Antiglycolipid antibodies in Guillain-Barré syndrome and related diseases: review of clinical features and antibody specificities.
Guillain-Barré syndrome (GBS) is an acute inflammatory polyradiculoneuropathy that usually develops following a respiratory or intestinal infection. Although the pathogenic mechanisms of GBS have not been fully established, both humoral and cell-mediated immune factors have been shown to contribute to the disease process. Several antiglycosphingolipid (anti-GSL) antibodies have been found in the sera of patients with GBS or related diseases. Measurements of these antibody titers are very important in the diagnosis of GBS and in evaluating the effectiveness of treatments in clinical trials. The most common treatment strategies for these disorders involve plasmapheresis and the use of steroids for reducing anti-GSL antibody titers to ameliorate patients' clinical symptoms. Administration of intravenous immunoglobulin may also be beneficial in the treatment of neuropathies by suppressing the immune-mediated processes that are directed against antigenic targets in myelin and axons. In certain demyelinating neuropathies, the destruction or malfunctioning of the blood-nerve barrier, which results in the leakage of circulating antibodies into the peripheral nerve parenchyma, has been considered to be an initial step in development of the disease process. In addition, anti-GSL antibodies, such as anti-GM1, may cause nerve dysfunction and injury by interfering with the ion channel function at the nodes of Ranvier, where carbohydrate epitopes of glycoconjugates are located. These malfunctions thus contribute to the pathogenic mechanisms of certain demyelinating neuropathies.
Ariga
Toshio
T
Institute of Molecular Medicine and Genetics, Medical College of Georgia, Augusta, Georgia 30912, USA.
Yu
Robert K
RK
D000818:Animals; D000918:Antibody Specificity; D001323:Autoantibodies / Q000097:blood*; D006017:Glycolipids / Q000276:immunology*; D020275:Guillain-Barre Syndrome / Q000097:blood / Q000276:immunology* / Q000628:therapy; D006801:Humans; D010523:Peripheral Nervous System Diseases / Q000097:blood / Q000276:immunology / Q000628:therapy
D001323:Autoantibodies; D006017:Glycolipids