From the Guidelines
Guillain-Barré syndrome is an immune-mediated disease where the body's immune system mistakenly attacks the peripheral nerves, leading to damage of the myelin sheath or nerve axons, and resulting in muscle weakness and sensory signs. This condition typically occurs after an infection, such as a respiratory or gastrointestinal illness, and is thought to be caused by an aberrant immune response to these infections, as noted in a 2019 study published in Nature Reviews Neurology 1. The immune system, while fighting the infection, incorrectly identifies components of the peripheral nerves as foreign invaders, leading to an attack on the nerves. As a result, the nerves cannot transmit signals efficiently between the brain, spinal cord, and the rest of the body, causing the characteristic symptoms of muscle weakness that typically starts in the legs and can spread upward, sometimes affecting breathing and other vital functions.
The pathophysiology of Guillain-Barré syndrome involves an inflammatory response that damages the peripheral nervous system (PNS), leading to a range of clinical presentations, including acute flaccid paralysis, as discussed in the 2019 study 1. The disease can be classified into several subtypes, including acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor sensory axonal neuropathy (AMSAN), each with distinct electrophysiological characteristics. The exact trigger for Guillain-Barré syndrome varies, but common preceding infections include Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus, and Zika virus, as mentioned in the study 1.
Key points about the pathophysiology of Guillain-Barré syndrome include:
- The condition is an immune-mediated disease that damages the peripheral nerves
- The immune system mistakenly attacks the myelin sheath or nerve axons, leading to muscle weakness and sensory signs
- The disease typically occurs after an infection, such as a respiratory or gastrointestinal illness
- The condition can lead to a range of clinical presentations, including acute flaccid paralysis
- The disease can be classified into several subtypes, each with distinct electrophysiological characteristics, as noted in the 2019 study 1.
From the Research
Pathophysiology of Guillain-Barré Syndrome
The pathophysiology of Guillain-Barré Syndrome (GBS) is characterized by:
- An autoimmune response, where the immune system mistakenly attacks the peripheral nerves, leading to inflammation and damage [ 2 ]
- The involvement of T cells, macrophages, B cells, and complement in initiating and perpetuating attacks on gangliosides of Schwann cells and axons [ 3 ]
- Alterations in immune cell functioning, which may play a role in differences in GBS with age, alongside general age-related declines in reparative processes [ 3 ]
Key Factors Contributing to GBS
Some key factors that contribute to the development of GBS include:
- Autoimmune factors, such as antibodies, which can be removed through plasma exchange [ 2 ]
- Inflammation of peripheral nerves, which can lead to paralysis and other symptoms [ 2 ]
- The potential role of an aging immune system, which may contribute to the increased prevalence and severity of GBS with age [ 3 ]
Treatment and Management
Treatment and management of GBS may involve:
- Plasma exchange, which can remove antibodies and other potentially injurious factors from the blood stream [ 2 ]
- Intravenous immunoglobulin (IVIG), which can help to reduce the severity of symptoms [ 4, 5 ]
- Supportive care, such as mechanical ventilation, to manage respiratory failure and other complications [ 5 ]