Guillain-Barré Syndrome Typically Presents 2-3 Weeks After URTI or GI Infection
Yes, Guillain-Barré Syndrome (GBS) typically presents 2-3 weeks after an upper respiratory tract infection (URTI) or gastrointestinal (GI) infection. 1
Antecedent Infections in GBS
- About two-thirds of patients who develop GBS report symptoms of an infection in the 6 weeks preceding the onset of the condition 1
- These infections are thought to trigger the immune response that causes GBS through molecular mimicry 2
- The most common preceding infections include:
- Campylobacter jejuni (gastrointestinal)
- Cytomegalovirus
- Hepatitis E virus
- Mycoplasma pneumoniae
- Epstein-Barr virus
- Zika virus 1
Pathophysiology of Post-Infectious GBS
- GBS is thought to be caused by an aberrant immune response to infections that results in damage to peripheral nerves 1
- Molecular mimicry between pathogen-borne antigens and gangliosides in peripheral nerves leads to cross-reactive antibodies 2
- In a subgroup of patients, serum antibodies are found against gangliosides, which are present at high densities in the axolemma and other components of peripheral nerves 1
- The subtype and severity of GBS are partly determined by the nature of the antecedent infection and specificity of antibodies 2
Clinical Presentation Timeline
- The classic presentation involves rapidly progressive bilateral weakness that typically begins in the legs and progresses to the arms and cranial muscles 1
- Most patients with GBS reach their maximum disability within 2 weeks after symptom onset 1
- In patients who reach maximum disability within 24 hours of disease onset or after 4 weeks, alternative diagnoses should be considered 1
- GBS is a monophasic illness, although some patients can deteriorate after first stabilizing or improving on therapy (treatment-related fluctuations) 1
Diagnostic Considerations
- Diagnosis of GBS is based on the patient history, neurological examination, electrophysiological studies, and cerebrospinal fluid (CSF) examination 1
- CSF typically shows a combination of increased protein level and normal cell count, though this may be normal early in the disease course 1
- The absence of an antecedent illness does not exclude a diagnosis of GBS, as infections can be subclinical 1
- HIV infection should be considered in patients presenting with GBS-like symptoms, as GBS can occur at any stage of HIV infection 3
Clinical Variants and Subtypes
- Several distinct clinical variants of GBS exist, including:
- Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) - most common in Western countries
- Acute motor axonal neuropathy (AMAN) - more common in Asia
- Acute motor sensory axonal neuropathy (AMSAN)
- Miller Fisher syndrome (MFS) - characterized by ophthalmoplegia, areflexia, and ataxia 4
- The specific variant may be influenced by the type of preceding infection 2
Prognosis and Treatment
- Despite current treatment, GBS remains a severe disease:
- About 25% of patients require artificial ventilation
- About 20% of patients are still unable to walk after 6 months
- 3-10% of patients die 4
- Treatment options include:
- Intravenous immunoglobulin (0.4 g/kg daily for 5 days)
- Plasma exchange (200-250 ml/kg for 5 sessions) 1
- Recovery is usually most extensive in the first year after disease onset but can continue for more than 5 years 1
- Recurrent episodes of GBS are rare, affecting 2-5% of patients 1
Clinical Pitfalls and Caveats
- Pain can be confusing in making the diagnosis, especially when it precedes the onset of weakness 4
- Young children (<6 years) may present with nonspecific or atypical features such as poorly localized pain, refusal to bear weight, irritability, or unsteady gait 1
- About 5% of patients initially diagnosed with GBS develop chronic inflammatory demyelinating polyradiculoneuropathy with acute onset (A-CIDP) 4
- Treatment-related fluctuations (TRFs) occur in 6-10% of patients and should be distinguished from clinical progression without initial response to treatment 1