PubMed · 13203775
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A BERGLUND. 1954-08-05. Polyradiculoneuriter.. https://pubmed.ncbi.nlm.nih.gov/13203775/
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The annual incidence of Guillain-Barré Syndrome is 1.5 per 100000 people. It has an associated mortality of about 5%, with ten percent of patients severely disabled one year after the first onset of neurological signs. To treat this condition, specialist teams, access to intensive care and rehabilitation are essential and should be provided in appropriate hospital units. Neither oral nor intravenous corticosteroids are beneficial. Plasma exchange (PE) is the first line treatment having been found to improve outcomes in several randomised, controlled clinical trials. The indications for treatment have now been clarified. Two PE are recommended in patients who are able to walk (mild) with two additional PE if they deteriorate. In patients unable to walk unaided (moderate), four plasma exchanges are sufficient, likewise in those who require mechanically ventilation (severe form). It is not useful to add further PE in more severe disease or if there is no response. High-dose of intravenous immunoglobulin (0.4 g/kg daily for 5 days) and PE are equally effective in intermediate and severe forms. The choice between the two treatments depends on their respective contra-indications and local availability. A trial is underway to assess the indications and optimal dose of IVIg and number of PE to be used in different severities of disease.
Several therapies are currently used in dys-immune neuropathies including steroids,plasma exchange (PE), high-dose intravenous immunoglobulins(IVIg), and immuno-suppressive agents (IS). Even if there is substantial evidence that these treatments may improve the course of the neuropathy, their effectiveness is far from being complete and is sometime hampered by the occurrence of associated side effects. In Guillain-Barré syndrome (GBS),IVIg and PE are similarly effective in accelerating the recovery but there is still little evidence that they can reduce mortality or long-term disability. Recent reports on the association of intravenous methylprednisolone or interferon-beta (IFN-beta) to IVIg did not result in significant further improvement. In chronic inflammatory demyelinating polyradiculoneuropathy(CIDP) steroids, PE, and IVIG are initially similarly effective. The short-term effect of PE and IVIgand the side effects associated with the long-term use of steroids have prompted the use of several IS, interferon and,more recently, the anti-CD20 monoclonal-antibody Rituximab, but their efficacy has still to be proved in controlled studies. The recent identification of multifocal motor neuropathy(MMN) was shortly followed by the finding of an effective therapy. Almost 80% of patients respond toIVIg whose effect needs to be maintained with periodic infusions for long periods of time, and tends to decrease after several years. Also in this condition a number of immune modulating agents have been used to reduce the frequency or improve the effectiveness of IVIg,but their efficacy has not been sofar confirmed in randomized trials. Similar conclusions can be drawn for neuropathies associated with monoclonal gammopathies where only PE and IVIg have proved to be effective in controlled studies,while the promising initial results obtained with Rituximab in neuropathy associated IgM monoclonal gammopathy awaits confirmation from controlled trials.
We discuss two of the most common of the acquired inflammatory neuropathies: Guillain-Barre syndrome and chronic inflammatory demyelinating polyneuropathy, as well as their variants. We review their clinical presentation, electrophysiologic findings, and management, highlighting knowledge gained from the recent literature. Unfortunately, although treatments exist for both Guillain-Barre syndrome and chronic inflammatory demyelinating polyneuropathy, none are completely curative and all have significant potential side effects and/or expense. Better understanding of the underlying pathophysiology of these diseases is needed in order to develop more targeted therapies.