From the Guidelines
Guillain-Barré syndrome typically starts with numbness and tingling in the extremities, often beginning in the feet and hands, and then progresses upward, followed by muscle weakness that develops rapidly over days to weeks. This condition is considered to be the consequence of a preceding infection that triggers an immune response, which is responsible for the demyelination and axonal degeneration of peripheral nerves and nerve roots 1. The immune system mistakenly attacks peripheral nerves, damaging the myelin sheath that insulates nerve fibers, disrupting nerve signal transmission and causing the characteristic ascending paralysis.
Key Points
- The condition often follows a respiratory or gastrointestinal infection by 1-3 weeks, with common triggers including Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus, or Zika virus 2, 3.
- Early recognition is crucial as some patients may develop respiratory muscle weakness requiring ventilatory support.
- The progression of symptoms typically peaks within 2-4 weeks, after which the condition plateaus before gradual improvement begins.
- Treatment with immunomodulatory agents, such as vaccines or biologic drugs, have also been associated with GBS in rare individuals 1.
Clinical Presentation
The clinical presentation of Guillain-Barré syndrome is heterogeneous, and several distinct clinical variants exist, including acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor sensory axonal neuropathy (AMSAN) 3.
Diagnosis and Management
Diagnosis of GBS is based on the patient history and neurological, electrophysiological, and cerebrospinal fluid (CSF) examinations 2, 3. Intravenous immunoglobulin and plasma exchange are equally effective in treating GBS; no other treatments have been proven to be effective 2. The efficacy of repeat treatment in patients who have shown insufficient clinical response is uncertain; nevertheless, this practice is common in patients who show deterioration after an initial treatment response 2. Clinical improvement is usually most extensive in the first year after disease onset and can continue for >5 years 2.
From the Research
Guillain-Barré Syndrome Onset
- The exact cause of Guillain-Barré syndrome (GBS) is unknown, but it is thought to be triggered by a preceding gastrointestinal or respiratory infection in most cases 4, 5.
- Approximately 50-70% of cases appear 1-2 weeks after a respiratory or gastrointestinal infection, or another immune stimulus that induces an aberrant autoimmune response targeting peripheral nerves and their spinal roots 4.
- The interplay between the microbial and host factors that dictate whether and how the immune response shifts towards autoreactivity is still unclear 4.
- Triggers for GBS include infection and (rarely) vaccination; cross-reactivity between infectious and neural epitopes has been well demonstrated, particularly for Campylobacter jejuni and motor axonal forms of GBS in which non-protein gangliosides are antigenic 6.
Clinical Presentation
- GBS typically presents with sensory symptoms and weakness over several days, often leading to quadriparesis 5.
- Approximately 70% of patients report a recent preceding upper or lower respiratory tract infection or gastrointestinal illness 5.
- Approximately 30% of patients require intubation and ventilation because of respiratory failure 5.
Pathophysiology
- GBS is a classically postinfectious, immune-mediated, monophasic polyradiculoneuropathy, and is the leading global cause of acquired neuromuscular paralysis 7.
- The immunopathological process driving nerve injury is ill-defined, and the disease activity and treatment response are currently monitored through interval neurological examination and outcome measures 7.