From bench to bedside--experimental rationale for immune-specific therapies in the inflamed peripheral nerve

Abstract

Guillain-Barré syndrome and chronic inflammatory demyelinating polyradiculoneuropathy are autoimmune-mediated inflammatory diseases of the PNS. In recent years, substantial progress has been made towards understanding the immune mechanisms that underlie these conditions, in large part through the study of experimental models. Here, we review the available animal models that partially mimic human Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy, and discuss the wide range of therapeutic approaches that have been successfully established in these models of inflammatory neuropathies. Transfer of this preclinical knowledge to patients has been far less successful, and inflammatory neuropathies are still associated with significant morbidity and mortality. We will summarize successful therapeutic trials in human autoimmune neuropathies to provide a vantage point for the transfer of experimental treatment strategies to clinical practice in immune-mediated diseases of the peripheral nerve.