Definition and epidemiology
Guillain-Barré syndrome (GBS) is an acute, immune-mediated polyradiculoneuropathy and the commonest cause of acute flaccid paralysis in children since the near-elimination of poliomyelitis. Annual incidence is roughly 0.4–1.4 per 100,000 children, rising with age. It is typically post-infectious, following Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus, Mycoplasma pneumoniae, Zika or other infections by 1–4 weeks. The acute inflammatory demyelinating polyneuropathy (AIDP) subtype predominates in Western series; axonal variants (AMAN/AMSAN) are commoner in Asia, including India, and are associated with Campylobacter and anti-ganglioside antibodies. Miller Fisher syndrome (ophthalmoplegia, ataxia, areflexia) is an important variant.
Clinical features
The hallmark is a progressive, ascending, symmetrical flaccid weakness with areflexia/hyporeflexia. Typical features:
- Weakness starting in the legs and ascending; usually symmetric
- Diminished or absent deep tendon reflexes
- Distal paraesthesiae; sensory signs are often mild relative to weakness
- Pain (neuropathic/back/limb) is common in children and may dominate early, sometimes mimicking a refusal to walk
- Autonomic dysfunction — labile blood pressure/heart rate, arrhythmia, ileus, urinary retention
- Cranial nerve and bulbar involvement — facial weakness, dysphagia, and the feared respiratory muscle weakness
Progression is by definition within 4 weeks (most reach nadir by 2 weeks), distinguishing GBS from chronic inflammatory demyelinating polyneuropathy (CIDP).
Diagnosis
GBS is primarily clinical, supported by two investigations:
- CSF — albuminocytologic dissociation (raised protein with a normal cell count, typically <10–50 cells/µL). It may be normal in the first week, so a normal early CSF does not exclude GBS.
- Nerve conduction studies — demyelinating or axonal features supporting the diagnosis and subtype.
The Brighton Collaboration case definition grades diagnostic certainty: Level 1 (highest) requires the clinical picture plus supportive CSF and NCS; Level 2 the clinical picture with either CSF or NCS; Level 3 the clinical picture alone. MRI may show nerve-root enhancement. Exclude mimics: transverse myelitis/cord compression (look for a sensory level and sphincter involvement early), myasthenia, botulism, tick paralysis, hypokalaemia, and toxic neuropathies.
See the full criteria: Guillain-Barré Syndrome Algorithm
Red flags
- Respiratory compromise — declining vital capacity, rapid/shallow breathing, weak cough; monitor and have airway support ready (do not wait for hypoxia/hypercapnia)
- Bulbar weakness — dysphagia, pooling secretions, aspiration risk
- Autonomic instability — arrhythmia, BP swings (continuous monitoring)
- Rapid progression to non-ambulation
- A clear sensory level or early bladder/bowel dysfunction — reconsider a cord lesion
Management overview
Care is supportive plus immunotherapy. Admit; monitor respiratory function (serial FVC/NIF where feasible), swallowing, and cardiovascular autonomic status, escalating to HDU/PICU for ventilatory or autonomic compromise. Immunotherapy is indicated for non-ambulant or rapidly-progressing children: IVIG 2 g/kg over 2–5 days, or plasma exchange; the two are equally effective and are not combined. Corticosteroids are not effective in GBS and should not be used as monotherapy. Provide neuropathic pain control (gabapentin/carbamazepine; opioids with caution given ileus/autonomic risk), VTE prophylaxis in immobile adolescents, bowel/bladder care, and early physiotherapy/rehabilitation.
Prognosis in children is generally favourable, with most regaining independent walking over weeks to months, though axonal variants and those needing ventilation recover more slowly. Watch for treatment-related fluctuation and the small subset that evolves into CIDP.
References
- Sejvar JJ, et al. Brighton Collaboration GBS case definitions. Vaccine. 2011;29:599–612.
- Leonhard SE, et al. GBS diagnosis and management in ten steps. Nat Rev Neurol. 2019;15:671–683.
- Nelson Textbook of Pediatrics, 21st ed.
Decision support for qualified clinicians only — verify against current primary guidelines and your clinical judgement.