ConferenceNeuromuscular disorders : NMD · November 2024
The 275th ENMC workshop on the diagnosis and management of seronegative myasthenia gravis (SNMG) was held on February 9-11, 2024. The participants included experts in the field of adult and pediatric MG together with patient representatives. This workshop ...
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Journal ArticleLancet Neurol · March 2024
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 mycoph ...
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Journal ArticleJ Clin Neuromuscul Dis · December 1, 2023
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 stud ...
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ConferenceMuscle Nerve · July 2023
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 po ...
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ConferenceMuscle Nerve · April 2023
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 trea ...
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Journal ArticleMuscle Nerve · August 2022
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 (SF ...
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Journal ArticleMuscle Nerve · June 2, 2022
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 (CNE). SFEMG records action potentials from single muscle fibers (SFA ...
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Journal ArticleMuscle Nerve · January 2022
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 treat ...
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Journal ArticleNeurotherapeutics · October 2021
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. in Neurotherapeutics XX, 2021). This pape ...
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Journal ArticleMuscle Nerve · March 2021
BACKGROUND: Patients with myasthenia gravis (MG) may be particularly vulnerable during the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic due to risk of worsening disease during infection, potential adverse impacts of coronavirus dis ...
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Journal ArticleMuscle Nerve · February 2021
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 wer ...
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Journal ArticleNeurology · January 19, 2021
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 devel ...
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Journal ArticleMuscle Nerve · January 2021
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 ...
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Journal ArticleBMJ Open · September 18, 2020
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. PARTICIPANTS: The study inclu ...
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Journal ArticleMuscle Nerve · July 2020
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 ...
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Journal ArticleClin Neurophysiol · July 2020
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 ...
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Journal ArticleClin Neurophysiol · September 2019
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 neurophysi ...
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Journal ArticleMuscle Nerve · September 2019
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 ...
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Journal ArticleClin Neurophysiol · August 2019
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 partic ...
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Journal ArticleNeurology · April 17, 2018
OBJECTIVE: To investigate the efficacy and safety of belimumab, a fully human immunoglobulin G1λ monoclonal antibody against B-lymphocyte stimulator, in participants with generalized myasthenia gravis (MG) who remained symptomatic despite standard of care ...
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Journal ArticleMuscle Nerve · April 2018
INTRODUCTION: 3,4-diaminopyridine has been used to treat Lambert-Eaton myasthenia (LEM) for 30 years despite the lack of conclusive evidence of efficacy. METHODS: We conducted a randomized double-blind placebo-controlled withdrawal study in patients with L ...
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Journal ArticleAnn N Y Acad Sci · February 2018
Myasthenia gravis (MG) presents many challenges for establishing treatment efficacy through clinical trials. Among these are the rarity and heterogeneity of the disease, spontaneous fluctuations, prolonged latency to effect for many immunosuppressive drugs ...
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Journal ArticleMuscle Nerve · January 2018
INTRODUCTION: Reference values (RVs) are required to separate normal from abnormal values obtained in electrodiagnostic (EDx) testing. However, it is frequently impractical to perform studies on control subjects to obtain RVs. The Extrapolated Reference Va ...
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Journal ArticleMuscle Nerve · January 2018
INTRODUCTION: We report the reliability of a new measure, the triple-timed up-and-go (3TUG) test, for assessing clinical function in patients with Lambert-Eaton myasthenia (LEM). METHODS: Intrarater reproducibility and interrater agreement of the 3TUG test ...
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Journal ArticleAnn N Y Acad Sci · January 2018
A task force of the Myasthenia Gravis Foundation of America recently published a formal consensus statement intended to be a treatment guide for clinicians caring for myasthenia gravis (MG) patients worldwide. Its development was stimulated by the fact tha ...
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Journal ArticleJ Neuromuscul Dis · 2018
Comparing results from jitter studies performed with voluntary and electrical activation is difficult to perform quantitatively, particularly in complex signals as seen in reinnervation. High jitter values in individual spikes in these multispike signals c ...
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Journal ArticleClin Neurophysiol · November 2017
The safety factor of neuromuscular transmission can be assessed by measuring the neuromuscular jitter, which reflects the time variability of processes in the motor end-plate. Jitter is increased in any condition with disturbed end-plate function, such as ...
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Journal ArticleMuscle Nerve · July 2017
INTRODUCTION: The objective of this study was to determine if single-fiber electromyography (SFEMG) jitter accurately reflects change in severity in myasthenia gravis (MG). METHODS: We reviewed jitter and outcome data from all MG patients in our clinic who ...
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Journal ArticleMuscle Nerve · December 2016
INTRODUCTION: The MG-QOL15 is a validated, health-related quality of life (HRQOL) measure for myasthenia gravis (MG). Widespread use of the scale gave us the opportunity to further analyze its clinimetric properties. METHODS: We first performed Rasch analy ...
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Journal ArticleMuscle Nerve · December 2016
INTRODUCTION AND METHODS: Information from myasthenia gravis (MG) patients treated and evaluated for at least 2 years between 1980 and 2014 was reviewed to assess the effect of demographics, antibody status and titer, thymus histology, and clinical severit ...
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Journal ArticleNeurology · July 26, 2016
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 interna ...
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Journal ArticleMuscle Nerve · March 2016
INTRODUCTION: The aim of this study was to create reference values for jitter measured with concentric needle electrodes. METHODS: Operators worldwide contributed recordings from orbicularis oculi (OO), frontalis (FR), and extensor digitorum (ED) muscles i ...
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Journal ArticleMuscle Nerve · March 2016
INTRODUCTION: In this study we evaluated the safety, tolerability, and efficacy of prednisone in patients with ocular myasthenia gravis (OMG) concurrently treated with pyridostigmine. METHODS: This investigation was a randomized, double-blind, placebo-cont ...
