Scholars@Duke
Vern Charles Juel

Vern Charles Juel

Professor of Neurology
Neurology, Neuromuscular Disease
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Selected Publications

Immune dysregulation in chronic inflammatory demyelinating polyneuropathy.

Journal Article J Neuroimmunol · June 15, 2024 OBJECTIVE: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disorder of the peripheral nerves with an incompletely understood underlying pathophysiology. This investigation focused on defining B and T cell frequencies, T cell funct ... Full text Link to item Cite

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

Journal Article Lancet 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 ... Full text Link to item Cite

Lambert-Eaton Myasthenic Syndrome BMJ Best Practice

Internet Publication · February 21, 2024 Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disorder of the neuromuscular junction. LEMS occurs either as a paraneoplastic disorder in association with an underlying cancer (CA-LEMS), or without cancer and as part of a more general autoi ... Link to item Cite

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

Journal Article J 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 ... Full text Link to item Cite

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

Conference Muscle 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 ... Full text Link to item Cite

Clinicoserological insights into patients with immune checkpoint inhibitor-induced myasthenia gravis.

Journal Article Ann Clin Transl Neurol · May 2023 To compare the immunopathology of immune checkpoint inhibitor-induced myasthenia gravis (ICI-MG) and idiopathic MG, we profiled the respective AChR autoantibody pathogenic properties. Of three ICI-MG patients with AChR autoantibodies, only one showed compl ... Full text Link to item Cite

Safety and efficacy of zilucoplan in patients with generalised myasthenia gravis (RAISE): a randomised, double-blind, placebo-controlled, phase 3 study.

Journal Article Lancet Neurol · May 2023 BACKGROUND: Generalised myasthenia gravis is a chronic, unpredictable, and debilitating rare disease, often accompanied by high treatment burden and with an unmet need for more efficacious and well tolerated treatments. Zilucoplan is a subcutaneous, self-a ... Full text Link to item Cite

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

Conference Muscle 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 ... Full text Link to item Cite

First-in-Human Clinical Trial to Assess the Safety, Tolerability and Pharmacokinetics of Single Doses of NTM-1633, a Novel Mixture of Monoclonal Antibodies against Botulinum Toxin E.

Journal Article Antimicrob Agents Chemother · April 19, 2022 Botulism is a rare, life-threatening paralytic disease caused by botulinum neurotoxin (BoNT). Available treatments including an equine antitoxin and human immune globulin are given postexposure and challenging to produce and administer. NTM-1633 is an equi ... Full text Link to item Cite

A Case of Probable Multifocal Motor Neuropathy With Clinical Stability for Ten Years After a Single Treatment of Rituximab.

Journal Article J Clin Neuromuscul Dis · March 1, 2022 Multifocal motor neuropathy is a rare, immune-mediated motor neuropathy with asymmetric, often debilitating progressive weakness. The efficacy of intravenous immunoglobulin in this disease is well established; however, the response typically wanes over tim ... Full text Link to item Cite

Eculizumab in refractory generalized myasthenia gravis previously treated with rituximab: subgroup analysis of REGAIN and its extension study.

Journal Article Muscle Nerve · December 2021 INTRODUCTION/AIMS: Individuals with refractory generalized myasthenia gravis (gMG) who have a history of rituximab use and experience persistent symptoms represent a population with unmet treatment needs. The aim of this analysis was to evaluate the effica ... Full text Link to item Cite

Consensus disease definitions for neurologic immune-related adverse events of immune checkpoint inhibitors.

Journal Article J Immunother Cancer · July 2021 Expanding the US Food and Drug Administration-approved indications for immune checkpoint inhibitors in patients with cancer has resulted in therapeutic success and immune-related adverse events (irAEs). Neurologic irAEs (irAE-Ns) have an incidence of 1%-12 ... Full text Link to item Cite

Safety, Tolerability, and Pharmacokinetics of NTM-1632, a Novel Mixture of Three Monoclonal Antibodies against Botulinum Toxin B.

Journal Article Antimicrob Agents Chemother · June 17, 2021 Botulism is a rare, life-threatening paralytic disease caused by Clostridium botulinum neurotoxin (BoNT). Available treatments, including an equine antitoxin and human immune globulin, are given postexposure and challenging to produce and administer. NTM-1 ... Full text Link to item Cite

Reduced plasmablast frequency is associated with seronegative myasthenia gravis.

