The identification of specific disorders caused by immune-mediated attack on Schwann cells and myelin have both improved and confused our understanding and classification of chronic inflammatory and immune-mediated neuropathies. Some investigators consider all neuropathies that respond to immunosuppressive treatment as chronic inflammatory demyelinating polyneuropathy (CIDP), regardless of whether there is evidence for demyelination. Other investigators rely on a very strict definition of CIDP and do not include variations. The goal of this article is to clarify the spectrum of these disorders and provide a logical classification scheme that has clinical utility and yet allows for new information that might distinguish new variations. Therapeutic strategies are also discussed.
CIDP (chronic inflammatory demyelinating polyneuropathy) - myelin-associated glycoprotein - CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, IgM paraproteinemia, cold agglutinins, and disialosyl antibodies) - paraprotein - MMN (multifocal motor neuropathy) - Lewis-Sumner syndrome - multifocal acquired demyelinating sensory and motor (neuropathy) - paraprotein - conduction block