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Journal ArticleMuscle Nerve · August 2015
INTRODUCTION: Mycophenolate mofetil (MMF) is frequently used to treat myasthenia gravis, but there is little information to guide clinicians on the safety of reducing the dose in well-controlled patients. METHODS: This retrospective chart review at 3 insti ...
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Journal ArticleJAMA Neurol · April 2015
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 und ...
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Journal ArticleNeurol Neuroimmunol Neuroinflamm · April 2015
OBJECTIVE: To characterize B-cell subsets in patients with muscle-specific tyrosine kinase (MuSK) myasthenia gravis (MG). METHODS: In accordance with Human Immunology Project Consortium guidelines, we performed polychromatic flow cytometry and ELISA assays ...
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Journal ArticleNeurotherapeutics · April 2015
Tirasemtiv is a fast skeletal troponin activator that sensitizes the sarcomere to calcium and increases muscle force following subtetanic nerve input. In an animal model of myasthenia gravis (MG), single oral doses of tirasemtiv improved muscle force and r ...
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Journal ArticleContinuum (Minneap Minn) · October 2014
PURPOSE OF REVIEW: This article reviews the clinical presentations, diagnostic findings, and treatment options for autoimmune myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome. RECENT FINDINGS: Immunologic research is unraveling the immunopathol ...
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Journal ArticleJ Autoimmun · August 2014
Muscle specific tyrosine kinase myasthenia gravis (MuSK MG) is a form of autoimmune MG that predominantly affects women and has unique clinical features, including prominent bulbar weakness, muscle atrophy, and excellent response to therapeutic plasma exch ...
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ConferenceMuscle Nerve · July 2014
INTRODUCTION: We determined health plan paid costs and healthcare resource usage of patients with chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: CIDP patients from 9 U.S. commercial health plans with claims in 2011 were identified from ...
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Journal ArticleClin Neurophysiol · June 2014
OBJECTIVE: We reviewed our experience to determine if the decremental pattern during low frequency repetitive nerve stimulation (LF-RNS) distinguishes between the Lambert-Eaton myasthenic syndrome (LEMS) and myasthenia gravis (MG). METHODS: LF-RNS studies ...
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Journal ArticleMuscle Nerve · April 2014
INTRODUCTION: The objective of this study is to determine if change in acetylcholine receptor antibody (AChR-ab) levels reflects change in clinical severity in patients with myasthenia gravis (MG). METHODS: We reviewed results from a prospective trial in M ...
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Chapter · January 1, 2014
Single-fiber electromyography (SFEMG) is a selective electromyography (EMG) recording technique that uniquely identifies action potentials (APs) from individual muscle fibers, thus allowing measurement of fiber density (FD) and neuromuscular jitter. FD qua ...
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Chapter · January 1, 2014
The Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune condition, in which weakness is caused by blocked release of the neurotransmitter acetylcholine from the motor nerve terminal. This condition was first described in patients with lung cancer ...
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Journal ArticleMuscle Nerve · January 2014
INTRODUCTION: Acetylcholine receptor (AChR) and striated muscle antibodies (StrAbs) are found frequently in myasthenia gravis (MG) patients with thymoma. In this study we aimed to determine the positive predictive value (PPV) and negative predictive value ...
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Journal ArticleMuscle Nerve · February 2013
INTRODUCTION: Venous access for therapeutic plasma exchange (TPE) in myasthenia gravis (MG) can be achieved by central venous catheters (CVC) or peripheral veins (PV), and the preferred method varies among providers. We evaluated our institutional experien ...
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Journal ArticleAnn N Y Acad Sci · December 2012
Clinical registries may facilitate research on myasthenia gravis (MG) in several ways: as a source of demographic, clinical, biological, and immunological data on large numbers of patients with this rare disease; as a source of referrals for clinical trial ...
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Journal ArticleAnn N Y Acad Sci · December 2012
Efficacy of Prednisone in the Treatment of Ocular Myasthenia (EPITOME) is a multicenter, randomized, double blind, placebo-controlled trial that is being conducted under the auspices of the Muscle Study Group. EPITOME is the first randomized control trial ...
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Journal ArticleExpert Rev Clin Immunol · July 2012
Myasthenia gravis is an autoimmune disorder of the neuromuscular junction. A number of molecules, including ion channels and other proteins at the neuromuscular junction, may be targeted by autoantibodies leading to abnormal neuromuscular transmission. In ...
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Journal ArticleMuscle Nerve · June 2012
The recommendations for clinical research standards published in 2000 by a task force of the Medical Scientific Advisory Board (MSAB) of the Myasthenia Gravis Foundation of America (MGFA) were largely successful in introducing greater uniformity in the rec ...
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Journal ArticleMuscle Nerve · June 2012
INTRODUCTION: The MG Composite (MGC) is a validated outcome measure of the clinical manifestations of myasthenia gravis. METHODS: We performed Rasch analyses of the MGC to investigate additional properties, including its unidimensionality and the appropria ...
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Journal ArticleMuscle Nerve · March 2012
INTRODUCTION: In this study we estimated the costs paid by U.S. health plans for treating myasthenia gravis (MG) in 2009 and determined the major cost drivers. METHODS: One hundred thirteen MG patients were matched by propensity scores with 339 non-MG pati ...
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Journal ArticleContemp Clin Trials · March 2012
INTRODUCTION: Registries are becoming increasingly important for rare diseases as experimental therapies develop. This report describes the methodology behind the National Registry of Myotonic Dystrophy (DM) and Facioscapulohumeral Muscular Dystrophy (FSHD ...
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Journal ArticleMuscle Nerve · December 2011
INTRODUCTION: Little is known about the costs of managing rare diseases, and comprehensive healthcare costs have not been reported for myasthenia gravis (MG). We evaluated the direct costs and healthcare resource utilization in insured MG patients. METHODS ...