Journal Article Muscle Nerve · April 2021 BACKGROUND: The immunopathology of autoimmune seronegative myasthenia gravis (SN MG) is poorly understood. Our objective was to determine immune profiles associated with a diagnosis of SN MG. METHODS: We performed high-dimensional flow cytometry on blood s ... Full text Link to item Cite

Immune dysregulation in chronic inflammatory demyelinating polyneuropathy.

Journal Article J Neuroimmunol · June 15, 2024 OBJECTIVE: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disorder of the peripheral nerves with an incompletely understood underlying pathophysiology. This investigation focused on defining B and T cell frequencies, T cell funct ... Full text Link to item Cite

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

Journal Article Lancet 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 ... Full text Link to item Cite

Lambert-Eaton Myasthenic Syndrome BMJ Best Practice

Internet Publication · February 21, 2024 Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disorder of the neuromuscular junction. LEMS occurs either as a paraneoplastic disorder in association with an underlying cancer (CA-LEMS), or without cancer and as part of a more general autoi ... Link to item Cite

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

Journal Article J 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 ... Full text Link to item Cite

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

Conference Muscle 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 ... Full text Link to item Cite

Clinicoserological insights into patients with immune checkpoint inhibitor-induced myasthenia gravis.

Journal Article Ann Clin Transl Neurol · May 2023 To compare the immunopathology of immune checkpoint inhibitor-induced myasthenia gravis (ICI-MG) and idiopathic MG, we profiled the respective AChR autoantibody pathogenic properties. Of three ICI-MG patients with AChR autoantibodies, only one showed compl ... Full text Link to item Cite

Safety and efficacy of zilucoplan in patients with generalised myasthenia gravis (RAISE): a randomised, double-blind, placebo-controlled, phase 3 study.

Journal Article Lancet Neurol · May 2023 BACKGROUND: Generalised myasthenia gravis is a chronic, unpredictable, and debilitating rare disease, often accompanied by high treatment burden and with an unmet need for more efficacious and well tolerated treatments. Zilucoplan is a subcutaneous, self-a ... Full text Link to item Cite

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

Conference Muscle 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 ... Full text Link to item Cite

First-in-Human Clinical Trial to Assess the Safety, Tolerability and Pharmacokinetics of Single Doses of NTM-1633, a Novel Mixture of Monoclonal Antibodies against Botulinum Toxin E.

Journal Article Antimicrob Agents Chemother · April 19, 2022 Botulism is a rare, life-threatening paralytic disease caused by botulinum neurotoxin (BoNT). Available treatments including an equine antitoxin and human immune globulin are given postexposure and challenging to produce and administer. NTM-1633 is an equi ... Full text Link to item Cite

A Case of Probable Multifocal Motor Neuropathy With Clinical Stability for Ten Years After a Single Treatment of Rituximab.

Journal Article J Clin Neuromuscul Dis · March 1, 2022 Multifocal motor neuropathy is a rare, immune-mediated motor neuropathy with asymmetric, often debilitating progressive weakness. The efficacy of intravenous immunoglobulin in this disease is well established; however, the response typically wanes over tim ... Full text Link to item Cite

Eculizumab in refractory generalized myasthenia gravis previously treated with rituximab: subgroup analysis of REGAIN and its extension study.

Journal Article Muscle Nerve · December 2021 INTRODUCTION/AIMS: Individuals with refractory generalized myasthenia gravis (gMG) who have a history of rituximab use and experience persistent symptoms represent a population with unmet treatment needs. The aim of this analysis was to evaluate the effica ... Full text Link to item Cite

Consensus disease definitions for neurologic immune-related adverse events of immune checkpoint inhibitors.

Journal Article J Immunother Cancer · July 2021 Expanding the US Food and Drug Administration-approved indications for immune checkpoint inhibitors in patients with cancer has resulted in therapeutic success and immune-related adverse events (irAEs). Neurologic irAEs (irAE-Ns) have an incidence of 1%-12 ... Full text Link to item Cite

Safety, Tolerability, and Pharmacokinetics of NTM-1632, a Novel Mixture of Three Monoclonal Antibodies against Botulinum Toxin B.

