Guillain Barré syndrome.
Guillain Barré syndrome is one of the best examples of a post infectious immune disease and offers insights into the mechanism of tissue damage in other more common autoimmune diseases. Controlled epidemiological studies have linked it to infection with Campylobacter jejuni in addition to other viruses including cytomegalovirus and Epstein Barr virus. The syndrome includes several pathological subtypes, of which the most common is a multifocal demyelinating disorder of the peripheral nerves in close association with macrophages. Evidence from histological examination of peripheral nerve biopsy and postmortem samples suggests that both cell mediated and humoral mechanisms are involved in the pathogenesis. Immunological studies suggest that at least one third of patients have antibodies against nerve gangliosides, which in some cases also react with constituents of the liposaccharide of C jejuni. In the Miller Fisher variant of the disease, these antiganglioside antibodies have been shown to produce neuromuscular block, and may in part explain the clinical signs of that disorder. Treatment with both intravenous immunoglobulin and plasma exchange reduces the time taken for recovery to occur, although mortality remains around 8%, with about 20% of patients remaining disabled.
Winer
J B
JB
Department of Neurology, Queen Elizabeth Hospital, Edgbaston, Birmingham B15 2TH, UK. j.b.winer@bham.ac.uk
D001323:Autoantibodies / Q000097:blood; D001369:Axons / Q000276:immunology; D001424:Bacterial Infections / Q000276:immunology; D004594:Electrophysiology; D005260:Female; D005732:Gangliosides / Q000276:immunology; D005938:Glucocorticoids / Q000627:therapeutic use; D020275:Guillain-Barre Syndrome / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D006801:Humans; D016756:Immunoglobulins, Intravenous; D008214:Lymphocytes / Q000276:immunology; D008264:Macrophages / Q000473:pathology; D008297:Male; D009469:Neuromuscular Junction / Q000276:immunology; D010951:Plasma Exchange; D011379:Prognosis
D001323:Autoantibodies; D005732:Gangliosides; D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; C039129:GQ1b ganglioside