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Journal Article · September 6, 2011
Diseases of the neuromuscular junction are rare, and few physicians have seen enough patients with them to easily recognize all the ways in which they can present. Yet, the most important step in making the diagnosis is to think of these conditions. This c ...
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Journal ArticleMuscle Nerve · July 2011
INTRODUCTION: Myasthenia gravis (MG) patients with autoantibodies to muscle-specific tyrosine kinase (MuSK) represent a distinct subset of those with this disease. Treatment and outcomes data in these patients are limited and conflicting. METHODS: We revie ...
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Journal ArticleMuscle Nerve · January 2011
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The MG-QOL15 is helpful in informing the clinician about the patient's perception of the extent of and dissatisfaction with myasthenia gravis (MG)-related dysfunction. The aims of this study were to determine the usefulness of the MG-QOL15 for following in ...
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Journal ArticleCurr Opin Neurol · October 2010
PURPOSE OF REVIEW: Important concepts regarding the pathogenesis, clinical features, diagnosis and treatment of muscle-specific tyrosine kinase (MuSK) antibody positive myasthenia gravis will be reviewed. Special attention will be paid to clinical phenotyp ...
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Journal ArticleAutoimmunity · August 2010
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Immunosuppression is the mainstay of treatment for myasthenia gravis (MG). In this paper, we review the mechanisms of action and clinical application of corticosteroids and different classes of immunosuppressive drugs that are currently used in MG patients ...
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Journal ArticleNeurology · May 4, 2010
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OBJECTIVE: To study the concurrent and construct validity and test-retest reliability in the practice setting of an outcome measure for myasthenia gravis (MG). METHODS: Eleven centers participated in the validation study of the Myasthenia Gravis Composite ...
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Journal ArticleMuscle Nerve · May 2010
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Two recent randomized, controlled trials failed to demonstrate a benefit of mycophenolate mofetil (MMF) over prednisone in the treatment of myasthenia gravis (MG). We reviewed our experience with MMF in MG to determine whether these trials may have been un ...
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Journal ArticleMuscle Nerve · February 2010
Health-related quality of life (HRQOL) estimates can play an important role in patient care by providing information about the patient's perception of impairment and disability and the degree to which the patient tolerates disease manifestations. The 15-it ...
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Journal ArticleAm J Obstet Gynecol · November 2009
OBJECTIVE: The purpose of this study was to assess the effect of pregnancy and first vaginal delivery on urethral striated sphincter neuromuscular function. STUDY DESIGN: Quantitative electromyographic (EMG) interference pattern analysis of the urethral sp ...
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Journal ArticleMuscle Nerve · September 2009
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Neuromuscular jitter is generally recorded with a single fiber (SF) electromyography (EMG) electrode. Due to concern about using reusable needle electrodes, an acceptable alternative for the SF electrode has been sought. This is a review of the issues invo ...
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Journal ArticleAm J Obstet Gynecol · May 2009
OBJECTIVE: The objective of the study was to assess the prevalence of levator ani denervation and reinnervation 6 months after the first delivery. STUDY DESIGN: Ninety-six primigravida women underwent quantitative electromyography of the levator ani during ...
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Journal ArticleLancet Neurol · May 2009
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Acquired myasthenia gravis (MG) is an autoimmune disorder of the neuromuscular junction in which patients experience fluctuating skeletal muscle weakness that often affects selected muscle groups preferentially. The target of the autoimmune attack in most ...
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Journal ArticleBrain Res Bull · December 16, 2008
Although developmental exposures of rats to low levels of the organophosphate pesticides (OPs), chlorpyrifos (CPF) or diazinon (DZN), both cause persistent neurobehavioral effects, there are important differences in their neurotoxicity. The current study e ...
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Journal ArticleMuscle Nerve · December 2008
We assessed the performance of items from the Quantitative Myasthenia Gravis (QMG), MMT (Manual Muscle Test), and MG-ADL (Myasthenia Gravis - Activities of Daily Living) scales, using data from two recently completed treatment trials of generalized MG. Ite ...
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Journal ArticleMuscle Nerve · November 2008
We determined the strength of correlation among, and responsiveness of, outcome measures used in a multicenter, double-blind, placebo-controlled trial of mycophenolate mofetil in combination with prednisone in myasthenia gravis (MG). The primary efficacy m ...
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Journal ArticleExpert Opin Pharmacother · October 2008
Myasthenia gravis (MG) is an autoimmune disease that leads to muscular weakness, which can significantly affect the patient's daily functions. If left untreated, the mortality rate can be as high as 30%. Effective immunosuppression is the cornerstone of tr ...
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Journal ArticleJ Neuroimmunol · September 15, 2008
Clinical vignettes are presented of five patients with MuSK-antibody positive myasthenia gravis, each of which demonstrates a diagnostic or therapeutic issue that is unique to or characteristic of this condition. Consideration of these issues leads to ques ...
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Journal ArticleNeurology · August 5, 2008
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BACKGROUND: This prospective, randomized, double-blind, placebo-controlled, phase III trial assessed the efficacy, safety, and tolerability of mycophenolate mofetil (MMF) as a steroid-sparing agent in patients with myasthenia gravis (MG). METHODS: Patients ...
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Journal ArticleNeurology · August 5, 2008
OBJECTIVE: To test the hypothesis that mycophenolate mofetil (MMF) with prednisone provides better control of myasthenic weakness than prednisone alone in the initial management of generalized myasthenia gravis (MG). METHODS: Eighty immunosuppression naïve ...
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Journal ArticleMuscle Nerve · August 2008
In 2000 a Task Force of the Myasthenia Gravis Foundation of America recommended development of a quality of life (QOL) measure specific for myasthenia gravis (MG). Extant investigations have relied solely on assessment of physical aspects of daily living i ...