Journal Article Antimicrob Agents Chemother · June 17, 2021 Botulism is a rare, life-threatening paralytic disease caused by Clostridium botulinum neurotoxin (BoNT). Available treatments, including an equine antitoxin and human immune globulin, are given postexposure and challenging to produce and administer. NTM-1 ... Full text Link to item Cite

Reduced plasmablast frequency is associated with seronegative myasthenia gravis.

Journal Article Muscle Nerve · April 2021 BACKGROUND: The immunopathology of autoimmune seronegative myasthenia gravis (SN MG) is poorly understood. Our objective was to determine immune profiles associated with a diagnosis of SN MG. METHODS: We performed high-dimensional flow cytometry on blood s ... Full text Link to item Cite

Knowledge and perceptions of the COVID-19 pandemic among patients with myasthenia gravis.

Journal Article Muscle 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 ... Full text Link to item Cite

Efficacy and Safety of Rozanolixizumab in Moderate to Severe Generalized Myasthenia Gravis: A Phase 2 Randomized Control Trial.

Journal Article Neurology · February 9, 2021 OBJECTIVE: To explore the clinical efficacy and safety of subcutaneous (SC) rozanolixizumab, an anti-neonatal Fc receptor humanized monoclonal antibody, in patients with generalized myasthenia gravis (gMG). METHODS: In this phase 2a, randomized, double-bli ... Full text Link to item Cite

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

Journal Article Muscle 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 ... Full text Link to item Cite

Effect of Ezogabine on Cortical and Spinal Motor Neuron Excitability in Amyotrophic Lateral Sclerosis: A Randomized Clinical Trial.

Journal Article JAMA Neurol · February 1, 2021 IMPORTANCE: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease of the motor nervous system. Clinical studies have demonstrated cortical and spinal motor neuron hyperexcitability using transcranial magnetic stimulation and thresh ... Full text Link to item Cite

Post-intervention Status in Patients With Refractory Myasthenia Gravis Treated With Eculizumab During REGAIN and Its Open-Label Extension.

Journal Article Neurology · January 26, 2021 OBJECTIVE: To evaluate whether eculizumab helps patients with anti-acetylcholine receptor-positive (AChR+) refractory generalized myasthenia gravis (gMG) achieve the Myasthenia Gravis Foundation of America (MGFA) post-intervention status of minimal manifes ... Full text Open Access Link to item Cite

Imbalance in T follicular helper cells producing IL-17 promotes pro-inflammatory responses in MuSK antibody positive myasthenia gravis.

Journal Article J Neuroimmunol · August 15, 2020 A detailed understanding of the role of Tfh cells in MuSK-antibody positive myasthenia gravis (MuSK-MG) is lacking. We characterized phenotype and function of Tfh cells in MuSK-MG patients and controls. We found similar overall Tfh and follicular regulator ... Full text Link to item Cite

Consistent improvement with eculizumab across muscle groups in myasthenia gravis.

Journal Article Ann Clin Transl Neurol · August 2020 OBJECTIVE: To assess whether eculizumab, a terminal complement inhibitor, improves patient- and physician-reported outcomes (evaluated using the myasthenia gravis activities of daily living profile and the quantitative myasthenia gravis scale, respectively ... Full text Open Access Link to item Cite

'Minimal symptom expression' in patients with acetylcholine receptor antibody-positive refractory generalized myasthenia gravis treated with eculizumab.

Journal Article J Neurol · July 2020 BACKGROUND: The efficacy and tolerability of eculizumab were assessed in REGAIN, a 26-week, phase 3, randomized, double-blind, placebo-controlled study in anti-acetylcholine receptor antibody-positive (AChR+) refractory generalized myasthenia gravis (gMG), ... Full text Open Access Link to item Cite

Inhibition of the transcription factor ROR-γ reduces pathogenic Th17 cells in acetylcholine receptor antibody positive myasthenia gravis.

