What is Guillain-Barré Syndrome (GBS)?

From the Guidelines

Guillain-Barré syndrome (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.

Definition and Pathogenesis

GBS typically develops after a respiratory or gastrointestinal infection, with symptoms beginning in the legs and ascending upward 1. The condition results from antibodies mistakenly targeting peripheral nerve components following an infection that triggers molecular mimicry, where the immune system confuses nerve structures with infectious agents.

Clinical Presentation and Diagnosis

Patients with GBS typically present with weakness and sensory signs in the legs that progress to the arms and cranial muscles, although the clinical presentation of the disease is heterogeneous and several distinct clinical variants exist 1. 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 and Management

Treatment focuses on immunotherapy, with intravenous immunoglobulin (IVIG) at 0.4g/kg daily for 5 days or plasma exchange (PLEX) with 5 exchanges over 2 weeks being equally effective first-line treatments 1. Supportive care is crucial, including respiratory monitoring (as 25% of patients require mechanical ventilation), DVT prophylaxis, pain management, and physical therapy. Some key points to consider in treatment include:

• 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 intravenous immunoglobulin and could theoretically be a preferred treatment option for GBS in low-income and middle-income countries (LMIC) 1.
• Small volume plasma exchange (SVPE) is a novel, relatively low-cost, simple technique for selective removal of plasma, and has been shown to be a safe and feasible treatment for GBS in resource-limited settings 1.

Prognosis and Outcome

Most patients recover, though recovery can take months to years, with about 20% experiencing long-term disability 1. Early recognition and treatment are essential, as prompt therapy within 2 weeks of symptom onset leads to better outcomes. The mortality rate for patients with GBS is estimated at 3–10% even with the best medical care available 1.

From the Research

Definition and Characteristics of Guillain-Barré Syndrome (GBS)

• Guillain-Barré Syndrome (GBS) is an acute polyneuropathy with a variable degree of weakness that reaches its maximal severity within 4 weeks 2.
• The disease is mostly preceded by an infection and generally runs a monophasic course 2.
• GBS can be subdivided into the acute inflammatory demyelinating polyneuropathy (AIDP), the most frequent form in the western world; acute motor axonal neuropathy (AMAN), most frequent in Asia and Japan; and in Miller-Fisher syndrome (MFS) 2.

Treatment and Management of GBS

• Both intravenous immunoglobulin (IVIg) and plasma exchange (PE) are effective in GBS, while steroids alone are ineffective 2, 3, 4.
• IVIg is usually the preferred treatment due to practical reasons 2.
• The efficacy of IVIg in hastening recovery and reducing long-term morbidity from GBS has been demonstrated in several studies 3, 4.
• The most efficacious dose of IVIg in hastening recovery and reducing long-term morbidity from GBS is still being researched, with some studies suggesting that high-dose IVIg may be more effective than low-dose IVIg 3, 4.

Prognosis and Outcome of GBS

• Important clinical factors associated with poor outcome in GBS include age, presence of preceding diarrhea, and the severity of disability in the early course of disease 5.
• The Erasmus GBS Outcome Scale (EGOS) is a clinical prognostic scoring scale that combines these clinical factors to predict outcome in GBS patients 5.
• About 25% of patients need artificial ventilation and 20% are still unable to walk unaided after 6 months, highlighting the need for further research into effective treatments and management strategies 2, 5.
• A second IVIg dose may be beneficial for patients with a poor prognosis, although further investigation is needed to confirm this 5.