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Journal ArticleMuscle Nerve · August 2008
We describe the process whereby a recently developed myasthenia gravis (MG)-specific quality-of-life (QOL) instrument was reduced from 60 items to 15 items while maintaining potential usefulness in the clinic and in prospective treatment trials. In data fr ...
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Journal ArticleClin Neurophysiol · June 2008
OBJECTIVE: Peripheral nerve ultrasound is an emerging tool in the diagnosis of carpal tunnel syndrome (CTS). Although numerous publications have cited an increased median nerve area at the wrist to be the diagnostic of CTS, there has been considerable vari ...
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Journal ArticleAnn N Y Acad Sci · 2008
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Two randomized controlled trials of mycophenolate mofetil (MMF) in the treatment of myasthenia gravis (MG) were recently completed. Although neither study demonstrated efficacy of MMF in the population of patients studied, there are valuable lessons in the ...
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Journal ArticleNeurology · July 25, 2006
BACKGROUND: Krabbe disease (KD) is associated with marked central and peripheral demyelination and nerve conduction studies (NCS) typically show a mixed sensorimotor demyelinating peripheral neuropathy (PN). OBJECTIVES: To further characterize the PN in a ...
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Journal ArticleNeurology · July 25, 2006
BACKGROUND: Hematopoietic stem cell transplantation (HSCT) may slow the progression of Krabbe disease (KD) if performed early in the disease. The authors' studies indicate that more than 90% of patients with KD have severe abnormalities in peripheral nerve ...
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Journal ArticleClin Neurophysiol · September 2005
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OBJECTIVE: To examine the clinical manifestations and patterns of electromyographic abnormalities in MuSK-Ab positive myasthenia gravis. METHODS: The clinical evaluations and electrodiagnostic testing results of 20 MuSK-Ab positive myasthenia gravis patien ...
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Journal ArticleAm J Phys Med Rehabil · August 2005
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Disorders of the neuromuscular junction have a wide range of clinical presentations, which frequently poses a diagnostic challenge to evaluating clinicians. This article describes the tests used in the diagnosis of diseases of the neuromuscular junction, r ...
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Journal ArticleNeurology · June 14, 2005
We prospectively tested the quantitative myasthenia gravis score (QMG) for responsiveness and longitudinal construct validity in 53 patients with myasthenia gravis. Index of responsiveness was high. Longitudinal construct validity was confirmed by the corr ...
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Journal ArticleSemin Neurol · March 2004
The clinical history and neurological examination provide the most important data on which the diagnosis of autoimmune myasthenia gravis (MG) is based. MG produces symptomatic weakness that predominates in certain muscle groups and typically fluctuates in ...
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Journal ArticleNeurology · November 25, 2003
The authors report a retrospective analysis of the use of mycophenolate mofetil (MyM) in 85 patients with autoimmune myasthenia gravis. The Myasthenia Gravis Foundation of America (MGFA) postintervention status (PIS) was used to characterize the treatment ...
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Journal ArticleAnn N Y Acad Sci · September 2003
A high index of suspicion is essential in arriving at the correct diagnosis of Lambert-Eaton myasthenic syndrome (LEMS). LEMS should be considered in the differential in any patient who has proximal weakness, reduced or absent muscle stretch reflexes, and ...
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Journal ArticleNeurology · June 24, 2003
Serum antibodies to muscle-specific receptor tyrosine kinase were detected in 12 of 32 patients with generalized seronegative MG. All were women, with onset between ages 21 and 59 years. Seven had prominent neck, shoulder, or respiratory muscle weakness an ...
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Journal ArticleExpert Rev Neurother · September 2002
Myasthenia gravis is the best understood of the autoimmune diseases and a number of treatments are currently used to produce clinical improvement. However, due to the scarcity of evidence-based and comparative data, there is still no consensus on many ther ...
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Journal ArticleJ Clin Neuromuscul Dis · September 2002
This article reviews controversies surrounding the concept of myasthenic crisis. Literature review and our own experience demonstrate that there is disagreement about how a crisis should be defined, what the implications of a crisis are, whether crises are ...
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Journal ArticleCurr Neurol Neurosci Rep · January 2002
Recent advances in the diagnosis and treatment of acquired myasthenia gravis (MG) are reviewed. Increased awareness about the need for more uniform methods of reporting treatment trials for MG has prompted systematic review of the literature and inspired a ...
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Journal ArticleMuscle Nerve Suppl · 2002
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The major clinical impact of single-fiber electromyography has been from its role in confirming, or excluding, the diagnosis of myasthenia gravis (MG). Jitter measurements also have a clinical role in demonstrating changes in disease severity in patients w ...
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Journal ArticleMuscle Nerve Suppl · 2002
Isaacs' syndrome (acquired neuromyotonia) is an antibody-mediated potassium channel disorder (channelopathy). The target channel proteins of the antigens are voltage-gated potassium channels (VGKCs), especially dendrotoxin-sensitive fast potassium channels ...
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Journal ArticleMuscle Nerve Suppl · 2002
The major clinical impact of single-fiber electromyography has been from its role in confirming, or excluding, the diagnosis of myasthenia gravis (MG). Jitter measurements also have a clinical role in demonstrating changes in disease severity in patients w ...
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Journal ArticleJ Immunol · February 15, 2001
The human thymus is required for establishment of the T cell pool in fetal life, but postnatal thymectomy does not lead to immunodeficiency in humans. Because thymectomy in humans is performed for treatment of myasthenia gravis (MG), we have studied patien ...
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Journal ArticleNeurology · January 9, 2001
In an open-label study, 12 patients with refractory MG or who were taking only corticosteroids and required additional immunosuppression received mycophenolate mofetil 1 g twice daily for 6 months. A reduction of three points in a quantified MG score and t ...
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Journal ArticleAm J Obstet Gynecol · December 2000
OBJECTIVE: The purpose of this study was to compare results of electromyographic assessment of muscular recruitment between nulliparous control subjects without pelvic floor dysfunction and parous subjects with genuine stress urinary incontinence and with ...