Journal Article Exp Neurol · March 2020 IL-17 producing CD4 T cells (Th17) cells increase significantly with disease severity in myasthenia gravis (MG) patients. To suppress the generation of Th17 cells, we examined the effect of inhibiting retinoic acid receptor-related-orphan-receptor-C (RORγ) ... Full text Link to item Cite

Clinical outcome measures following plasma exchange for MG exacerbation.

Journal Article Ann Clin Transl Neurol · October 2019 Our objective is to report longitudinal results of the MG-ADL, MG-Composite, MG-MMT, and MG-QoL15 in an open-label trial of therapeutic plasma exchange in myasthenia gravis. Ten MG patients experiencing exacerbation had assessments prior to, immediately fo ... Full text Link to item Cite

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

Journal Article Muscle 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 ... Full text Link to item Cite

Eculizumab improves fatigue in refractory generalized myasthenia gravis.

Journal Article Qual Life Res · August 2019 PURPOSE: To evaluate the effect of eculizumab on perceived fatigue in patients with anti-acetylcholine receptor antibody-positive, refractory, generalized myasthenia gravis (MG) using the Quality of Life in Neurological Disorders (Neuro-QOL) Fatigue subsca ... Full text Open Access Link to item Cite

Correction to: Eculizumab improves fatigue in refractory generalized myasthenia gravis.

Journal Article Qual Life Res · August 2019 The article "Eculizumab improves fatigue in refractory generalized myasthenia gravis", written by "Henning Andersen, Renato Mantegazza, Jing Jing Wang, Fanny O'Brien, Kaushik Patra, James F. Howard Jr. and The REGAIN Study Group" was originally published e ... Full text Open Access Link to item Cite

Long-term safety and efficacy of eculizumab in generalized myasthenia gravis.

Journal Article Muscle Nerve · July 2019 INTRODUCTION: Eculizumab is effective and well tolerated in patients with antiacetylcholine receptor antibody-positive refractory generalized myasthenia gravis (gMG; REGAIN; NCT01997229). We report an interim analysis of an open-label extension of REGAIN, ... Full text Open Access Link to item Cite

Long-term effect of thymectomy plus prednisone versus prednisone alone in patients with non-thymomatous myasthenia gravis: 2-year extension of the MGTX randomised trial.

Journal Article Lancet Neurol · March 2019 BACKGROUND: The Thymectomy Trial in Non-Thymomatous Myasthenia Gravis Patients Receiving Prednisone (MGTX) showed that thymectomy combined with prednisone was superior to prednisone alone in improving clinical status as measured by the Quantitative Myasthe ... Full text Link to item Cite

Tacrolimus inhibits Th1 and Th17 responses in MuSK-antibody positive myasthenia gravis patients.

Journal Article Exp Neurol · February 2019 Muscle specific tyrosine kinase antibody positive myasthenia gravis (MuSK- MG) is characterized by autoantibodies against the MuSK protein of the neuromuscular junction resulting in weakness of bulbar and proximal muscles. We previously demonstrated that p ... Full text Link to item Cite

Single fiber electromyography.

Journal Article Handb Clin Neurol · 2019 Single fiber electromyography (SFEMG) is a highly selective technique that permits assessment of individual muscle fiber action potentials (MFAPs). This selectivity is achieved with a specialized concentric needle electrode with a 25-μm diameter recording ... Full text Link to item Cite

Clinical neurophysiology of neuromuscular junction disease.

Journal Article Handb Clin Neurol · 2019 The neuromuscular junction (NMJ) is a cholinergic synapse where quantal release of acetylcholine (ACh) from motor nerve terminals generates a local endplate potential (EPP) on the muscle fiber. EPPs that reach threshold depolarize the entire muscle fiber a ... Full text Link to item Cite

Circulating microRNA plasma profile in MuSK+ myasthenia gravis.

Journal Article J Neuroimmunol · December 15, 2018 Muscle-specific tyrosine kinase antibody positive myasthenia gravis (MuSK+ MG) is an immunological subtype with distinctive pathogenic mechanisms and clinical features. The aim of this study was to analyze the circulating plasma microRNA profile of patient ... Full text Link to item Cite

The relationship between deficit in digit span and genotype in nonsense mutation Duchenne muscular dystrophy.

