Chapter 22 Antibodies to glycoconjugates in neuropathy and motor neuron disease

Increased titers of antibodies that recognize carbohydrate determinants of glycolipids or glycoproteins glycoconjugates) are associated with distinct neuropathic syndromes. This chapter describes the syndromes that are associated with anti-glycoconjugate antibodies. The chapter discusses what is known about the specificity of the antibodies and the mechanisms that might be responsible for their development. Clinical syndromes associated with anti-glycoconjugate antibodies include demyefinating neuropathy, motor neuron disease, and sensory neuropathy. Isolated cases of neuropathy associated with monoclonal IgM antibodies to other glycolipids have been reported. Anti-glycoconjugate antibodies share several important characteristics. They are typically directed against carbohydrate determinants, they are frequently IgMs and occur as monoclonal gammopathies, and they are present in low titers in many normal individuals. Many of the anti-carbohydrate antibodies occur as IgM monoclonal gammopathies, suggesting that they are derived from single B-cell clones which undergo transformation. However, it is not known how the B-cells that secrete anti-glycoconjugate antibodies in patients with neuropathy become stimulated or whether they respond to the same regulatory mechanisms as normal B-cells.