STEROIDS AND IVIG IN EBV-RELATED PEDIATRIC GUILLAIN-BARRE SYNDROME

Objectives: To assess the benefit of the combination of IVIG and steroids in the treatment of patients with EBV (Epstein Barr virus)-related GBS (Guillain-Barre syndrome).

Methods: We retrospectively reviewed 3 female patients-ages 5–15yrs (A,B,C) with clinical, biochemical and neurophysiologic evidence of GBS and serologic evidence of recent secondary EBV infection. All patients presented with ascending paralysis and cranial nerve involvement preceded by an upper respiratory tract illness. The patients were graded according to the GBS scale. All patients had cytoalbumino-dissociation in CSF. Neurophysiology studies at presentation were consistent with an acute segmental demyelinating polyneuropathy in patients A and C and with an acute axonal polyneuropathy in patient B. All patients were treated promptly with IVIG (2grams/kg). Patients A and B had a severe course with peak GBS score of 5 (ventilation assistance). Because of clinical worsening in these patients, high dose intravenous steroids were added to the regimen. Patient C had a less severe presentation with peak GBS score of 3 (walking with assistance) and was treated only with IVIG.

Results: Complete recovery (GBS score of 0) was achieved after 8 weeks for patients A and B and after 16 weeks for patient C.

Conclusions: Patients with EBV-related GBS who where treated with IVIG and steroids had a shorter recovery period than the patient who was treated with IVIG alone despite the more severe clinical and neurophysiology profile. Steroids may be a consideration as adjunctive therapy in patients with severe EBV-related GBS but larger trials are needed to further evaluate this benefit.