Journal Article Neurology · September 25, 2018 OBJECTIVE: To evaluate the relationship between deficit in digit span and genotype in nonsense mutation (nm) Duchenne muscular dystrophy (DMD) (nmDMD). METHODS: We investigated the relationship between normalized digit-span forward (d-sf) and digit-span ba ... Full text Link to item Cite

Reply.

Journal Article Muscle Nerve · May 2018 Full text Link to item Cite

3,4-diaminopyridine base effectively treats the weakness of Lambert-Eaton myasthenia.

Journal Article Muscle 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 ... Full text Link to item Cite

Reliability of the triple-timed up-and-go test.

Journal Article Muscle 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 ... Full text Link to item Cite

Safety and efficacy of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis (REGAIN): a phase 3, randomised, double-blind, placebo-controlled, multicentre study.

Journal Article Lancet Neurol · December 2017 BACKGROUND: Complement is likely to have a role in refractory generalised myasthenia gravis, but no approved therapies specifically target this system. Results from a phase 2 study suggested that eculizumab, a terminal complement inhibitor, produced clinic ... Full text Link to item Cite

Population Pharmacokinetics/Pharmacodynamics of 3,4-Diaminopyridine Free Base in Patients With Lambert-Eaton Myasthenia.

Conference CPT Pharmacometrics Syst Pharmacol · September 2017 Lambert-Eaton myasthenia (LEM) is a rare autoimmune disorder associated with debilitating muscle weakness. There are limited treatment options and 3,4-diaminopyridine (3,4-DAP) free base is an investigational orphan drug used to treat LEM-related weakness. ... Full text Link to item Cite

Common Entrapment Neuropathies.

Journal Article Continuum (Minneap Minn) · April 2017 PURPOSE OF REVIEW: This article addresses relevant peripheral neuroanatomy, clinical presentations, and diagnostic findings in common entrapment neuropathies involving the median, ulnar, radial, and fibular (peroneal) nerves. RECENT FINDINGS: Entrapment ne ... Full text Link to item Cite

B10 Cell Frequencies and Suppressive Capacity in Myasthenia Gravis Are Associated with Disease Severity.

Journal Article Front Neurol · 2017 Myasthenia gravis (MG) is a T cell-dependent, B cell-mediated disease. The mechanisms for loss of self-tolerance in this disease are not well understood, and recently described regulatory B cell (Breg) subsets have not been thoroughly investigated. B10 cel ... Full text Open Access Link to item Cite

International clinimetric evaluation of the MG-QOL15, resulting in slight revision and subsequent validation of the MG-QOL15r.

Journal Article Muscle 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 ... Full text Link to item Cite

Effect of therapeutic plasma exchange on immunoglobulins in myasthenia gravis.

Journal Article Autoimmunity · November 2016 An integrated understanding of therapeutic plasma exchange (TPE) effects on immunoglobulins, autoantibodies, and natural or acquired (vaccine) protective antibodies in patients with autoimmune myasthenia gravis (MG) is lacking. Prior studies measured TPE e ... Full text Link to item Cite

Efficacy of prednisone for the treatment of ocular myasthenia (EPITOME): A randomized, controlled trial.

Journal Article Muscle 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 ... Full text Link to item Cite

The Neuromuscular Junction and Myasthenia Gravis

Chapter · July 1, 2015 The neuromuscular junction is a specialized region where motor nerves chemically synapse with muscle fibers. Each neuromuscular junction is composed of a motor nerve terminal, synaptic space, and the highly-folded endplate region of the skeletal muscle fib ... Cite

Characterization of B cells in muscle-specific kinase antibody myasthenia gravis.

Journal Article Neurol 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 ... Full text Open Access Link to item Cite

A Double-Blinded, Randomized, Placebo-Controlled Trial to Evaluate Efficacy, Safety, and Tolerability of Single Doses of Tirasemtiv in Patients with Acetylcholine Receptor-Binding Antibody-Positive Myasthenia Gravis.

Journal Article Neurotherapeutics · 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 ... Full text Link to item Cite

Ataluren treatment of patients with nonsense mutation dystrophinopathy.

