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, characterized by rapid-onset muscle weakness that can progress to paralysis. It typically begins with tingling and weakness in the extremities, often starting in the legs and spreading upward. GBS usually develops after a respiratory or gastrointestinal infection, as the immune response triggered by these infections cross-reacts with nerve components 1. The incidence of GBS can increase during outbreaks of infectious diseases, and it occurs more frequently in males than in females, with the incidence increasing with age, although all age groups can be affected 1.
Key Characteristics of GBS
- Annual global incidence of approximately 1–2 per 100,000 person-years 1
- Patients typically present with weakness and sensory signs in the legs that progress to the arms and cranial muscles 1
- Diagnosis is based on patient history and neurological, electrophysiological, and cerebrospinal fluid (CSF) examinations 1
- Disease progression can be rapid, with most patients reaching their maximum disability within 2 weeks 1
- About 20% of patients develop respiratory failure and require mechanical ventilation 1
Treatment and Management
Treatment primarily involves immunotherapy such as intravenous immunoglobulin (IVIG) at 2g/kg divided over 2-5 days or plasma exchange (5 exchanges over 2 weeks). Supportive care is crucial, including respiratory monitoring, 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% may have residual disability 1. Prompt diagnosis and treatment are essential to improve outcomes and reduce the risk of long-term complications.
Prognosis and Outcome
Most patients with GBS show extensive recovery, especially in the first year after disease onset, with about 80% regaining the ability to walk independently at 6 months after disease onset 1. However, death occurs in 3–10% of cases, most commonly due to cardiovascular and respiratory complications 1. Long-term residual complaints are common and can include neuropathic pain, weakness, and fatigue, but recovery from these complaints may still occur >5 years after disease onset 1.
From the Research
Definition of GBS
- 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 most cases driven by an ill-defined immunopathological process 2.
Clinical Features and Diagnosis
- Diagnosis of GBS relies on clinical features, supported by laboratory findings and electrophysiology 2.
- The disease is characterized by nerve injury, with the primary modalities of treatment being intravenous immunoglobulin and plasma exchange, regardless of the electrophysiological subtype 2.
Treatment and Outcomes
- Studies have compared the effectiveness of plasma exchange (PE) and intravenous immunoglobulin (IVIg) in treating GBS, with some showing that PE has a more significantly 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 long-term outcomes being equivalent 4.
- The choice of treatment may depend on various factors, including the severity of the disease and the patient's response to treatment, with some studies suggesting that IVIg may be slightly more effective in certain cases 4, 5.