Exchange Transfusion: A Low-Cost Alternative for Severe Childhood Guillain-Barré Syndrome

Abstract

The high cost and nonavailability of plasmapheresis and intravenous immunoglobulin are prohibitive for the treatment of Guillain-Barré syndrome in resource-poor settings. Exchange transfusion can be an alternative therapy for severe disease in children. The effectiveness of exchange transfusion was evaluated in nine children (median age 6 years) with severe Guillain-Barré syndrome (functional disability score ≥ 4). All patients had lower limb weakness for a median duration of 4 days (range 2—16 days). Upper limb weakness (n = 8), respiratory involvement (n = 7), pooling of oral secretions (n = 3), and facial palsy (n = 1) were other motor signs. Four children developed respiratory failure; three were ventilated. Other clinical features included leg pains, meningismus and Lasègue's sign, backache, excessive sweating, and hemodynamic instability. Two children had albuminocytologic dissociation on cerebrospinal fluid analysis. Nerve conduction velocity testing was done in three patients; two had suggestive findings. One course of exchange transfusion could be performed in seven patients. Six children (86%) responded well with one or more of the following: a halt in progressive motor weakness, improvement in leg pains, meningismus, and straight-leg raising within 24 to 48 hours; one could be weaned off the ventilator by 60 hours. All had improvement in motor power of at least one muscle group by one grade within 3 to 7 days. Two patients died, whereas five (71% of the treatment group) were discharged and could walk independently by 4 months. Two untreated ones had died. Exchange transfusion seems to be a safe and effective alternative for severe Guillain-Barré syndrome. It should be offered whenever intravenous immunoglobulin or plasmapheresis is not available or affordable. Replacement of immunosaturated red cells, removal of activated T cells and monocytes, and transient thrombocytopenia are possible explanations for its effectiveness in addition to removal of pathogenic autoantibodies, immune complexes, cytokines, and complements. (J Child Neurol 2006;21:960—965; DOI 10.2310/7010.2006.00224).