Guillain-Barré Syndrome, Chronic Inflammatory Demyelinating Polyradiculoneuropathy, and Axonal Degeneration and Regeneration

Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) are acquired demyelinating diseases of the peripheral nervous system (PNS), characterized by progressive or relapsing proximal and distal muscle weakness with possible sensory loss (Saperstein et al., 2001; Hughes and Cornblath, 2005). Historically GBS was described in 1916 in two French soldiers as an acute, postinfectious paralysis with elevated CSF protein but without cells. At that time also epidemics of poliomyelitis have occurred producing “Landry paralysis”, as a direct consequence of enterovirus infection, and in contrast to GBS is usually asymmetrical and associated with CSF pleocytosis. In the following decades, with the availability of electrodiagnosis the clinical hallmarks of GBS have been described more precisely. Estimated annual disease incidence in industrialized countries is up to 2/100,000. In contrast to CIDP, about 50% of GBS cases are linked with preceding infections, typically diarrhea caused by C. jejuni (Enders et al., 1993) or infections of the respiratory tract (Hadden et al., 2001). In GBS acute respiratory failure or cardiac arrhythmias due to dysfunction of the autonomic nervous system cause at least 3% mortality even in specialized centers, especially in elderly patients with rapid disease progression and those suffering from multimorbidity (Hughes and Cornblath, 2005). In addition, both in GBS and CIDP, residual symptoms and chronic fatigue may lead to long-lasting disability in about half (Merkies et al., 1999).