Guillain-Barré syndrome (GBS)

Characteristics

Guillain-Barré Syndrome (GBS) is the most common form of acute neuromuscular paralysis. It affects 1–2 per 100,000 people annually. GBS can occur at any age but is more common in men and increases with age. The syndrome causes rapidly progressive limb weakness, often with sensory disturbances and pain. About 25% of cases include respiratory insufficiency or severe autonomic dysfunction. Patients vary significantly in clinical features, severity, and progression. GBS subtypes include a motor form, a paraparetic form, and Miller Fisher Syndrome (MFS), characterized by ophthalmoplegia, ataxia, and areflexia. EMG and histological findings reveal further diversity, such as the demyelinating form (AIDP) and axonal forms (AMAN, AMSAN).

Treatment and Disease Course

Effective treatments for GBS include intravenous immunoglobulins (IVIg) and plasmapheresis. Many patients initially worsen during treatment before stabilizing and recovering over months or years. Although recovery occurs, it is often incomplete, leaving residual symptoms. In the Netherlands, 3% of patients die, 3% experience a recurrence, and 5–10% develop acute-onset chronic inflammatory demyelinating polyneuropathy (CIDP).

Cause

GBS is almost always triggered by an abnormal immune response to an infection. Common causes include infections with Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus, Zika virus, and Mycoplasma pneumoniae (especially in children). Bacterial infections can lead to the production of cross-reactive antibodies that attack gangliosides or other glycolipids in peripheral nerves. This occurs through molecular mimicry. The mechanism behind virus-related GBS remains unclear.

Research

In 1992, Erasmus MC, in collaboration with the Dutch GBS Study Group, published the first study demonstrating the efficacy of IVIg in GBS. Since then, Erasmus MC, the Dutch GBS Study Group, and the International GBS Outcome Study (IGOS) have conducted extensive research into the causes, diagnosis, treatment, and long-term outcomes of GBS.