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Journal ArticleAm J Obstet Gynecol · November 2000
OBJECTIVES: Our aims were to introduce a method of digital quantitative electromyography of the levator ani and external anal sphincter muscles and to establish reference values. STUDY DESIGN: Fifteen nulliparous, symptom-free women underwent concentric ne ...
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Journal ArticleJ Clin Neuromuscul Dis · September 2000
PURPOSE: To determine if the jitter in the extensor digitorum communis (EDC) muscle in patients with purely ocular myasthenia gravis (OMG) predicts the subsequent development of generalized myasthenic weakness-Methods: Included in this retrospective study ...
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Journal ArticleNeurology · August 8, 2000
The authors reviewed the records of patients with myasthenia gravis who took cyclosporine for at least 6 months between November 1987 and January 1999. Of 57 patients who took cyclosporine for an average of 3.5 years, 55 (96%) had clinical improvement. The ...
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Journal ArticleNeurology · June 13, 2000
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The authors reviewed the incidence of cancer, repetitive nerve stimulation findings, and response to treatment in 73 patients with Lambert-Eaton myasthenic syndrome. Thirty-one patients (42%) had lung cancer, 29 small cell. Doubling of the compound motor a ...
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Journal ArticleJ Clin Neuromuscul Dis · June 2000
Afterdischarges in motor nerve stimulation studies help distinguish slow channel congenital myasthenic syndrome (SCCMS) from acquired myasthenia gravis (MG) We present a patient with fatigable weakness in whom afterdischarges were not initially apparent. A ...
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Journal ArticleMuscle Nerve · June 2000
Electromyographic recordings of fibrillation potentials (FPs) and positive sharp waves (PSWs) demonstrate transformation of FP to PSW and vice versa, atypical firing patterns, changes in waveform shape and amplitude, and time-locked potentials. The etiolog ...
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Journal ArticlePostgrad Med · April 2000
Myasthenic crisis, or respiratory failure requiring intubation and mechanical ventilation, may be caused by infections, aspiration, physical and emotional stress, and changes in medication. Although no single factor determines the need for respiratory supp ...
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Journal ArticleNeurology · February 8, 2000
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OBJECTIVES: The authors report the results of a prospective, placebo-controlled, randomized study to evaluate the effectiveness of 3,4-diaminopyridine (DAP) in patients with Lambert-Eaton myasthenic syndrome (LEMS) and to determine the acute and long-term ...
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Journal ArticleMuscle Nerve · December 1999
X-linked bulbospinal neuronopathy (XLBSN) or Kennedys disease is a rare inherited neuromuscular disease characterized by adult-onset muscle weakness, usually in a limb-girdle distribution. It is frequently misdiagnosed despite a distinctive clinical presen ...
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Journal ArticleNeuromuscul Disord · October 1999
Miyoshi myopathy is a rare autosomal recessive distal myopathy characterized by early and prominent involvement of the posterior compartment of the legs. We describe two patients with the clinical diagnosis of Miyoshi myopathy who demonstrated marked infla ...
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Journal ArticleNeurology · April 22, 1999
We report four cases of Lambert-Eaton myasthenic syndrome (LEMS) or myasthenia gravis (MG) associated with pulmonary neuroendocrine carcinoma having prolonged survival. The tumors were atypical carcinoid or large cell neuroendocrine carcinoma. LEMS is asso ...
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Journal ArticleMuscle Nerve · April 1999
The term continuous motor unit activity (CMUA) may be used to refer to the involuntary, sustained activity of motor units caused by hyperactivity of peripheral motor nerves. CMUA has been reported in association with acquired neuropathies such as chronic i ...
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Journal ArticleNeurology · October 1997
Outcome after carpal tunnel surgery was studied retrospectively in 32 patients with peripheral neuropathy and carpal tunnel syndrome. Nocturnal paresthesias were almost universally relieved, followed in order of responsiveness by pain, numbness, and weakne ...
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Journal ArticleJ Clin Neurophysiol · September 1996
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A review of quantitative methods for electromyography is given. Background information about motor unit anatomy, physiology, and pathology is provided to explain some of the presented electrophysiological phenomena. Different aspects of quantitation, such ...
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Journal ArticleMed Biol Eng Comput · September 1996
The stepwise linear discriminant analysis method is used to develop optimal combinations of features measured from the electromyographic interference pattern, with the aim of minimising the misclassification rate in controls while maximising the correct cl ...
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Journal ArticleJ Clin Neurophysiol · September 1996
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The electromyographic interference pattern (EMG-IP) contains information about the number, firing rate, and recruitment characteristics of motor units, and information regarding the waveforms of the recruited motor units. Muscle and nerve diseases produce ...
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Journal ArticleMuscle Nerve · September 1996
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Single-fiber electromyography (SFEMG) is a selective recording technique in which a needle electrode with a small recording surface in the side is used to identify action potentials from individual muscle fibers. The SFEMG parameters of greatest clinical u ...
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Journal ArticleProc Natl Acad Sci U S A · August 20, 1996
Voltage-gated Ca2+ channels are categorized as either high-voltage activated (HVA) or low-voltage activated (LVA), and a subtype (or subtypes) of HVA Ca2+ channels link the presynaptic depolarization to rapid neuro-transmitter release. Reductions in transm ...
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Journal ArticleAnn Neurol · May 1995
The Lambert-Eaton myasthenic syndrome (LEMS) is a rare condition in which weakness results from a presynaptic abnormality of acetylcholine release at the neuromuscular junction. It was first described as a paraneoplastic syndrome in patients with lung canc ...
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Journal ArticleMuscle Nerve · September 1994
We compared changes in amplitude and area of surface recorded compound motor action potentials (CMAPs) during 20-Hz repetitive nerve stimulation and after maximum voluntary contraction in patients with the Lambert-Eaton myasthenic syndrome (LEMS), myasthen ...