Journal Article Muscle Nerve · October 2014 INTRODUCTION: Dystrophinopathy is a rare, severe muscle disorder, and nonsense mutations are found in 13% of cases. Ataluren was developed to enable ribosomal readthrough of premature stop codons in nonsense mutation (nm) genetic disorders. METHODS: Random ... Full text Link to item Cite

Characterization of CD4 and CD8 T cell responses in MuSK myasthenia gravis.

Journal Article J 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 ... Full text Open Access Link to item Cite

Is the decremental pattern in Lambert-Eaton syndrome different from that in myasthenia gravis?

Journal Article Clin 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 ... Full text Link to item Cite

Does change in acetylcholine receptor antibody level correlate with clinical change in myasthenia gravis?

Journal Article Muscle 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 ... Full text Link to item Cite

Do acetylcholine receptor and striated muscle antibodies predict the presence of thymoma in patients with myasthenia gravis?

Journal Article Muscle 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 ... Full text Link to item Cite

A randomized, double-blind, placebo-controlled phase II study of eculizumab in patients with refractory generalized myasthenia gravis.

Journal Article Muscle Nerve · July 2013 INTRODUCTION: Complement activation at the neuromuscular junction is a primary cause of acetylcholine receptor loss and failure of neuromuscular transmission in myasthenia gravis (MG). Eculizumab, a humanized monoclonal antibody, blocks the formation of te ... Full text Link to item Cite

Nerve ultrasound in diabetic polyneuropathy: correlation with clinical characteristics and electrodiagnostic testing.

Journal Article Muscle Nerve · March 2013 INTRODUCTION: Diabetic polyneuropathy (DPN) is increasingly prevalent in the USA, but nerve ultrasound (US) findings have not been assessed systematically. Our aim was to establish the sonographic characteristics of lower extremity nerves in DPN and correl ... Full text Link to item Cite

A retrospective study of complications of therapeutic plasma exchange in myasthenia.

Journal Article Muscle 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 ... Full text Link to item Cite

Median nerve ultrasound as a screening tool in carpal tunnel syndrome: correlation of cross-sectional area measures with electrodiagnostic abnormality.

Journal Article Muscle Nerve · December 2012 INTRODUCTION: Sonographically measured median nerve cross-sectional area (CSA) at the wrist is increased in patients with carpal tunnel syndrome (CTS). Ultrasound of the median nerve may be useful in screening for electrodiagnostic (EDx) abnormalities. MET ... Full text Link to item Cite

Evaluation of neuromuscular junction disorders in the electromyography laboratory.

Journal Article Neurol Clin · May 2012 Neuromuscular junction (NMJ) disorders may be demonstrated using repetitive nerve stimulation (RNS) testing and single-fiber electromyography (SFEMG). RNS testing with low frequency stimulation reduces the safety factor of neuromuscular transmission (NMT) ... Full text Link to item Cite

INCREASED OCCURRENCE OF LATE ONSET MYASTHENIA GRAVIS

Conference MUSCLE & NERVE · October 1, 2011 Link to item Cite

Myasthenic syndrome caused by plectinopathy.

Journal Article Neurology · January 25, 2011 BACKGROUND: Plectin crosslinks intermediate filaments to their targets in different tissues. Defects in plectin cause epidermolysis bullosa simplex (EBS), muscular dystrophy (MD), and sometimes pyloric atresia. Association of EBS with a myasthenic syndrome ... Full text Link to item Cite

Core clerkship directors: their current resources and the rewards of the role.

Journal Article Acad Med · April 2010 PURPOSE: To conduct a national multidisciplinary investigation assessing core clinical clerkships and their directors, variances in resources from national guidelines, and the impact of the clerkship director role on faculty members' academic productivity, ... Full text Link to item Cite

Construct and concurrent validation of the MG-QOL15 in the practice setting.

Journal Article Muscle 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 ... Full text Link to item Cite

Myasthenic crisis: smoother sailing ahead.

Journal Article Eur J Neurol · July 2009 Full text Link to item Cite

MuSK-antibody positive myasthenia gravis: questions from the clinic.

Journal Article J 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 ... Full text Link to item Cite

The ultrasonographic wrist-to-forearm median nerve area ratio in carpal tunnel syndrome.

Journal Article Clin 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 ... Full text Link to item Cite

Autoimmune disorders of neuromuscular transmission.

