Guillain-Barré Syndrome in Children
Definition and Epidemiology
Guillain-Barré Syndrome (GBS) is an acute immune-mediated polyneuropathy characterized by rapidly progressive, ascending flaccid paralysis. In children, the annual incidence is 0.4-1.3 cases per 100,000 population. While it can occur at any age, there are two peak age distributions in children: during early childhood (3-6 years) and in adolescence.
Pathophysiology and Subtypes
Pathophysiological Mechanism:
- Autoimmune response targeting peripheral nerves and nerve roots
- Molecular mimicry between infectious agents and nerve components
- Demyelination and/or axonal damage
- Inflammatory infiltrates and complement activation
Major Subtypes:
- Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)
- Most common form in Western countries
- Primary demyelination with secondary axonal damage
- Usually good recovery potential
- Acute Motor Axonal Neuropathy (AMAN)
- More common in Asia and Latin America
- Pure motor involvement
- Primary axonal damage
- Acute Motor-Sensory Axonal Neuropathy (AMSAN)
- Severe form with poor prognosis
- Both motor and sensory involvement
- Extensive axonal damage
- Miller Fisher Syndrome (MFS)
- Characterized by ophthalmoplegia
- Ataxia and areflexia
- Anti-GQ1b antibodies often present
Triggering Factors:
- Infections:
- Campylobacter jejuni (most common)
- Cytomegalovirus
- Epstein-Barr virus
- Mycoplasma pneumoniae
- SARS-CoV-2
- Zika virus
- Vaccinations (rare)
- Surgery
- Trauma
Clinical Manifestations
Classical Presentation:
- Motor Symptoms:
- Progressive, symmetric ascending weakness
- Proximal and distal muscle involvement
- Decreased or absent deep tendon reflexes
- Respiratory muscle weakness (potential)
- Sensory Symptoms:
- Paresthesias
- Numbness
- Neuropathic pain
- Proprioception disturbances
- Autonomic Dysfunction:
- Cardiac arrhythmias
- Blood pressure fluctuations
- Gastrointestinal dysmotility
- Urinary retention
- Sudomotor dysfunction
Pediatric-Specific Features:
- More rapid progression compared to adults
- Higher incidence of facial nerve involvement
- Pain more common as presenting symptom
- Better overall prognosis than adults
- Increased risk of posterior reversible encephalopathy syndrome (PRES)
Diagnosis
Diagnostic Criteria:
- Required Features:
- Progressive weakness in more than one limb
- Areflexia or hyporeflexia
- Supportive Features:
- Progressive symptoms over days to weeks
- Relative symmetry
- Mild sensory symptoms
- Cranial nerve involvement
- Autonomic dysfunction
- Absence of fever at onset
Diagnostic Studies:
- CSF Analysis:
- Albuminocytologic dissociation
- Elevated protein with normal cell count
- May be normal in first week
- Electrodiagnostic Studies:
- Nerve conduction studies (NCS)
- Electromyography (EMG)
- F-wave analysis
- H-reflex studies
- Serological Testing:
- Anti-ganglioside antibodies
- Recent infection workup
- Inflammatory markers
- Imaging:
- MRI with gadolinium (nerve root enhancement)
- Chest X-ray (respiratory status)
Management
Acute Treatment:
- Immunotherapy:
- Intravenous Immunoglobulin (IVIG):
- Dose: 2g/kg total
- Given over 2-5 days
- First-line treatment in children
- Plasmapheresis:
- Alternative to IVIG
- Technical challenges in small children
- 5 exchanges over 7-10 days
- Intravenous Immunoglobulin (IVIG):
Supportive Care:
- Respiratory Support:
- Regular monitoring of respiratory function
- Vital capacity measurements
- Early intubation if needed
- Ventilatory support protocols
- Pain Management:
- Regular pain assessment
- Neuropathic pain medications
- Non-pharmacological interventions
- Prevention of Complications:
- DVT prophylaxis
- Pressure ulcer prevention
- Nutritional support
- Early rehabilitation
Rehabilitation:
- Physical Therapy:
- Range of motion exercises
- Progressive strengthening
- Gait training
- Occupational Therapy:
- Activities of daily living
- Adaptive equipment
- School reintegration
- Speech Therapy:
- Swallowing assessment
- Communication aids if needed
Monitoring and Prognosis
Clinical Monitoring:
- Respiratory function:
- Vital capacity
- Negative inspiratory force
- Oxygen saturation
- Blood gases
- Cardiovascular parameters
- Neurological status
- Pain levels
- Autonomic function
Prognosis:
- Favorable Factors:
- Younger age
- Early treatment
- AIDP subtype
- Absence of mechanical ventilation
- Poor Prognostic Factors:
- Rapid progression
- Need for ventilation
- Axonal forms (AMAN/AMSAN)
- Delayed treatment
Long-term Outcomes:
- Complete recovery: 60-80% of children
- Residual deficits: 20-40%
- Mortality: <5% in children
- Recurrence rate: 1-6%
References and Further Reading
- Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barré syndrome. Lancet. 2016;388(10045):717-727.
- Roodbol J, et al. Recognizing Guillain-Barré syndrome in preschool children. Neurology. 2011;76(9):807-810.
- Ryan MM. Pediatric Guillain-Barré syndrome. Curr Opin Pediatr. 2013;25(6):689-693.
- van den Berg B, et al. Guillain-Barré syndrome: pathogenesis, diagnosis, treatment and prognosis. Nat Rev Neurol. 2014;10(8):469-482.
Note: This document is intended for medical professionals and should be regularly updated based on new research and guidelines. Clinical judgment should always be exercised in the management of individual patients.