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Journal ArticleNeurology · July 1994
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We assessed the influence of race, sex, and puberty upon clinical features and outcome in 115 patients with autoimmune juvenile myasthenia gravis (JMG). These demographic variables influenced not only disease incidence but also disease severity, response t ...
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Journal ArticleNeurol Clin · May 1994
Electrodiagnostic studies are valuable in confirming the diagnosis of a disorder of neuromuscular transmission. They are used to distinguish presynaptic and postsynaptic abnormalities. These studies provide an objective measure of the severity of the illne ...
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Journal ArticleNeurol Clin · May 1994
Although there is general agreement among experienced physicians on the usefulness of different treatments for myasthenia gravis (MG), so many variations are possible that in practice, similar patients treated by different physicians rarely are treated the ...
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Journal ArticleMuscle Nerve · February 1994
We performed RNS and SFEMG studies of the same muscle in 46 patients with myasthenia gravis. Maximum decrement to 3-5-Hz stimulation before and after maximum voluntary exercise, percentage of action potential pairs with increased jitter and blocking, and m ...
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Journal ArticleNeurology · May 1993
We analyzed relationships among pubertal stage at disease onset, sex, disease severity, and acetylcholine receptor antibody (AChR Ab) levels in 46 patients with autoimmune juvenile myasthenia gravis (JMG). Female predominance was least in children with pre ...
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Journal ArticleMuscle Nerve · May 1993
We report the use of single fiber electromyography (SFEMG) to demonstrate changes in the physiologic abnormality of myasthenia gravis (MG) during pregnancy. A 23-year-old became pregnant 15 months after the onset of mild ocular weakness. On initial evaluat ...
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Journal ArticleJ Clin Neurophysiol · April 1993
This is a review of the neurophysiological techniques used clinically to demonstrate abnormalities of neuromuscular transmission. The use of these tests in myasthenia gravis, Lambert-Eaton myasthenic syndrome, congenital myasthenic syndromes, and other con ...
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Journal ArticleMuscle and Nerve · 1992
Single fiber electromyography (SFEMG) measurements of fiber density and jitter are used in the diagnosis of a variety of peripheral nervous system disorders. However, the normal values of these measurements for most muscles and age groups are not well docu ...
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Journal ArticleNeurology · July 1991
Serial single-fiber EMG (SF-EMG) studies in the frontalis muscle showed increased jitter with normal fiber density 15 days after facial nerve trauma. Both measurements were increased thereafter. The maximum increases occurred at 37 days, coincident with th ...
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Journal ArticleMuscle Nerve · February 1991
Motor unit action potentials (MUAPs) and the electromyographic (EMG) interference pattern (IP) were recorded from the biceps muscle of 5 normal subjects using both a concentric needle (CN) and a disposable monopolar needle (MN) electrode. The MUAPs recorde ...
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Journal ArticleMuscle Nerve · January 1991
Factors that affect the shape of the so-called "normal cloud" of the turns and amplitude measurements of the electromyographic interference pattern are investigated. As the force of voluntary contraction increases from low to moderate levels, the number of ...
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Journal ArticleMuscle Nerve · October 1990
There is currently considerable interest in using disposable concentric needle (CN) electrodes for clinical electromyography (EMG). To determine how these electrodes compare with reusable CN electrodes, we have compared signals recorded by these two electr ...
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Journal ArticleMuscle Nerve · October 1990
When the force of muscle contraction is increased, the amplitude of the EMG envelope (ENAMP) increases. The ENAMP is usually assessed subjectively and its value in EMG analysis has been established. In this article we describe a method to make automatic me ...
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Journal ArticleMuscle Nerve · March 1990
Fifty-four quantitative electromyographic (EMG) studies were made in 37 patients with inflammatory myopathy (IM) at different points in their clinical course and treatment. All studies were performed in the biceps brachii which varied in clinical strength. ...
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Journal ArticleMedical & Biological Engineering & Computing · November 1, 1989
A technique of extracting individual motor unit action potentials (MUAPs) from EMG signals by median averaging, a modification of an existing method, is presented. To compare different techniques of MUAP extraction, 89 MUAPs were recorded with a concentric ...
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Journal ArticleMuscle Nerve · August 1989
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In two patients with hemifacial spasm (HFS), single-fiber EMG recordings in facial muscles demonstrated low jitter in the late responses produced by stimulation of peripheral branches to other facial muscles. Surgical decompression of the facial nerve in o ...
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Journal ArticleMuscle Nerve · April 1989
Motor unit action potentials (MUAPs) were recorded from the biceps muscle of normal subjects and of patients with nerve or muscle diseases. Principal component analysis of the MUAP amplitude, area, area/amplitude ratio, duration, and the number of turns an ...
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Journal ArticleMuscle Nerve · March 1989
Normal motor units (MUs) were simulated and their architecture altered to simulate the changes produced by myopathy. The concentric needle electromyographic recordings of motor unit action potentials (MUAPs) from the MUs were then also simulated. These sim ...
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Journal ArticleMuscle Nerve · February 1989
We report four patients with myasthenia gravis (MG) in whom single-fiber electromyography (SFEMG) jitter measurements were normal in some muslces while they were taking pyridostigmine and became abnormal 2-14 days after the medication was discontinued. Whe ...
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Journal ArticleMuscle Nerve · February 1989
This study was performed to evaluate an automatic method of motor unit action potential (MUAP) analysis developed in our laboratory. MUAPs were recorded from the biceps brachii muscle of 68 normal subjects and 122 patients with nerve or muscle disease. The ...
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Journal ArticleMuscle Nerve · January 1989
A middle-aged man with lymphomatous meningitis developed acute cervical radiculopathy. Repetitive stimulation of an affected nerve revealed a "U-Shaped" decrement. The decrement was absent 10 days later. We hypothesize that the decrement was secondary to s ...
