Evidence-based guideline: Intravenous immunoglobulin in the treatment of neuromuscular disorders: Report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology
ABSTRACT To assess the evidence for the efficacy of IV immunoglobulin (IVIg) to treat neuromuscular disorders.
The MEDLINE, Web of Science, and EMBASE databases were searched (1966-2009). Selected articles were rated according to the American Academy of Neurology's therapeutic classification of evidence scheme; recommendations were based on the evidence level.
IVIg is as efficacious as plasmapheresis and should be offered for treating Guillain-Barré syndrome (GBS) in adults (Level A). IVIg is effective and should be offered in the long-term treatment of chronic inflammatory demyelinating polyneuropathy (Level A). IVIg is probably effective and should be considered for treating moderate to severe myasthenia gravis and multifocal motor neuropathy (Level B). IVIg is possibly effective and may be considered for treating nonresponsive dermatomyositis in adults and Lambert-Eaton myasthenic syndrome (Level C). Evidence is insufficient to support or refute use of IVIg in the treatment of immunoglobulin M paraprotein-associated neuropathy, inclusion body myositis, polymyositis, diabetic radiculoplexoneuropathy, or Miller Fisher syndrome, or in the routine treatment of postpolio syndrome or in children with GBS (Level U). IVIg combined with plasmapheresis should not be considered for treating GBS (Level B). More data are needed regarding IVIg efficacy as compared with other treatments/treatment combinations. Most studies concluded IVIg-related serious adverse effects were rare. Given the variable nature of these diseases, individualized treatments depending on patient need and physician judgment are important.
- SourceAvailable from: Raffi Tachdjian
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- "In addition, a total of 3 patients had median pain scores that were lowered by 50%. Despite several limitations to the study, including the trial size, the authors showed that there was a significant reduction in pain associated with CRPS as a result of IVIG treatment . "
ABSTRACT: Introduction Common variable immunodeficiency (CVID) represents a large heterogeneous group of antibody-deficiency syndromes associated with a wide range of clinical features and a lack of defined causes in the realm of primary immunodeficiencies. Here, we present a case of CVID in a 62-year-old white male patient with a history of longstanding complex regional pain syndrome (CRPS). Case Presentation His medical history included multiple sinus infections per year and several pneumonias requiring antibiotics. He has had various back surgeries, including a laminectomy at the L4 level 1 year prior to his diagnosis. Thereafter, he underwent four sympathetic nerve blocks with minimal pain relief. Blood chemistries showed a normal white blood cell count with a normal differential, but increased erythrocyte sedimentation rate and C-reactive protein levels. Total Ig (Immunoglobulin)G was 611 mg/dL (normal 700–1,600), IgG1 was 425 mg/dL (341–894), IgG2 was 114 mg/dL (171–632), IgG3 was 14.4 mg/dL (18.4–106), and IgG4 was 7.4 mg/dL (2.4–121). IgA was 47 mg/dL (normal 70–400), IgM was 131 mg/dL (40–230), and IgE was 4.5 kU/L (<4.0). He only had 10 of 23 pneumococcal titers in the protective range post-vaccination. Upon treatment of the CVID with intravenous immunoglobulin, the patient’s pain levels were significantly decreased and have been maintained for more than 2 years. Conclusion Therefore, immunoglobulin therapy appears to have been beneficial in the treatment of the patient’s symptoms of CRPS, including pain. Additional studies investigating the mechanism by which immunoglobulin therapy may reduce the inflammation and pain of CRPS are needed.12/2013; 2(2). DOI:10.1007/s40122-013-0020-0
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- "The effect of IVIG is seen in less than a week and the benefit can last for few weeks. However limited data for the effectiveness of IVIG for myasthenia gravis without crisis are available [6,7]. "
ABSTRACT: Patient: Female, 70 Final Diagnosis: SA block induced by pyridostigmine Symptoms: Asymptomatic Medication: Pyridostigmine Clinical Procedure: Pacemaker insertion Specialty: Electrophysiology. Unusual clinical course. Myasthenia gravis requires a long-term treatment with a parasympathomimetic agent, which may result in bradycardia and asystole. Pharmacologic treatment with a reversible inhibitor of inosine monophosphate dehydrogenase (IMPDH) and Methylprednisolone is seen to improve the muscular symptoms but may reinforce potential bradyarrhythmias. This potential side effect can be treated with the levo isomer of atropine, Hyoscyamine, or Glycopyrollate in an intact conduction system. A 70-year old Caucasian female patient with a family history of myasthenia gravis presented with mild weakness of the bilateral facial muscles, moderate dysarthria, dysphagia, diplopia predominantly on the right side and difficulty tracking ocular movements bilaterally. The treatment with pharmacological agents was initiated. Subsequently she developed asymptomatic bradycardia and SA-block. An improvement on Hyoscyamine failed to appear. A dual chamber pacemaker was placed. In symptomatic or asymptomatic bradycardia with significant high grade SA-block in patients with myasthenia gravis the insertion of a permanent pacemaker can be the definitive solution.American Journal of Case Reports 09/2013; 14:359-61. DOI:10.12659/AJCR.889484
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- "Expert consensus supports high-dose oral prednisone as first-line therapy; however, as many as 30%–40% of patients may fail to respond, and up to 40% or more experience major adverse events with long-term steroid use.2 Steroid-sparing or alternative immunosuppressive therapies, including methotrexate, azathioprine, cyclosporine, and mycophenolate mofetil, have a long onset of action and risks, including toxicity to the kidneys, liver, and bone marrow.1 Intravenous immunoglobulin is considered a second-line therapy for dermatomyositis, but not for polymyositis.3 However, intravenous immunoglobulin does not have a US Food and Drug Administration (FDA) indication for myositis, and is very expensive with a risk of acute renal failure.1,4 "
ABSTRACT: Effective and tolerable treatment options for patients with dermatomyositis and polymyositis are limited. This retrospective case review describes treatment with adrenocorticotropic hormone (ACTH) gel in five patients who experienced a disease exacerbation and either failed or were unable to tolerate the side effects of previous therapy with steroids, intravenous immunoglobulins, and steroid-sparing drugs. Patients received ACTH gel subcutaneous injections of 80 U (1 mL) twice weekly (four patients) or once weekly (one patient) over the course of 12 weeks for short-term treatment of symptom exacerbations. Manual muscle testing using the Medical Research Council scale was assessed at baseline and at 3 months. Improvement was seen in all patients, including improved muscle strength, decreased pain, and resolution of skin involvement. All patients tolerated the treatment well, and no significant side effects occurred. The treatment of dermatomyositis and polymyositis is an approved use for ACTH gel, and these anecdotal reports would suggest consideration of ACTH gel as a therapeutic option. Further investigation is warranted.Drug Design, Development and Therapy 06/2012; 6:133-9. DOI:10.2147/DDDT.S33110 · 3.03 Impact Factor