From the Guidelines
Guillain-Barré Syndrome (GBS) is an acute immune-mediated polyneuropathy characterized by rapidly progressive, symmetrical muscle weakness that typically begins in the lower extremities and ascends upward, with the cardinal features including progressive weakness, diminished or absent deep tendon reflexes, and albuminocytologic dissociation in cerebrospinal fluid, as described in the 2019 study published in Nature Reviews Neurology 1. The features of GBS include:
- Progressive weakness that reaches its nadir within four weeks
- Diminished or absent deep tendon reflexes
- Albuminocytologic dissociation in cerebrospinal fluid (elevated protein with normal cell count)
- Antecedent respiratory or gastrointestinal infections, commonly with Campylobacter jejuni, cytomegalovirus, or Epstein-Barr virus, typically 1-3 weeks before symptom onset
- Sensory symptoms like paresthesias or numbness, which are usually less prominent than motor deficits
- Autonomic dysfunction, which can manifest as cardiac arrhythmias, blood pressure fluctuations, urinary retention, or ileus
- Respiratory muscle weakness, which may necessitate mechanical ventilation in severe cases GBS variants include:
- Acute inflammatory demyelinating polyneuropathy (AIDP)
- Acute motor axonal neuropathy (AMAN)
- Acute motor and sensory axonal neuropathy (AMSAN)
- Miller Fisher syndrome, characterized by ophthalmoplegia, ataxia, and areflexia The pathophysiology of GBS involves immune-mediated damage to peripheral nerves, with antibodies targeting components of peripheral nerve myelin or axons through molecular mimicry between microbial and nerve antigens, as discussed in the 2019 study published in Nature Reviews Neurology 1. According to the 2021 study published in Nature Reviews Neurology, the mortality rate for GBS is 2-10%, with disparities evident between regions, and about 20% of patients with GBS are unable to walk unaided 6 months after disease onset 1. The 2019 study published in Nature Reviews Neurology also notes that the probability of regaining walking ability can be calculated in individual patients using the modified Erasmus GBS outcome score (mEGOS) prognostic tool, and that about 80% of patients with GBS regain the ability to walk independently at 6 months after disease onset 1.
From the Research
Features of Guillain-Barré Syndrome
The features of Guillain-Barré Syndrome (GBS) include:
- Rapidly progressive and generally ascending symmetrical muscle weakness, accompanied by decreased or absent osteotendinous reflexes 2
- Inflammatory process that may affect the myelin or the axon 3
- Four clinical forms:
- Acute inflammatory demyelinating polyradiculoneuropathy
- Acute motor axonal neuropathy
- Acute sensory and motor axonal neuropathy
- The Miller-Fisher variant, characterized by ophthalmoplegia, ataxia, and areflexia, with little muscle weakness 3
- Diagnosis based on albumin-cytological dissociation and specific neurophysiological alterations of each clinical form 3
- Treatment depends on the degree of severity, with options including intravenous immunoglobulin (IVIg), plasmapheresis, and immunoadsorption 3
Clinical Manifestations
Clinical manifestations of GBS include:
- Limb weakness
- Areflexia
- Sensory loss
- Neuromuscular paralysis affecting the respiratory, facial, and bulbar functions 4
- Symmetrical ascending muscle weakness with reduced osteotendinous reflexes and variable sensory involvement 2
Treatment Options
Treatment options for GBS include:
- Plasmapheresis
- Intravenous immunoglobulin (IVIg)
- Glucocorticoids
- Supportive therapy in an intensive care unit for severe cases 2
- Rituximab for cases with antibodies against neurophysin and contactin 3
- Corticosteroid bolus in initial stages for severe axonal cases 3
Diagnosis and Differential Diagnoses
Diagnosis of GBS may be challenging due to overlapping symptoms with other neurological and infectious illnesses, requiring careful consideration of differential diagnoses, including:
- Exposure to toxins
- Central nervous system infections
- Metabolic or serum ion alterations
- Demyelinating pathologies
- Conditions amenable to neurosurgical intervention 2