Guillain-Barré Syndrome Diagnosis and Treatment

Introduction to GBS

Guillain-Barré Syndrome (GBS) typically presents 2-3 weeks after an upper respiratory tract infection (URTI) or gastrointestinal (GI) infection, according to the American Academy of Neurology 1, 2

About two-thirds of patients who develop GBS report symptoms of an infection in the 6 weeks preceding the onset of the condition, as reported by the American Academy of Neurology 3
Cytomegalovirus is one of the preceding infections associated with GBS, as noted by the American Academy of Neurology 4
Mycoplasma pneumoniae is another preceding infection linked to GBS, according to the American Academy of Neurology 5
Zika virus is also a preceding infection associated with GBS, as reported by the American Academy of Neurology 3

GBS is thought to be caused by an aberrant immune response to infections that results in damage to peripheral nerves, according to the American Academy of Neurology 1
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, as noted by the American Academy of Neurology 2

The classic presentation involves rapidly progressive bilateral weakness that typically begins in the legs and progresses to the arms and cranial muscles, as described by the American Academy of Neurology 4
Most patients with GBS reach their maximum disability within 2 weeks after symptom onset, according to the American Academy of Neurology 1
In patients who reach maximum disability within 24 hours of disease onset or after 4 weeks, alternative diagnoses should be considered, as recommended by the American Academy of Neurology 4
GBS is a monophasic illness, although some patients can deteriorate after first stabilizing or improving on therapy (treatment-related fluctuations), as noted by the American Academy of Neurology 1

Diagnosis of GBS is based on the patient history, neurological examination, electrophysiological studies, and cerebrospinal fluid (CSF) examination, according to the American Academy of Neurology 1
CSF typically shows a combination of increased protein level and normal cell count, though this may be normal early in the disease course, as reported by the American Academy of Neurology 2
The absence of an antecedent illness does not exclude a diagnosis of GBS, as infections can be subclinical, as noted by the American Academy of Neurology 3

Acute motor sensory axonal neuropathy (AMSAN) is a distinct clinical variant of GBS, as described by the American Academy of Neurology 1

Plasma exchange (200-250 ml/kg for 5 sessions) is a treatment option for GBS, as recommended by the American Academy of Neurology 6
Recovery is usually most extensive in the first year after disease onset but can continue for more than 5 years, according to the American Academy of Neurology 2
Recurrent episodes of GBS are rare, affecting 2-5% of patients, as reported by the American Academy of Neurology 5

Young children (<6 years) may present with nonspecific or atypical features such as poorly localized pain, refusal to bear weight, irritability, or unsteady gait, as noted by the American Academy of Neurology 4
Treatment-related fluctuations (TRFs) occur in 6-10% of patients and should be distinguished from clinical progression without initial response to treatment, according to the American Academy of Neurology 5