Chapter 38 - Guillain-Barre Syndrome

Abstract

Guillain-Barre syndrome (GBS) has emerged as the most frequent cause of acute flaccid paralysis worldwide. Its most frequent form, acute inflammatory demyelinating polyneuropathy (AIDP), is the prototypic acquired demyelinating disease of the peripheral nervous system. The importance of GBS in this text lies both in its own prominence as a major cause of neurologic morbidity and in the similarities and contrasts with acquired demyelinating disorders of the central nervous system. This chapter outlines the history of GBS, followed by its clinical manifestations, pathology of AIDP, pathophysiology, and molecular genetics. It is noted that GBS and multiple sclerosis (MS) represent the major immune-mediated disorders of the PNS and the CNS, respectively. The data for GBS suggests that the immunologic mechanism can involve molecular mimicry, at least in some GBS variants. Finally, in both GBS and MS, it is likely that multiple mechanisms render the axon vulnerable. These mechanisms include damage as a bystander to inflammatory disease, as a consequence of the intimate cell-cell interactions between the myelin-forming cell and axon, and possibly as the target of the immune attack.