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Journal ArticleElectroencephalogr Clin Neurophysiol · December 1988
This study was performed to compare different techniques of analyzing the electromyographic interference pattern (IP). Recordings were made from the biceps muscle with a concentric needle electrode at different sites and at different constant levels of vol ...
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Journal ArticleJ Neurol Sci · October 1988
Serum was obtained from 7 patients with the Lambert-Eaton myasthenic syndrome (LES), 3 patients with small-cell carcinoma of the lung (SCCL), and 9 healthy control subjects. Serum samples were applied in vitro to the rat neuromuscular junction (for 1-3 h f ...
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Journal ArticleElectroencephalogr Clin Neurophysiol · August 1988
Using computer simulation techniques, reinnervation of motor units (MUs) was studied by increasing the number of muscle fibers in the MU without changing the MU territory. The fiber density (FD) measured by single fiber EMG electrodes, the amplitude, area ...
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Journal ArticleElectroencephalogr Clin Neurophysiol · June 1988
Computer simulations indicate that measurements of the area of motor unit action potentials (MUAPs) recorded with a concentric needle electrode could be useful in differentiating between neuropathy and myopathy. However, MUAP area varies markedly when the ...
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Journal ArticleMuscle Nerve · February 1988
Computer simulations of motor unit action potentials (MUAPs) as measured by a concentric needle (CN) electromyography (EMG) electrode in normal motor units (MUs) indicated that the MUAP amplitude is determined mainly by the proximity of the electrode to th ...
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Journal ArticleAppl Neurophysiol · 1988
A patient developed continuous patterned involuntary movements of abduction-adduction, flexion-extension of his right lower extremity following surgical placement of spinal dorsal root entry zone lesions for the treatment of phantom limb pain. The stereoty ...
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Journal ArticleMuscle Nerve · May 1987
We have reviewed the electromyographic (EMG) studies of 17 patients with chronic progressive external ophthalmoplegia (CPEO). In 13 of 17 patients, conventional concentric needle EMG demonstrated a "myopathic" pattern, usually predominating in the shoulder ...
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Journal ArticleAnn Neurol · March 1987
A new dietary regimen has been administered for periods ranging from 60 days to 1 1/2 years in 34 patients with various forms of X-linked adrenoleukodystrophy (ALD), as well as in 1 patient with neonatal ALD and 1 patient with infantile Refsum's disease. T ...
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Journal ArticleMuscle Nerve · 1987
The double-step technique of repetitive nerve stimulation was compared with repetitive nerve stimulation to a distal and a proximal muscle and with single fiber needle electromyography in 10 patients with myasthenia gravis. We conclude that the double-step ...
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Journal ArticleIEEE/Engineering in Medicine and Biology Society Annual Conference · December 1, 1986
The physiologic motor unit (MU) consists of an anterior horn cell, its axon and nerve branches and all the muscle fibers it enervates. The muscle fibers of a single MU are distributed randomly within a certain portion of the muscle (the MU territory), inte ...
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Journal ArticlePediatrics · July 1986
A 10-year-old girl was treated for an acute asthmatic attack with ventilation and a high-dose steroids regimen. An areflexic paralysis of the four limbs was noted when artificial ventilatory support was withdrawn. Electromyography and a muscle biopsy revea ...
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Journal ArticleMuscle Nerve · June 1986
The electromyographic (EMG) interference pattern (IP) was simulated by adding together motor unit action potentials (MUAPs) of different sizes that had been recorded by a concentric needle EMG electrode. The number of turns (NT) of the simulated IP increas ...
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Journal ArticleMuscle Nerve · June 1986
We have developed three new features of the electromyographic interference pattern (IP), based on the turns and amplitude of the signal, to quantitate some of the features of the IP that are usually assessed subjectively by an electromyographer. The activi ...
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Journal ArticleNeurology · April 1986
In four patients who received periocular injections of botulinum toxin for blepharospasm, abnormal neuromuscular transmission was demonstrated by single-fiber EMG in arm muscles. The time course with which the abnormalities developed and cleared, as well a ...
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Journal ArticleMuscle Nerve · 1986
Single-fiber electromyography (SFEMG) demonstrates abnormal jitter in virtually all (99%) patients with myasthenia gravis (MG). One muscle, the extensor digitorum communis, is abnormal in most patients with this disease, but to obtain the maximum diagnosti ...
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Journal ArticleMuscle Nerve · 1986
We have defined three new features of the electromyographic (EMG) interference pattern (IP): activity, upper centile amplitude (UCA), and number of small segments (NSS). These parameters were measured in simulated IPs constructed by adding together motor u ...
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Journal ArticleMuscle Nerve · 1986
The electromyographic (EMG) interference pattern (IP) was measured in the biceps muscle of 16 normal male and 17 normal female subjects. The activity, upper centile amplitude (UCA), and the number of small segments (NSS) (defined in a companion paper) were ...
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Journal ArticleIEEE Transactions on Biomedical Engineering · 1984
Macro electromyography (EMG) is a recently described recording technique allowing a nonselective recording of motor unit activity. The pick-up area of the electrode, the cannula of a modified single-fiber electrode, covers the entire motor unit territory. ...
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Journal ArticleArch Neurol · June 1983
Symptoms of McArdle's disease (muscle phosphorylase deficiency) commonly begin in childhood or adolescence. Late onset of the disease is rare. We describe a 76-year-old man whose symptoms began at age 74 years with sudden onset of proximal muscle weakness ...
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Journal ArticleJ Neurol Sci · June 1983
Patients with myasthenia gravis (MG) have increased tolerance to the neuromuscular blocking properties of suxamethonium (SCh) and decamethonium (C10) and exhibit a reversal of the C10-induced block by neostigmine. The effects of these drugs were compared i ...
