Guillain-Barré Syndrome Clinical Presentation and Disease Course

Clinical Presentation

The American Academy of Neurology notes that Guillain-Barré syndrome is characterized by rapidly progressive bilateral weakness typically starting in the legs and ascending to the arms and cranial muscles, accompanied by decreased or absent reflexes, often preceded by an infection within 6 weeks of symptom onset 1, 2
Bilateral ascending weakness, typically starting in the legs and progressing to the arms and cranial muscles, is a common presentation of Guillain-Barré syndrome 1, 2
Distal paresthesias or sensory loss that precede or accompany weakness are also characteristic of Guillain-Barré syndrome 1, 2
Decreased or absent reflexes in most patients at presentation and almost all patients at nadir is a key feature of Guillain-Barré syndrome 1
Pain, including muscular, radicular, or neuropathic pain, is frequently reported and can be an early symptom of Guillain-Barré syndrome 1, 3
Dysautonomia, including blood pressure/heart rate instability, pupillary dysfunction, and bowel/bladder dysfunction, can occur in patients with Guillain-Barré syndrome 1, 4
Acute or subacute onset with maximum disability typically reached within 2 weeks is typical of Guillain-Barré syndrome 1, 5

The disease course of Guillain-Barré syndrome is typically monophasic, with maximum disability reached within 2 weeks 1
Treatment-related fluctuations and relapses occur in a minority of patients (2-5%) with Guillain-Barré syndrome 3, 5
Respiratory failure develops in about 20% of patients with Guillain-Barré syndrome 5
Mortality is estimated at 3-10% even with best medical care for patients with Guillain-Barré syndrome 5
60-80% of patients with Guillain-Barré syndrome can walk independently 6 months after disease onset 5
Recovery from Guillain-Barré syndrome can continue for more than 3 years after onset 3

About two-thirds of patients with Guillain-Barré syndrome report symptoms of infection in the 6 weeks preceding disease onset 1, 2
Six pathogens have been temporally associated with Guillain-Barré syndrome in case-control studies, including Campylobacter jejuni, Cytomegalovirus, Hepatitis E virus, Mycoplasma pneumoniae, Epstein-Barr virus, and Zika virus 1, 5
Complement activation, macrophage infiltration, and edema are typical characteristics of affected peripheral nerves in Guillain-Barré syndrome 5

In children with Guillain‑Barré syndrome, diminished or absent deep‑tendon reflexes are the hallmark finding; loss of reflexes has been reported in 100 % of patients in some series and in 82‑93 % of patients in other series【6】.
The classic sensorimotor form of pediatric GBS is characterized by rapidly progressive, symmetrical limb weakness and sensory signs accompanied by absent or reduced tendon reflexes【6】.