Abstract: | Abstract. This paper reviews recent treatment strategies of immunemediated neuropathies, in particular it includes data regardingGuillain-Barré syndrome (GBS), chronic inflammatorydemyelinating polyneuropathy (CIDP), multifocal motor neuropathy(MMN), neuropathy with IgM monoclonal gammopathy and otherdysglobulinemic neuropathies. In the treatment of Guillain-Barré syndrome, there is no significant difference between IVIg,plasma exchange or plasma exchange followed by IVIg. However,for reasons of convenience and safety, IVIg is used as standardtreatment in most centers. There is so far insufficient evidencefor the use of corticosteroids in the therapy of GBS. Intreating CIDP corticosteroids, intravenous immunoglobulin andplasma exchange seem to be equally effective. However, the highcosts and relative lack of availability of IVIg, the onlyshort-term benefit and the invasive nature of the plasmaexchange procedure, and on the other hand serious long-term sideeffects of corticosteroids are the most important disadvantagesof these treatments and have to be taken into considerationbefore a decision about therapy can be made. In multifocal motorneuropathy the intravenous immunoglobulin therapy is the onlytreatment that has been shown to be effective in controlledtrials. However, inadequate response in a proportion ofpatients, high cost and variable availability of IVIg show theneed for the search of adjunctive immunosuppressive therapies.Neuropathies with IgM monoclonal gammopathy may improve aftertreatment with chemotherapeutic agents, though the long-termeffects are not known. In addition, such treatment modalitiesmay be associated with serious side effect and even severetoxicity. Recent data support the use of a new promising drug:Rituximab, a monoclonal antibody directed against the B cellsurface membrane marker CD 20.This article has been adapted from an ENS TeachingCourse in Istanbul, Turkey, June 2003. |