mycophenolic-acid and Motor-Neuron-Disease

Multifocal motor neuropathy (MMN) is characterised by progressive, predominantly distal, asymmetrical limb weakness and usually multiple partial motor nerve conduction blocks. Intravenous immunoglobulin (IVIg) is beneficial but the role of immunosuppressive agents is uncertain. This is an update of a review first published in 2002 and previously updated in 2003, 2005, 2008 and 2011.. To assess the effects of immunosuppressive agents for the treatment of multifocal motor neuropathy.. On 22 September 2014 we searched the Cochrane Neuromuscular Disease Group Specialized Register, CENTRAL, MEDLINE, EMBASE and LILACS for trials of MMN. We also searched two trials registers for ongoing studies.. We planned to include randomised controlled trials (RCTs) and quasi-RCTs. We considered prospective and retrospective case series and case reports in the Discussion.. Two review authors searched the titles and abstracts of the articles identified and extracted the data independently.. Only one RCT of an immunosuppressive or immunomodulatory agent has been performed in MMN. This study randomised 28 participants and showed that mycophenolate mofetil, when used with IVIg, did not significantly improve strength, function or reduce the need for IVIg. No serious adverse events were observed. The study was deemed at low risk of bias. We summarised the results of retrospective and prospective case series in the discussion.. According to moderate quality evidence, mycophenolate mofetil did not produce significant benefit in terms of reducing need for IVIg or improving muscle strength in MMN. Trials of other immunosuppressants should be undertaken.

Topics: Drug Therapy, Combination; Humans; Immunoglobulins, Intravenous; Immunologic Factors; Immunosuppressive Agents; Motor Neuron Disease; Muscle Strength; Mycophenolic Acid; Polyneuropathies; Randomized Controlled Trials as Topic

Multifocal motor neuropathy is characterised by progressive, predominantly distal, asymmetrical limb weakness and usually multiple partial motor nerve conduction blocks. Intravenous immunoglobulin is beneficial but the role of immunosuppressive agents is uncertain. This is an update of a review first published in 2002 and previously updated in 2003, 2005 and 2008.. To provide the best available evidence from randomised controlled trials on the role of immunosuppressive agents for the treatment of multifocal motor neuropathy.. We searched the Cochrane Neuromuscular Disease Group Specialized Register (4 October 2011), the Cochrane Central Register of Controlled Trials (CENTRAL) (2011, Issue 3 in The Cochrane Library), MEDLINE (January 1966 to September 2011), EMBASE (January 1980 to September 2011), and LILACS (January 1982 to September 2011) for trials of multifocal motor neuropathy.. We planned to include randomised and quasi-randomised controlled trials. We considered prospective and retrospective case series and case reports in the Discussion.. Two review authors searched the titles and abstracts of the articles identified and extracted the data independently.. Only one randomised controlled trial of an immunosuppressive or immunomodulatory agent has been performed in multifocal motor neuropathy. This study randomised 28 participants and showed that mycophenolate mofetil, when used with intravenous immunoglobulin, did not significantly improve strength, function or reduce the need for intravenous immunoglobulin. No serious adverse events were observed. The study was deemed at low risk of bias. We summarised the results of retrospective and prospective case series in the discussion.. According to moderate quality evidence, mycophenolate mofetil did not produce significant benefit in terms of reducing need for intravenous immunoglobulin or improving muscle strength. Trials of other immunosuppressants should be undertaken.

