Randomized Double-Blind Placebo-Controlled Trial of the Corticosteroid-Sparing Effects of Immunoglobulin in Myasthenia Gravis.

Background And Objectives: Myasthenia gravis (MG) is an autoimmune disease characterized by dysfunction at the neuromuscular junction. Treatment frequently includes corticosteroids (CSs) and IV immunoglobulin (IVIG). This study was conducted to determine whether immune globulin (human), 10% caprylate/chromatography purified (IGIV-C) could facilitate CS dose reduction in CS-dependent patients with MG.

Comparison of fixed cell-based assay to radioimmunoprecipitation assay for acetylcholine receptor antibody detection in myasthenia gravis.

Objective: To compare specificity and sensitivity of a commercially available fixed cell-based assay (F-CBA) to radioimmunoprecipitation assay (RIPA) for acetylcholine receptor antibody (anti-AChR) detection in myasthenia gravis (MG).

Methods: In this retrospective diagnostic cohort study we reviewed the clinical information of suspected MG patients evaluated at the London Health Sciences Centre MG clinic who had anti-AChR RIPA and then F-CBA performed, in order to classify them as MG or non-MG. Classification of each patient as anti-AChR F-CBA-negative/positive, RIPA-negative/positive, and MG/non-MG permitted specificity and sensitivity calculations for each assay.

International Consensus Guidance for Management of Myasthenia Gravis: 2020 Update.

Objective: To update the 2016 formal consensus-based guidance for the management of myasthenia gravis (MG) based on the latest evidence in the literature.

Methods: In October 2013, the Myasthenia Gravis Foundation of America appointed a Task Force to develop treatment guidance for MG, and a panel of 15 international experts was convened. The RAND/UCLA appropriateness method was used to develop consensus recommendations pertaining to 7 treatment topics.

Classically Described, Paradoxically Underrecognized: A Case of Proximal Weakness, Hyporeflexia/Areflexia, and Dysautonomia.

We present a case of chronic, progressive proximal weakness with dysautonomia and hyporeflexia/areflexia ultimately diagnosed with Lambert-Eaton myasthenic syndrome. An approach to neuroanatomical localization is discussed leading to the appropriate selection of electrodiagnostic studies. The electrophysiologic triad of Lambert-Eaton myasthenic syndrome is demonstrated with diffusely reduced motor amplitudes, decrement with low-frequency repetitive nerve stimulation, and increment of motor amplitudes after maximum voluntary contraction.

Epidemiological evidence for a hereditary contribution to myasthenia gravis: a retrospective cohort study of patients from North America.

Objectives: To approximate the rate of familial myasthenia gravis and the coexistence of other autoimmune disorders in the patients and their families.

Design: Retrospective cohort study.

Setting: Clinics across North America.

A Phase 3 Multicenter, Prospective, Open-Label Efficacy and Safety Study of Immune Globulin (Human) 10% Caprylate/Chromatography Purified in Patients with Myasthenia Gravis Exacerbations.

Background: Myasthenia gravis (MG) is an autoimmune disorder affecting neuromuscular transmission. Exacerbations may involve increasing bulbar weakness and/or sudden respiratory failure, both of which can be critically disabling. Management of MG exacerbations includes plasma exchange and intravenous immunoglobulin (IVIG); they are equally effective, but patients experience fewer side effects with IVIG.

The Positive Predictive Value of Onconeural Antibody Testing: A Retrospective Review.

Paraneoplastic syndromes (PNS) are immune-mediated neurologic diseases that occur as an indirect effect of malignancy, and can be challenging to diagnose. Onconeural antibodies have a greater than 95% association with cancer, and their presence in a patient with neurologic symptoms is reportedly highly indicative of PNS. However, we performed a single-centre retrospective review to determine the positive predictive value of onconeural antibody testing, and found it to be concerningly low (39%).

Effect of Treating Acute Optic Neuritis With Bioequivalent Oral vs Intravenous Corticosteroids: A Randomized Clinical Trial.

Importance: Intravenous (IV) administration of corticosteroids is the standard of care in the treatment of acute optic neuritis. However, it is uncertain whether a bioequivalent dose of corticosteroid administered orally, which may be more cost-efficient and convenient for patients, is as effective as IV administration in the treatment of acute optic neuritis.