Journal Article Semin Neurol · April 2008 Myasthenia gravis and Lambert-Eaton syndrome are autoimmune disorders of the neuromuscular junction. The most common form of myasthenia gravis is associated with antibodies directed against the acetylcholine receptor on the postsynaptic membrane. In Lamber ... Full text Link to item Cite

The Lambert-Eaton myasthenic syndrome.

Journal Article Handb Clin Neurol · 2008 Full text Link to item Cite

Myasthenia gravis.

Journal Article Orphanet J Rare Dis · November 6, 2007 Myasthenia gravis (MG) is a rare, autoimmune neuromuscular junction disorder. Contemporary prevalence rates approach 1/5,000. MG presents with painless, fluctuating, fatigable weakness involving specific muscle groups. Ocular weakness with asymmetric ptosi ... Full text Open Access Link to item Cite

Needle EMG in certain uncommon clinical contexts.

Journal Article Muscle Nerve · March 2005 It is the position of the AANEM that, based on the literature, there are no contraindications to needle electromyography (EMG) in patients with lymphedema or prosthetic joints. In patients with lymphedema, clinical judgment in each individual circumstance ... Full text Link to item Cite

Autoimmune Myasthenia Gravis: Recommendations for Treatment and Immunologic Modulation.

Journal Article Curr Treat Options Neurol · January 2005 Treatment for myasthenia gravis should be individualized to each patient based on the clinical characteristics of myasthenia including the distribution, duration, and severity of weakness and resulting functional impairment; the risks for treatment complic ... Full text Link to item Cite

Reversible corpus callosum lesion in legionnaires' disease.

Journal Article J Neurol Neurosurg Psychiatry · April 2004 Legionnaires' disease is often associated with neurological findings. Despite such findings, computed tomography and neuropathological investigations are typically normal. This report describes a reversible lesion of the corpus callosum identified on magne ... Full text Link to item Cite

Myasthenia gravis: management of myasthenic crisis and perioperative care.

Journal Article Semin Neurol · March 2004 Myasthenic crisis may be defined as respiratory failure or delayed postoperative extubation for more than 24 hours resulting from myasthenic weakness. Myasthenic crisis results from weakness of upper airway muscles leading to obstruction and aspiration, we ... Full text Link to item Cite

Predict Resident Exam Performance (PREP) study.

Journal Article Neurology · April 22, 2003 Predictive relationships between Resident Inservice Training Examination (RITE) performance and first-attempt success on American Board of Psychiatry and Neurology (ABPN) neurology examinations were assessed in board-eligible residents at the University of ... Full text Link to item Cite

Narrowing in on the causative defect of an intriguing X-linked myopathy with excessive autophagy.

Journal Article Neurology · August 27, 2002 BACKGROUND: X-Linked myopathy with excessive autophagy (XMEA) is a childhood-onset slowly progressive disease of skeletal muscle with no cardiac, nervous system, or other organ involvement. Pathology is distinctive: membrane-bound autophagic vacuoles, mult ... Full text Link to item Cite

Electrophysiological findings in X-linked myopathy with excessive autophagy.

Journal Article Ann Neurol · May 2002 We report electrophysiological features and magnetic resonance imaging muscle findings in 4 patients and 1 female carrier of X-linked myopathy with excessive autophagy. Motor units were polyphasic with high mean amplitude and normal duration. The thigh mus ... Full text Link to item Cite

The prevalence of narcolepsy among Chinese in Hong Kong.

Journal Article Ann Neurol · May 2002 Narcolepsy is a lifelong, crippling sleep disorder. Although the discovery of the hypocretin system has been a breakthough in genetics, the epidemiological aspects of narcolepsy remain elusive. Ethnic predisposition was suggested to partially account for t ... Full text Link to item Cite

Gasoline sniffing multifocal neuropathy.

Journal Article Pediatr Neurol · November 2001 The polyneuropathy caused by chronic gasoline inhalation is reported to be a gradually progressive, symmetric, sensorimotor polyneuropathy. We report unleaded gasoline sniffing by a female 14 years of age that precipitated peripheral neuropathy. In contras ... Full text Link to item Cite

Motor neuron disease and serum monoclonal proteins: poor response to treatment of the paraproteinemia.

