Chronic inflammatory demyelinating polyradiculoneuropathy.
In chronic inflammatory demyelinating polyradiculopathy differing clinical subtypes are beginning to emerge as has already occurred with the Guillain-Barré syndrome. However, neither pathogenic correlates nor particular therapeutic approaches have yet been defined for these subgroups. The neurophysiological techniques of terminal latency index and of modified F ratio help differentiate chronic inflammatory demyelinating polyradiculopathy from IgM paraproteinaemic neuropathy. Diagnosis may be assisted by magnetic resonance imaging studies in which enlarged nerve roots and plexuses and gadolinium enhancement may be evident. Further insight into pathogenesis has come from studies showing pathogenic antibodies in a small percentage of patients. Immunohistological studies examining the presence of adhesion, co-stimulatory and antigen presenting molecules in nerve biopsies have shown that T cell activation can be initiated and perpetuated within nerve and that Schwann cells possess the necessary markers to function as antigen presenting cells. Recent clinical trials have confirmed the therapeutic short term efficacy of intravenous immunoglobulin and Prednisone.
Pollard
John D
JD
Department of Medicine, Room 475, University of Sydney, D06 Blackburn, Sydney, NSW 2006, Australia. jdpoll@med.usyd.edu.au
D001323:Autoantibodies / Q000276:immunology; D015415:Biomarkers / Q000032:analysis; D006801:Humans; D007107:Immune System / Q000276:immunology / Q000503:physiopathology; D007167:Immunotherapy / Q000379:methods / Q000639:trends; D008279:Magnetic Resonance Imaging; D010525:Peripheral Nerves / Q000187:drug effects / Q000276:immunology* / Q000503:physiopathology*; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy
D001323:Autoantibodies; D015415:Biomarkers