What is Guillain-Barré Syndrome (GBS)?

From the Guidelines

Guillain-Barré Syndrome (GBS) is a rare, immune-mediated disease of the peripheral nerves and nerve roots, usually triggered by infections, characterized by ascending bilateral symmetrical weakness, sensory signs, and autonomic dysfunction.

Key Features of GBS

• Annual global incidence of approximately 1–2 per 100,000 person-years 1
• More frequent in males than in females, with increasing incidence with age 1
• Clinical presentation is heterogeneous, with several distinct clinical variants, including acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor sensory axonal neuropathy (AMSAN) 1
• Diagnosis is based on patient history, neurological, electrophysiological, and cerebrospinal fluid (CSF) examinations 1
• Disease progression can be rapid, with most patients reaching their maximum disability within 2 weeks, and about 20% developing respiratory failure requiring mechanical ventilation 1
• Autonomic nervous system involvement can lead to cardiac arrhythmias, blood pressure instability, and mortality, estimated at 3–10% even with best medical care available 1

Pathogenesis and Triggers

• Thought to be caused by an aberrant immune response to infections, resulting in damage to peripheral nerves 1
• Preceding infections, such as Campylobacter jejuni, cytomegalovirus, and Epstein-Barr virus, can trigger different types of GBS 1
• Molecular mimicry between infectious agents and human nerve gangliosides can lead to the production of cross-reactive antibodies, activating complement and damaging nerves 1

Diagnosis and Management

• Diagnosis can be challenging, especially in low-income and middle-income countries (LMIC), due to limited facilities for CSF examination and nerve conduction studies 1
• Differential diagnosis depends on the clinical presentation and variant of GBS, and may include other infectious, autoimmune, and inflammatory conditions 1
• Management involves early recognition, admission to the intensive care unit, treatment indication and selection, monitoring and treatment of disease progression, and management of complications and sequelae 1

From the Research

Definition and Overview 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.
• It is a rare disease representing the most frequent cause of acute flaccid symmetrical weakness of the limbs and areflexia usually reaching its peak within a month 3.
• GBS is a potentially devastating yet treatable disorder, and it is the leading global cause of acquired neuromuscular paralysis 4.

Causes and Pathogenesis

• The etiology and pathogenesis of GBS remain largely enigmatic, and the syndrome results in death or severe disability in 9-17% of cases despite immunotherapy 3.
• GBS is linked to Campylobacter infection, but less than 0.1% of infections result in the syndrome 3.
• Activated macrophages and T cells and serum antibodies against gangliosides are observed, but their significance is unclear 3.

Clinical Features and Diagnosis

• Diagnosis of GBS relies on clinical features, supported by laboratory findings and electrophysiology 4.
• 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.
• Additionally, overlap syndromes exist, such as GBS-MFS overlap 2.

Treatment and Prognosis

• Both intravenous immunoglobulin (IVIg) and plasma exchange (PE) are effective in GBS, with IVIg usually being the preferred treatment 2.
• About 10% of GBS patients have a secondary deterioration within the first 8 weeks after start of IVIg, requiring repeated IVIg treatment 2.
• About 25% of patients require artificial ventilation during a period of days to months, about 20% of patients are still unable to walk after 6 months, and 3-10% of patients die 2.
• A larger increase in serum IgG levels after standard IVIg treatment seems to be related to an improved outcome after GBS 2.