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Chronic inflammatory demyelinating polyradiculoneuropathy: diagnostic criteria, clinal variants and response to therapy
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What is new in multifocal motor neuropathy in 2007-2008
No full textSeveral articles on multifocal motor neuropathy (MMN) have appeared in 2007 and 2008, broadening the boundaries of this neuropathy and improving its diagnosis. A number of retrospective studies confirmed the prolonged efficacy of high-dose intravenous immunoglobulins (IVIg) in this neuropathy. The efficacy of other immune therapies was not however, confirmed in other studies in MMN including a randomized controlled trial on mycophenolate mofetil as adjunctive therapy to IVIg. These negative results and the lack of new other studies on the cause of MMN should lead to some reconsideration on its pathogenesis
Chronic inflammatory demyelinating polyradiculoneuropathy and variants : where we are and where we should go
No full textChronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic and often disabling sensory motor neuropathy postulated as caused by an immune attack against peripheral nerve myelin. In addition to a classic sensory-motor polyneuropathy, other phenotypes of CIDP have been described including the Lewis-Sumner syndrome, distal acquired demyelinating symmetric (DADS) neuropathy, pure motor CIDP, pure sensory CIDP including chronic immune sensory polyradiculopathy (CISP), and focal CIDP. These phenotypes are currently considered to be variants of CIDP, even if the possibility that they represent different demyelinating neuropathies cannot be fully excluded considering differences in their response to therapy. Several data support the role of the immune system in the pathogenesis of CIDP even if the precise targets and actors (antibodies and lymphocytes) of this immune response remain uncertain. Recent studies have shown that the therapeutic response may differ in patients with peculiar clinical presentations supporting the hypothesis that different pathogenetic mechanisms may underlie the heterogeneity of CIDP. The majority of patients with CIDP show improvement after immune therapies including corticosteroids, plasma exchange, and high-dose intravenous immunoglobulin (IVIg). It remains unclear why none of the other immune therapies that were reported to be variably effective in other immune disorders proved to be effective also in CIDP
Antigenic determinants in IgM parapotein-related neuropathies
No full textThe association of neuropathy with IgM paraprotein has been known for several years, but only recently the pathogenetic relevance of this association has been clarified. Reactivity of the paraprotein with several neural antigens has been reported even if their pathogenetic relevance is not always clear. IgM binding to the Myelin-associated glycoprotein (MAG) is associated with a homogeneous demyelinating neuropathy, deposits of the paraprotein and complement on nerve myelin, and improvement concomitantly to anti-MAG IgM reduction. In particular, treatment with rituximab durably improved the neuropathy in two thirds of the patients, particularly those with moderately increased anti-MAG titres, which might be more easily reduced by this treatment. Several other IgM reactivities with nerve antigens have been reported in these patients, including several gangliosides and sulfatide even if, with the only exceptions of anti-sulfatide and anti-GQ1b ganglioside reactivities, their possible pathogenetic relevance remains to be established
Treatment of dysimmune neuropathies
No full textSeveral 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-Barre 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
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