Guillain-Barré Syndrome

Pathophysiology

Guillain-Barré Syndrome is an immune-mediated disorder triggered by a previous infection in approximately two-thirds of patients, with the immune system generating antibodies against pathogens that cross-react with components of peripheral nerves, as suggested by the American Academy of Neurology 1

Patients with Guillain-Barré Syndrome often present with progressive bilateral weakness, areflexia, and mild sensory symptoms, with key diagnostic features including albuminocytologic dissociation, characterized by elevated CSF protein with normal cell count, as recommended by the American Academy of Neurology and the National Institute of Neurological Disorders and Stroke 1
Electrodiagnostic studies, including nerve conduction studies and electromyography, are strongly recommended to support diagnosis, especially in atypical presentations, and may show reduced conduction velocities, reduced sensory and motor evoked amplitudes, and abnormal temporal dispersion, as noted by the American Academy of Neurology 1
Patients may have absent or decreased tendon reflexes in affected limbs, autonomic dysfunction, and reach maximum disability within 2 weeks, with about two-thirds of patients reporting an infection in the 6 weeks before onset, commonly Campylobacter jejuni, according to the American Academy of Neurology 1
Young children (<6 years) may present with atypical features, including poorly localized pain, refusal to bear weight, irritability, meningism, and unsteady gait, as noted by the American Academy of Neurology 1
CPK levels are typically normal or only mildly elevated in GBS, helping to differentiate between GBS and myositis, as noted by the American Academy of Neurology and the European Neurological Society 2, 3

Lumbar puncture should be performed immediately for patients with suspected GBS, as it can reveal albuminocytologic dissociation, a key diagnostic feature of GBS, as recommended by the American Academy of Neurology 1
The modified Erasmus GBS outcome score (mEGOS) can predict recovery in patients with GBS, as noted by the American Academy of Neurology and the European Academy of Neurology 1, 2

Intravenous immunoglobulin (IVIg) at a dosage of 0.4 g/kg/day for 5 consecutive days is recommended for patients unable to walk unaided within 2 weeks of symptom onset, as recommended by the American Academy of Neurology 1, 3, 2
Plasma exchange (PE) is an alternative to IVIg, with a dosage of 12-15 L in 4-5 exchanges over 1-2 weeks, and is effective when initiated within 4 weeks of symptom onset, as noted by the American Academy of Neurology and the European Academy of Neurology 1, 3, 2
Corticosteroids are not recommended as monotherapy for idiopathic GBS, but may be considered in forms related to immune checkpoint inhibitors, as noted by the European Academy of Neurology and the American Academy of Neurology 3, 2
The European Academy of Neurology suggests considering gabapentinoids, tricyclic antidepressants, or carbamazepine for pain management in GBS patients, with a comprehensive treatment plan including pharmacological and non-pharmacological interventions 4

Approximately 60-80% of patients are able to walk independently 6 months after the onset of the disease, with or without treatment, and recovery can continue for more than 3 years after onset, as noted by the American Academy of Neurology and the European Academy of Neurology 1, 2
Approximately 20% of patients are still unable to walk after 6 months, and the mortality rate is 3-10% despite optimal treatment, with factors associated with poor prognosis including advanced age, rapid progression of symptoms, need for mechanical ventilation, and severe axonopathy on electrophysiological studies, as noted by the European Academy of Neurology and the American Academy of Neurology 3, 1

The annual global incidence of GBS is approximately 1-2 cases per 100,000 person-years, with higher incidence rates reported in some regions (up to 2.1-2.27 cases per 100,000), as noted by the American Academy of Neurology and the European Academy of Neurology 1, 5
Campylobacter jejuni is the most frequently identified infectious trigger worldwide, and approximately two-thirds of patients report an infection in the 4-6 weeks preceding onset, as noted by the European Academy of Neurology and the American Academy of Neurology 5
Vaccines are rarely associated with GBS, with the 1976 swine flu vaccine showing a 7.3-fold increased risk, but subsequent influenza vaccines have shown only approximately one additional GBS case per million vaccinations, as noted by the American Academy of Neurology and the European Academy of Neurology 1, 6