18PGuillain-Barré syndrome in children: insights from a 20-year retrospective cohort study at a tertiary referral center

Abstract

Guillain-Barré syndrome (GBS) is the leading cause of acute flaccid paralysis in children. It is characterized by rapid-onset, bilateral, and symmetrical sensorimotor symptoms, typically progressing from the lower to upper limbs. Although clinical presentation may vary, severe cases can involve autonomic dysfunction. Electrophysiological studies are key for subtype classification: acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor-sensory axonal neuropathy (AMSAN). Rare variants include Miller Fisher syndrome, the pharyngo-cervical-brachial variant, and Bickerstaff encephalitis. This retrospective observational study analyzed the natural history of pediatric GBS through electronic medical records of patients followed in a neuromuscular clinic over the past 20 years. A total of 118 children (mean age: 7 years) were included. Time from symptom onset to medical attention averaged 3 days. Common initial symptoms were pain, distal weakness, and gait disturbance. Most patients presented with ascending and distal weakness; 40 showed atypical features, mainly bulbar involvement. At admission, ascending weakness, distal weakness, and generalized areflexia were predominant. Electrophysiological subtypes were: AMAN (56%), AIDP (30%), AMSAN (9%), and Miller Fisher (5%). Cytoalbuminologic dissociation was present in 44% of lumbar punctures. Anti-GM1 antibodies were the most frequently detected. Loss of ambulation occurred in 33%, with recovery within 2–3 months. Central nervous system involvement was observed in 15 patients, presenting with brainstem edema, rhombencephalitis, and diffuse spinal cord abnormalities on MRI. Severe cases (Paradiso scale Grades IV–V) responded well to combined therapy with high-dose methylprednisolone pulses plus plasmapheresis or immunoadsorption. In IVIG-refractory patients, this combination also proved effective. At one year, 21 patients had sequelae, primarily distal weakness. During follow-up, 9 patients showed progression to chronic inflammatory demyelinating polyneuropathy (CIDP), requiring long-term immunomodulatory management. In conclusion, pediatric GBS shows heterogenous onset. AMAN was the most prevalent subtype. Anti-GM1 antibodies were common. Functional recovery, including ambulation, generally occurred within 6 months, with a low incidence of long-term sequelae. In severe cases unresponsive to IVIG, a favorable response was observed with combined therapy using methylprednisolone pulses and plasmapheresis or immunoadsorption.