Journal Article J Clin Neuromuscul Dis · December 2000 An association between motor neuron disease (MND) and paraproteinemia has been previously reported. In a retrospective study, we found that 13 of 117 (11%) patients with MND who had serum protein electrophoresis studies were found to have a serum M-protem. ... Full text Link to item Cite

Isolated musculocutaneous neuropathy caused by a proximal humeral exostosis.

Journal Article Neurology · January 25, 2000 We report an isolated musculocutaneous neuropathy caused by a proximal humeral osteochondroma that became symptomatic after the patient played recreational basketball. Lesion resection resulted in complete deficit resolution. Mass lesions involving the mus ... Full text Link to item Cite

Myasthenia gravis in the tenth decade.

Journal Article Muscle Nerve · September 1999 Full text Link to item Cite

Clinical and electrophysiological findings in the migrant sensory 'neuritis' of wartenberg.

Journal Article J Clin Neuromuscul Dis · September 1999 Wartenberg's migrant sensory neuritis (WMSN) is an unusual disorder characterized by the occurrence of multiple mononeuropathies involving cutaneous sensory nerves. The duration of the disease is often one of several years, and the overall course is usuall ... Full text Link to item Cite

Neuroendocrine lung tumors and disorders of the neuromuscular junction.

Journal Article Neurology · 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 ... Full text Link to item Cite

Mitochondria in sporadic amyotrophic lateral sclerosis.

Journal Article Exp Neurol · September 1998 Mitochondria are abnormal in persons with amyotrophic lateral sclerosis (ALS) for unknown reasons. We explored whether aberration of mitochondrial DNA (mtDNA) could play a role in this by transferring mitochondrial DNA (mtDNA) from ALS subjects to mtDNA-de ... Full text Link to item Cite

Slow oscillations of cytochrome oxidase redox state and blood volume in unanesthetized cat and rabbit cortex. Interhemispheric synchrony.

Journal Article Adv Exp Med Biol · 1998 The purpose of this study was to determine the frequency characteristics and the degree of interhemispheric synchrony of slow (< 0.5 Hz), spontaneous oscillations of the regional cortical cytochrome oxidase redox state (CYT) and blood volume (CBV) in unane ... Full text Link to item Cite

Interhemispheric synchrony of slow oscillations of cortical blood volume and cytochrome aa3 redox state in unanesthetized rabbits.

Journal Article Brain Res · November 14, 1997 In order to study spontaneous, slow oscillations of regional oxidative metabolism and blood flow in the normal, unanesthetized cortex, adult rabbits were implanted with bilateral cortical windows and electrodes for polysomnography. Relative changes in the ... Full text Link to item Cite

The role of electrodiagnostic testing in carpal tunnel syndrome.

Journal Article Neurosurg Focus · July 15, 1997 Electrodiagnostic testing in patients who have upper-extremity symptoms, which may include carpal tunnel syndrome (CTS), has been the gold standard for diagnosis for many years. Despite their value, these tests are underutilized. The authors examined the u ... Full text Link to item Cite

The Chédiak-Higashi syndrome: CT and MR findings.

Journal Article Pediatr Radiol · 1994 Chédiak-Higashi syndrome (CHS) is a rare autosomal recessive disorder postulated to result from lack of regulation of fusion of the primary lysosomes. In this report we present the MR and CT features of the brain in a patient with known CHS. These findings ... Full text Link to item Cite

Low-frequency oscillations of cortical oxidative metabolism in waking and sleep.

Journal Article J Cereb Blood Flow Metab · April 1988 To study the changes in cortical oxidative metabolism and blood volume during behavioral state transitions, we employed reflectance spectrophotometry of the cortical cytochrome c oxidase (cyt aa3) redox state and blood volume in unanesthetized cats implant ... Full text Link to item Cite

A simplified method for monitoring the cytochrome aa3 redox state in bilateral cortical areas of unanesthetized cats.

Journal Article Brain Res · July 7, 1987 We describe a versatile optical system that enables the simultaneous monitoring of the redox state of cytochrome c oxidase (cytochrome aa3) in two homologous cortical areas under chronically implanted windows in cats. A single light source, broad bandpass ... Full text Link to item Cite