Humoral factors in peripheral nerve disease

Humoral factors including soluble substances transported by the blood stream and factors released at a target tissue may play a role in diseases of the peripheral nervous system. Various criteria have to be met in order to accept humoral factors as potential pathogens. In this review these general criteria are discussed, including the evidence provided by plasma exchange therapy, demonstration of circulating or deposited autoantibodies and immune complexes, identification of antigenic molecules, animal model diseases, passive transfer experiments, and the demonstration of circulating factors not directed against specific targets. In acute, chronic, and chronic relapsing inflammatory polyneuropathies, and in the polyneuropathy associated with monoclonal gammopathy, humoral factors have been identified, but their exact pathogenic role is not fully understood. In the Lambert-Eaton myasthenic syndrome, a disorder of the motor nerve terminal, pathogenic IgG-antibodies have been demonstrated by passive transfer experiments. In the experimental animal model disorders, the acute and chronic variants of experimental allergic neuritis, humoral factors including antibodies to myelin basic proteins and galactocerebroside and nonspecific humoral factors may all contribute to the ultimate peripheral nerve damage, but their relative importance in relation to cell-mediated immune reactions is not yet clear.