Child with Guillain-Barré Syndrome Responding to Plasmapheresis: A Case Report

Abstract

Intravenous immunoglobulin (IVIG) has long been regarded as the first-line treatment for Guillain-Barré syndrome (GBS), with plasmapheresis only being reserved for severe cases or used as an additional therapy of unproven efficacy. Here, we present the case of a 9-year-old girl with acute motor axonal neuropathy (AMAN), a rapidly progressive subtype of GBS that caused her to fall into respiratory failure. The patient failed to show a response 10 days after starting IVIG, but showed rather quick improvement with plasmapheresis. She received a total of 5 sessions of plasmapheresis on alternate days over a course of 8 days. Before starting plasmapheresis, her muscle strength was 2/5 in both upper limbs and 1/5 in both lower limbs, and she was dependent on mechanical ventilation. Following the first session, her power improved from 2/5 to 4/5 in the upper limbs, and the gag and sucking reflexes were recovered. On day 3, after the second session was initiated, she was extubated successfully (having been on a ventilator for 2 weeks) and remained on continuous positive airway pressure for the next 48 h, after which she was on room air. In addition, she was having hypertension from the first day of the diagnosis (which was due to autonomic instability), which improved after clonidine to maintain her blood pressure. She was also initially having urinary retention, then was off Foley’s catheter. The patient was discharged from the hospital 2 weeks following the first session of plasmapheresis, with power grade 4/5 in both her upper and lower limbs. Her cranial nerves had recovered fully, and she was able to walk with aids.