1,721,030 research outputs found
Update on the treatment of chronic inflammatory demyelinating polyradiculoneuropathy
No full textPurpose of review Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic and often disabling neuropathy, which often responds to immune therapies including corticosteroids, plasma exchange, and high-dose intravenous immunoglobulin (IVIg). The reasons for choosing one of these therapies and the factors that may predict response to these therapies remain, however, unclear. It is also unclear whether other immune therapies may be useful in CIDP. We will here review the studies addressing these points in CIDP. Recent findings Recent studies have shown that IVIg are initially more frequently effective than steroids in CIDP even if steroids, when effective, have a more prolonged efficacy. Some clinical and immunological features including the presence of antibodies against proteins at the nodes of Ranvier were associated with specific response to therapy even if the data need to be confirmed in large series of patients. A few anecdotal studies also reported the efficacy in some patients of new immune therapies whose efficacy needs to be confirmed in controlled trials. Summary IVIg are initially more frequently effective than steroids in CIDP, but the latter have a more prolonged efficacy. Some clinical presentations and their association with specific antibodies reactions may help in predicting the response to specific treatment. The possible benefit of new immune therapies await confirmation from randomized studies
Multifocal Motor Neuropathy : Current Therapies and Novel Strategies
No full textMultifocal motor neuropathy (MMN) is a purely motor mononeuritis multiplex characterized by the presence of conduction block on motor but not on sensory nerves and by the presence of high titers of anti-GM1 antibodies. Several data point to a pathogenetic role of the immune system in this neuropathy, although this has not yet been proved. Several uncontrolled studies and randomized controlled trials have demonstrated the efficacy of therapy with high-dose intravenous immunoglobulin (IVIg) in MMN. However, this therapy has a short-lasting effect that needs to be maintained with periodic infusions. This can be partly overcome by the use of subcutaneous immunoglobulin (SCIg) at the same dose. The high cost and need for repeated infusions have led to the search for other immune therapies, the efficacy of which have not yet been confirmed in randomized trials. In addition, some therapies, including corticosteroids and plasma exchange, are not only ineffective but have been associated with clinical worsening. More recently, a number of novel therapies have been investigated in MMN, including interferon-β1a, the anti-CD20 monoclonal antibody rituximab and the complement inhibitor eculizumab. Preliminary data from open-label uncontrolled studies show that some patients improve after these therapies; however, randomized controlled trials are needed to confirm efficacy. Until then, IVIg (and SCIg) remains the mainstay of treatment in MMN, and the use of other immune therapies should only be considered for patients not responding to, or becoming resistant to, IVIg
A retrospective analysis of the effect of high dose intravenous steroid therapy in patients with immune and non-immune mediarted neuropathies
No full textHigh dose intravenous steroid therapy in patients with idiopathic neuropathy and suspected immunological involvement
No full textEfficacy and tolerability of different brands of intravenous immunoglobulin in the maintenance treatment of chronic immune mediated neuropathies
No full textHigh-dose intravenous immunoglobulin (IVIg) is effective in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN). Not all brands of IVIg are however licensed for these neuropathies. We reviewed six patients with CIDP and seven with MMN treated with maintenance therapy with IVIg from 2009 to 2013. In all patients, we measured the Medical Research Council (MRC) and Overall Neuropathy Limitation Scale (ONLS) scores before each infusion, registered the monthly dose and brand of IVIg, and recorded adverse events. Patients were treated for 25–60 months (mean 49 months) alternating different brands of IVIg including IgVena, Gammagard, Kiovig, and Flebogamma. Minor and transient side effects were equally observed with each brand. No difference in the MRC or ONLS scores was observed in relation to the brand of IVIg used. Chronic maintenance treatment with IVIg in patients with MMN and CIDP was not associated with a different tolerability or efficacy despite the use of different brands of IVIg
Treatment of pain and paraesthesias in immune mediated neuropathies: a retrospective analysis on the efficacy and tolerability of drugs for neuropathic pain
No full text
High dose intravenous steroid therapy in patients with idiopathic neuropathy and suspected immunological involvement
No full textIdiopathic chronic immune-mediated neuropathies: chronic inflammatory demyelinating polyradiculoneuropathy and multifocal motor neuropathy
No full textChronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic demyelinating neuropathy which is deemed to be caused by an immune attack against peripheral nerve myelin [1–3]. It is a rare neuropathy with a prevalence ranging from 0.8/10,000 in Japan [4] to 8.9/100,000 in Olmstead County, USA [5]. CIDP usually presents with a relatively symmetric chronic relapsing or chronic progressive sensorimotor impairment that evolves over the years and that, if untreated, may lead to a consistent disability. A few different clinical presentations have been reported [3, 6] broadening the spectrum of CIDP. These include multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy also known as LewisSumner syndrome, distal acquired demyelinating symmetric (DADS) neuropathy, pure motor CIDP, pure sensory CIDP including chronic immune sensory polyradiculopathy (CISP) and focal CIDP. These forms are currently considered to be variants of CIDP, even if the presence of some differences in their response to therapy does not permit to exclude that they represent different forms of demyelinating neuropathies
- …