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Journal ArticleComput Programs Biomed · June 1983
This paper describes an interactive program for real time analysis of the neuromuscular jitter. Two implementations, using different hardware for data acquisition, reflect the modular design and flexibility of the software. Details on the time windows are ...
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Journal ArticlePflugers Arch · November 11, 1982
A simple biological signal generator capable of reproducing complex biopotential waveforms is described. It is constructed by a combination of digital and analog circuit components and can be used under different experimental conditions, such as in calibra ...
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Journal ArticleAnn Neurol · June 1981
The pattern of stapedial reflex fatigue in response to pulsed acoustic stimulation was measured and compared to results of repetitive nerve stimulation and single-fiber electromyography (EMG) in 89 patients with myasthenia gravis. Studies were also made on ...
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Journal ArticleAnn N Y Acad Sci · 1981
We have found a wide range of mean MEPP amplitude in intercostal muscle biopsies from 43 patients with MG, including several values in the normal range. There was no correlation between MEPP amplitude and the severity of clinical disease as assessed by man ...
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Journal ArticleJ Neurol Neurosurg Psychiatry · November 1980
In a patient with the Eaton-Lambert syndrome, 4-aminopyridine produced temporary improvement of clinical and electromyographic abnormalities. Application of the drug in vitro to intercostal muscle from the patient produced an increase in the evoked release ...
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Journal ArticleNeurology · July 1980
Adult female Lewis rats were rendered immunologically tolerant to human gamma globulin, and were given a single intravenous injection of human myasthenic or normal control serum containing 7.5 to 12 mg of immunoglobulin G. The mean amplitude of miniature e ...
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Journal ArticleMuscle Nerve · 1980
The effects of 4-aminopyridine (4-AP) on neuromuscular transmission were studied in vitro in the rat flexor digitorum longus muscles. 4-AP produced dose-dependent increases in endplate potential (EPP) amplitude, in rise time to peak, and in the average num ...
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Journal ArticleMuscle Nerve · 1980
The in vitro effects of 4-aminopyridine (4-AP) on neuromuscular transmission were determined by microelectrode techniques in intercostal muscles from patients with myasthenia gravis (MG) and the Eaton-Lambert syndrome (ELS), and in forelimb muscles from ra ...
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Journal ArticleNeurology · 1980
Adult female Lewis rats were rendered immunologically tolerant to human gamma globulin, and were given a single intravenous injection of human myasthenic or normal control serum containing 7.5 to 12 mg of immunoglobulin G. The mean amplitude of miniature e ...
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Journal ArticleMulti-phase Transport: Fundamentals, Reactor Safety, Applications · January 1, 1980
This study deals with the development of an efficient signal representation in bioelectric signal analysis. The authors used, as an example, macro EMG, which is a new electromyographic recording technique, and performed a quantitative comparison of the rec ...
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Journal ArticleMulti-phase Transport: Fundamentals, Reactor Safety, Applications · 1980
This study deals with the development of an efficient signal representation in bioelectric signal analysis. The authors used, as an example, macro EMG, which is a new electromyographic recording technique, and performed a quantitative comparison of the rec ...
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Journal ArticleJ Neurol Sci · April 1979
Electrophysiological investigations of the effects of bath-applied prednisolone at the neuromuscular junction were performed in muscles from normal rats and rats with experimental autoimmune myasthenia gravis (EAMG). In muscles from both groups, prednisolo ...
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Journal ArticleMuscle Nerve · 1979
Treatment of patients with D-penicillamine (D-P) has been associated with a syndrome similar to myasthenia gravis (MG). To explore this association, we examined the effects of D-P on neuromuscular transmission in rat muscle. In the first experiment, bath-a ...
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Journal ArticleNeurology · January 1979
One-hundred-sixty single-fiber EMG studies of the extensor digitorum communis muscle were performed on 127 patients with myasthenia gravis; 131 demonstrated defective neuromuscular transmission. Jitter determinations in the biceps, deltoid or frontalis mus ...
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Journal ArticleMuscle Nerve · 1978
Neonatal rats born of and nursed by mothers immunized with Torpedo acetylcholine receptor protein developed a defect of neuromuscular transmission as indicated by reduced miniature endplate potential amplitudes. It is likely that antibodies to the Torpedo ...
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Journal ArticleArch Neurol · February 1977
In rats immunized with purified acetylcholine receptor protein (AChRP) from Torpedo electroplax, a defect of neuromuscular transmission physiologically identical to that seen in myasthenia gravis developed. The most sensitive index of the neuromuscular blo ...
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Journal Article · January 1, 1977
This paper describes an automated instrumentation system for the acquisition, analysis, and storage of miniature end-plate potentials. The system provides immediate analysis and display of MEPP frequency, peak amplitude, duration, rise-time and decay time ...
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Journal ArticleElectroencephalography and Clinical Neurophysiology · January 1, 1977
Single-fiber EMG studies were performed 106 times in the extensor digitorum communis muscles of 68 patients with myasthenia gravis. The patients were in various stages of treatment, usually with high doses of corticosteroids. In each study the overall mean ...
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Journal ArticleAnesthesia and Analgesia · January 1, 1977
A 6 yr old healthy girl suffered a traction injury to her right sciatic nerve. Within 24 hours after the accident, she complained of well-localized pain in the right knee and reported that the area was exquisitely sensitive to touch. Another well-localized ...
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Journal ArticleArch Neurol · August 1976
We present a family with dominantly inherited myotonia congenita and painful, electrically silent muscle contractions after exertion. In two family members, painful muscle contractions occurred coincident with hypothyroidism, and improved after thyroid rep ...
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Journal ArticleArchives of Neurology · 1974
Exercise is known to abort attacks of familial hypokalemic periodic paralysis (FHoPP) and to improve muscle strength during attacks. Various explanations have been offered for this beneficial effect of muscle activity, none of them satisfactory. In the pre ...
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