Topics: Drug Therapy, Combination; Humans; Immunoglobulins, Intravenous; Immunologic Factors; Immunosuppressive Agents; Motor Neuron Disease; Mycophenolic Acid; Polyneuropathies; Randomized Controlled Trials as Topic

Multifocal motor neuropathy is characterised by progressive, predominantly distal, asymmetrical limb weakness and usually multiple partial motor nerve conduction blocks. Intravenous immunoglobulin is beneficial but the role of immunosuppressive agents is uncertain.. To provide the best available evidence from randomised controlled trials on the role of immunosuppressive agents for the treatment of multifocal motor neuropathy.. For this update, we searched the Cochrane Neuromuscular Disease Group Trials Register (October 8 2008), the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library 2008, Issue 4), MEDLINE (from January 1966 to October 8 2008), and EMBASE (from January 1980 to October 8 2008), for trials of multifocal motor neuropathy.. Randomised and quasi-randomised controlled trials were to be included and one was found. Prospective and retrospective case series and case reports were considered in the Discussion.. Two review authors searched the titles and abstracts of the articles identified and extracted the data independently.. In this update, we found the first randomised controlled trial of multifocal motor neuropathy. This study, which randomised 28 patients, showed that mycophenolate mofetil did not significantly improve strength or function or reduce the need for intravenous immunoglobulin. We summarised the results of retrospective and prospective case series in the discussion.. In the only randomised placebo-controlled trial of any immunosuppressive agent, mycophenolate mofetil did not produce significant benefit. Trials of other immunosuppressants should be undertaken.

Topics: Humans; Immunosuppressive Agents; Motor Neuron Disease; Mycophenolic Acid; Polyneuropathies; Randomized Controlled Trials as Topic

Multifocal motor neuropathy (MMN) is an immune-mediated disorder characterized by slowly progressive asymmetrical limb weakness. Treatment with immunoglobulins (IVIg) leads to improvement of muscle strength. Anecdotal evidence suggests that immunosuppressive drugs as adjunctive therapy may be beneficial. Mycophenolate mofetil (MMF) is a potent and safe immunosuppressant. Safety and efficacy of MMF as adjunctive therapy for MMN patients receiving IVIg maintenance treatment were evaluated in a randomized controlled trial. MMN patients responding to IVIg treatment were eligible for randomization. Muscle strength and functional status were assessed at monthly intervals for 1 year. Three months after the start of MMF or placebo treatment, IVIg doses were reduced stepwise, until a deterioration of functioning or decline in muscle strength could be observed. An IVIg dose reduction of 50% during adjunctive treatment was defined as a primary endpoint. Secondary outcome measures were improvement in muscle strength and functional status after 3 months and reduction of anti GM1-IgM titres after 12 months of MMF treatment. Twenty-eight patients were randomized. One patient allocated to MMF reached the primary endpoint of 50% IVIg dose reduction. After 12 months IVIg reduction did not differ significantly between the two treatment groups. Patients did not experience drug toxicity and none of the patients showed significant disease progression after 12 months. Muscle strength and functional scores after 3 months and anti GM1-IgM titres after 12 months did not change. Adjunctive treatment of MMN patients with MMF at a dose of 1 g twice daily is safe but does not alter disease course or allow significant reduction of IVIg doses.

Topics: Adult; Aged; Autoantibodies; Autoimmune Diseases; Chemotherapy, Adjuvant; Disease Progression; Double-Blind Method; Drug Administration Schedule; Female; G(M1) Ganglioside; Humans; Immunoglobulin M; Immunoglobulins, Intravenous; Immunosuppressive Agents; Male; Middle Aged; Motor Neuron Disease; Muscle Strength; Muscle, Skeletal; Mycophenolic Acid; Treatment Outcome

There has been an increasing interest in the use of mycophenolate as an immunomodulatory drug in neuromuscular diseases. We report five consecutive patients with treatment-resistant chronic inflammatory demyelinating polyradiculoneuropathy or multifocal motor neuropathy who were treated with mycophenolate. None showed clinically significant benefit. The use of mycophenolate did not result in the reduction in the dose of corticosteroid or other immunosuppressive agents in any patient. Side-effects, although not serious, were troubling enough for two patients to stop mycophenolate.

Topics: Adjuvants, Immunologic; Adolescent; Adult; Drug Resistance; Humans; Middle Aged; Motor Neuron Disease; Mycophenolic Acid; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Retreatment; Treatment Failure