypes of GBS: acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor sensory axonal neuropathy (AMSAN) 1.

Treatment

Treatment focuses on immunotherapy, with IVIG or plasma exchange being first-line options.

• IVIG is considered the first choice of treatment as it is easy to administer, widely available, and associated with a reduced frequency of adverse effects compared with plasma exchange 1.
• Plasma exchange is less costly than IVIG and could theoretically be a preferred treatment option for GBS in low-income and middle-income countries (LMIC) 1.

Supportive Care

Supportive care is crucial, including:

• Respiratory monitoring (as 25% of patients require ventilation) 1.
• DVT prophylaxis.
• Pain management with gabapentin or carbamazepine.
• Physical therapy. Most patients require hospitalization, with ICU admission for those with respiratory compromise or autonomic instability.

Outcome

While 80% of patients recover fully, recovery can take months to years, with about 20% experiencing long-term disability 1.

• The condition results from molecular mimicry, where antibodies targeting infectious agents cross-react with components of peripheral nerve myelin or axons, leading to demyelination and conduction block 1.
• Mortality is estimated at 3–10% for patients with GBS even with the best medical care available 1.

From the Research

Guillain-Barré Syndrome Overview

• Guillain-Barré syndrome (GBS) is an acute polyneuropathy characterized by acute flaccid paralysis with or without sensory/autonomous nerve dysfunction 2
• Current immunotherapies, including intravenous immunoglobulin (IVIg) and plasma exchange (PE), benefit patients by alleviating hyperreactive immune responses 2

Treatment Options

• Plasma exchange removes antibodies and other potentially injurious factors from the bloodstream, and has been shown to be beneficial in patients with GBS 3
• Intravenous immunoglobulin (IVIg) is also a commonly used treatment for GBS, and has been shown to be effective in improving symptoms and reducing disability 4, 5
• Other pharmacological agents, such as interferon beta-1a, brain-derived neurotrophic factor, and eculizumab, have been studied as potential treatments for GBS, but the evidence is limited and inconclusive 6

Comparison of Treatment Options

• A study comparing PE and IVIg found that PE had a more significant curative effect, with improved nerve function and clinical outcomes 4
• Another study found that IVIg had a slightly faster improvement in symptoms and disability compared to PE, but the long-term outcomes were equivalent 5
• A review of six trials comparing different pharmacological agents found that the evidence was limited and inconclusive, and that none of the interventions showed a clear benefit or harm 6

Clinical Considerations

• The decision to use PE or IVIg should be based on individual patient factors, such as disease severity and medical history 2
• Patients with severe GBS may require mechanical ventilation and intensive care, and should be closely monitored for complications 2
• Further research is needed to determine the optimal treatment strategy for GBS, and to identify new and effective treatments 6