Objective: To determine whether recovery of vision following treatment of acute optic neuritis with a high-dose IV corticosteroid is superior to that with a bioequivalent dose of an oral corticosteroid.

Myasthenia Gravis and Lambert-Eaton Myasthenic Syndrome.

Purpose Of Review: This article discusses the pathogenesis, diagnosis, and management of autoimmune myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS).

Recent Findings: Recognition of new antigenic targets and improved diagnostic methods promise to improve the diagnosis of MG, although the clinical phenotypes associated with newer antibodies have not yet been defined. Future therapies might specifically target the aberrant immune response.

A Review of Azathioprine-Associated Hepatotoxicity and Myelosuppression in Myasthenia Gravis.

Objectives: Myasthenia gravis (MG) is an autoimmune disorder in which antibodies interfere with neuromuscular transmission. Azathioprine (AZA) is an immunosuppressant frequently used for treatment of various autoimmune conditions, including MG. The literature suggests that the rates of AZA-associated hepatotoxicity and myelosuppression in MG are highly variable.

Hope, Coping, and Quality of Life in Adults with Myasthenia Gravis.

Unlabelled: Myasthenia gravis significantly impacts quality of life. However, the relationship between hope, coping, and quality of life (QOL)in myasthenia patients has not been studied (Kulkantrakorn & Jarungkiatkul, 2009; Raggi et al., 2010).

International consensus guidance for management of myasthenia gravis: Executive summary.

Objective: To develop formal consensus-based guidance for the management of myasthenia gravis (MG).

Methods: In October 2013, the Myasthenia Gravis Foundation of America appointed a Task Force to develop treatment guidance for MG, and a panel of 15 international experts was convened. The RAND/UCLA appropriateness methodology was used to develop consensus guidance statements.

Electrodiagnostic Testing and Treatment for Carpal Tunnel Syndrome in Canada.

Objectives: 1) Assess which electrodiagnostic studies Canadian clinicians use to aid in the diagnosis of carpal tunnel syndrome (CTS). 2) Assess whether Canadian clinicians follow the American Association of Neuromuscular & Electrodiagnostic Medicine/American Academy of Neurology/American Academy of Physical Medicine and Rehabilitation Practice Parameter for Electrodiagnostic Studies in CTS. 3) Assess how Canadian clinicians manage CTS once a diagnosis has been established.

A genome-wide association study of myasthenia gravis.

Importance: Myasthenia gravis is a chronic, autoimmune, neuromuscular disease characterized by fluctuating weakness of voluntary muscle groups. Although genetic factors are known to play a role in this neuroimmunological condition, the genetic etiology underlying myasthenia gravis is not well understood.

Objective: To identify genetic variants that alter susceptibility to myasthenia gravis, we performed a genome-wide association study.

Development of Myasthenia Gravis in a Patient with Chronic Myeloid Leukemia during Treatment with Nilotinib.

We report on a patient diagnosed with chronic myeloid leukemia (CML) who developed myasthenia gravis while on treatment with nilotinib. Autoimmune disease, including the development of myasthenia gravis, has been described in association with CML as well as the use of tyrosine kinase inhibitors. Second generation tyrosine kinase inhibitors are highly effective in the treatment of CML, although can result in adverse effects related to off-target kinase inhibition, and longer term reporting of adverse effects is required.

A comparison of EMG and muscle biopsy in ICU weakness.

Background: Patients can become weak in ICU from various etiologies and mechanisms. Establishing the diagnosis is invaluable for prognostic determination and specific management. We evaluated the relative contributions of clinical, laboratory, electomyographic studies (EMG), and percutaneous muscle biopsy (MB) in determining the cause of muscular weakness that developed in a series of patients while in ICU.

Clinical and electrophysiological parameters distinguishing acute-onset chronic inflammatory demyelinating polyneuropathy from acute inflammatory demyelinating polyneuropathy.

Up to 16% of chronic inflammatory demyelinating polyneuropathy (CIDP) patients may present acutely. We performed a retrospective chart review on 30 acute inflammatory demyelinating polyneuropathy (AIDP) and 15 acute-onset CIDP (A-CIDP) patients looking for any clinical or electrophysiological parameters that might differentiate AIDP from acutely presenting CIDP. A-CIDP patients were significantly more likely to have prominent sensory signs.