Comparative effectiveness of azathioprine and mycophenolate mofetil for myasthenia gravis (PROMISE-MG): a prospective cohort study.

Background: Myasthenia gravis is an autoimmune disorder of the neuromuscular junction. Treatment typically includes symptomatic oral cholinesterase inhibitors, immunosuppression, and immunomodulation. In addition to corticosteroids, azathioprine and mycophenolate mofetil are the most frequently used immunosuppressants in North America.

Pregnancy in Seronegative Myasthenia Gravis: A Single-Center Case Series.

Introduction: The course of double-seronegative myasthenia gravis (DSNMG) during and after pregnancy has not been well described.

Objective: To assess the course of DSNMG during pregnancy and within 6 months postpartum.

Methods: A retrospective cohort study of women with DSNMG seen in the Duke Myasthenia gravis (MG) Clinic after 2003.

Pregnancy in MuSK-positive myasthenia gravis: A single-center case series.

Introduction/aims: Myasthenia gravis (MG) with muscle-specific tyrosine kinase (MuSK) antibodies (MMG) is predominantly seen in women of childbearing age. Our objective in this study was to describe the course of MMG during pregnancy and within 6 months postpartum, and to document any effect on fetal health.

Methods: A retrospective review was performed of medical records of patients with MMG seen in the Duke Myasthenia Gravis Clinic from 2003 to 2022.

The Duke Myasthenia Gravis Clinic Registry: II. Analysis of outcomes.

Introduction/aims: The Duke Myasthenia Gravis (MG) Clinic Registry contains comprehensive physician-derived data on patients with MG seen in the Duke MG Clinic since 1980. The aim of this study was to report outcomes in patients seen in the clinic and treated according to the International Consensus Guidance statements.

Methods: This is a retrospective cohort study of patients initially seen after 2000 and followed for at least 2 years in the clinic.

Single fiber electromyography and measuring jitter with concentric needle electrodes.

This monograph contains descriptions of the single fiber electromyography (SFEMG) method and of the more recently implemented method of recording jitter with concentric needle electrodes (CNEs). SFEMG records action potentials from single muscle fibers (SFAPs), which permits measuring fiber density (FD), a sensitive measure of reinnervation, and jitter, a sensitive measure of abnormal neuromuscular transmission (NMT). With voluntary activation, jitter is measured between two SFAPs with acceptable amplitude and rise time.

Identifying a patient-centered outcome measure for a comparative effectiveness treatment trial in myasthenia gravis.

Introduction/aims: Data regarding the comparative effectiveness of myasthenia gravis (MG) treatments is not available. We used patient input to identify a patient-centered outcome measure (PCOM) for PROMISE-MG, a comparative effectiveness trial of MG treatments.

Methods: First, a questionnaire survey was administered to 58 people with MG at the patient meeting of the Myasthenia Gravis Foundation of America (MGFA), evaluating the impact of MG-related symptoms and MG treatments on patients' lives.

If Thymectomy Reduces the Risk of Progression of Ocular to Generalized Myasthenia Gravis, Who Should Receive it?

In this issue of Neurotherapeutics, Li et.al. report a large retrospective study of the beneficial effects of thymectomy on the progression of ocular myasthenia gravis (OMG) to generalized MG (GMG) (Huanhuan et al.

The Duke myasthenia gravis clinic registry: I. Description and demographics.

Introduction: The Duke Myasthenia Gravis (MG) Clinic Registry is a disease-specific database containing physician-derived data from patients seen in the Duke MG Clinic since 1980.

Methods: Data from 1060 MG patients initially seen between 1980 and 2008 were reviewed.

Results: Fifty-four percent were male.

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.

Single-institutional reference values for concentric needle jitter analysis using the extrapolated reference values procedure: Comparison to published reference values.

Background: The extrapolated reference values procedure (E-Ref) was used to compare data from a single institution with the recently published reference value (RV) for concentric electrode jitter.

Methods: Data from voluntarily activated concentric needle jitter studies in the frontalis muscle were obtained using retrospective chart review. All measured signals were reviewed for acceptable quality.

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.

AANEM - IFCN glossary of terms in neuromuscular electrodiagnostic medicine and ultrasound.

Modern neuromuscular electrodiagnosis (EDX) and neuromuscular ultrasound (NMUS) require a universal language for effective communication in clinical practice and research and, in particular, for teaching young colleagues. Therefore, the AANEM and the IFCN have decided to publish a joint glossary as they feel the need for an updated terminology to support educational activities in neuromuscular EDX and NMUS in all parts of the world. In addition NMUS has been rapidly progressing over the last years and is now widely used in the diagnosis of disorders of nerve and muscle in conjunction with EDX.

AANEM - IFCN Glossary of Terms in Neuromuscular Electrodiagnostic Medicine and Ultrasound.

Modern neuromuscular electrodiagnosis (EDX) and neuromuscular ultrasound (NMUS) require a universal language for effective communication in clinical practice and research and, in particular, for teaching young colleagues. Therefore, the AANEM and the IFCN have decided to publish a joint glossary as they feel the need for an updated terminology to support educational activities in neuromuscular EDX and NMUS in all parts of the world. In addition NMUS has been rapidly progressing over the last years and is now widely used in the diagnosis of disorders of nerve and muscle in conjunction with EDX.

Validation of the triple timed up-and-go test in Lambert-Eaton myasthenia.

Introduction: There are no validated, practical, and quantitative measures of disease severity in Lambert-Eaton myasthenia (LEM).

Methods: Data from the Effectiveness of 3,4-Diaminopyridine in Lambert-Eaton Myasthenic Syndrome (DAPPER) trial were analyzed to assess triple timed up-and-go (3TUG) reproducibility and relationships between 3TUG times and other measures of LEM severity.

Results: The coverage probability technique showed ≥0.

Standards for quantification of EMG and neurography.

This document is an update and extension of ICCN Standards published in 1999. It is the consensus of experts on the current status of EMG and Neurography methods. A panel of authors from different countries with different approach to routines in neurophysiological methods was chosen based on their particular interest and previous publications.

Guidelines for single fiber EMG.

This document is the consensus of international experts on the current status of Single Fiber EMG (SFEMG) and the measurement of neuromuscular jitter with concentric needle electrodes (CNE - CN-jitter). The panel of authors was chosen based on their particular interests and previous publications within a specific area of SFEMG or CN-jitter. Each member of the panel was asked to submit a section on their particular area of interest and these submissions were circulated among the panel members for edits and comments.

Research Gaps in Wilderness Medicine.

Introduction: Wilderness medicine involves the treatment of individuals in remote, austere environments. Given the high potential for injuries as well as the unique treatment modalities required in wilderness medicine, evidence-based clinical practice guidelines are necessary to provide optimal care. In this study, we identify evidence gaps from low-quality recommendations in wilderness medicine clinical practice guidelines and identify new/ongoing research addressing them.