Current Therapeutic Options in Severe Guillain-Barre?? Syndrome

Abstract

The classical form of Guillain-Barré syndrome (GBS) refers to an acute monophasic demyelinating motor and sensory polyneuropathy characterized by symmetric ascending flaccid weakness, along with sensory impairment and, less commonly, autonomic perturbations. Pure motor axonal forms, axonal motor, and sensory forms, as well as pure autonomic forms, have also been identified. A complex immune-mediated process leads to segmental demyelination accompanied with axonal involvement in protracted cases. Establishing strategies of immunomodulation may therefore halt and even reverse the harmful autoimmune insult to peripheral nerves. The present article reviews the current immunomodulatory options in severe GBS. A recent Cochrane meta-analysis of 6 randomized studies showed no significant improvement using corticosteroids, including either oral or intravenous methylprednisolone. Combined methylprednisolone and immunoglobulins shortened the time lapse to regain independent walking. Plasmapheresis (PE) was the first effectively proven method of immunomodulation, followed by intravenous immunoglobulins (IVIG). Both methods are comparable in their beneficial effect and were used either separately or in combination, but PE was more frequently associated with severe adverse effects requiring cessation of therapy, including a bleeding diathesis. In addition, PE is feasible only in major referral centers requiring the appropriate equipment and trained personnel. In addition, younger children may be at risk for bleeding after insertion of wide catheters. Therefore, in cases of severe GBS, IVIG is recommended as the first-line drug using a total empiric dose of 2 g/kg administered over 2 consecutive days, especially in children proven highly effective with negligible adverse effects. In protracted cases, the addition of intravenous corticosteroids to IVIG should be considered, which may shorten the duration to regain independent walking. If such therapy fails, PE should be applied using centrifugal blood separators with 5% albumin as the substitute solution.