79 - Acquired inflammatory demyelinating and axonal neuropathies

Abstract

Although diabetes is the leading cause of polyneuropathy worldwide, inflammatory neuropathies are a particularly important group of disorders because many of them are among the most treatable neuropathies encountered in clinical practice. Treatment of inflammatory neuropathies has advanced as the understanding of the immunopathology of this group of disorders has grown. The family of inflammatory neuropathies can be subdivided in several ways. Perhaps the most fundamental way is to separate acute from chronic processes. The inflammatory demyelinating and axonal neuropathies may be viewed as a heterogeneous collection of disorders that share the common feature of autoimmunity underlying their pathogenesis. A few of the subtypes of inflammatory neuropathies have well-recognized antibodies that clearly react with nerve antigens, suggesting a plausible pathogenic relationship. Among other inflammatory neuropathies antibodies may be identified, but their role in pathogenesis remains obscure. In others, cell-mediated immunity may be the primary pathogenic mechanism. Some disorders seem to be triggered by an antecedent infection, but in most, the inciting event is unknown. It remains uncertain why in some forms of neuropathy inflammation is self limited, whereas at other times a long-lasting autoimmune attack persists. Despite the large number of unanswered questions regarding inflammatory neuropathies, as a group, they make up the most treatable of the generalized neuropathic disorders. It is likely that even better treatments may follow an improved understanding of the pathogenesis of the inflammatory neuropathies.