Scholars@Duke
Janice Munn Massey

Janice Munn Massey

Professor of Neurology
Neurology, Neuromuscular Disease
masse005@mc.duke.edu
Duke Box 3403, Durham, NC 27710
1255 Hosp South, Duke South Clini 1L, Room 1255, Durham, NC 27710

Selected Publications

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

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

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

Neuromuscular Disorders and Pregnancy.

Journal Article Continuum (Minneap Minn) · February 1, 2022 PURPOSE OF REVIEW: This article provides an overview of neuromuscular disorders in pregnancy, with a focus on diagnosis and management. RECENT FINDINGS: Neuromuscular disorders with issues that occur in pregnancy include conditions that are acquired (inclu ... 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

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

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

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

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

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

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

Journal Article BMJ 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 ... Full text 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

Minimal manifestation status and prednisone withdrawal in the MGTX trial.

Journal Article Neurology · August 11, 2020 OBJECTIVE: To examine whether sustained minimal manifestation status (MMS) with complete withdrawal of prednisone is better achieved in thymectomized patients with myasthenia gravis (MG). METHODS: This study is a post hoc analysis of data from a randomized ... 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

Mononeuropathies in pregnancy.

Chapter · 2020 While signs and symptoms of peripheral neuropathy may appear to be similar among all patients, further evaluation both at the bedside and beyond demonstrate distinct differences in the pattern of certain neuropathies. A working knowledge of these differenc ... 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

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

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

Developing treatment guidelines for myasthenia gravis.

Journal Article Ann 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 ... 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

QMG and MG-ADL correlations: Study of eculizumab treatment of myasthenia gravis.

Journal Article Muscle Nerve · August 2017 INTRODUCTION: A phase 2 study of eculizumab for treating myasthenia gravis (MG) used the quantitative myasthenia gravis score (QMG) and myasthenia gravis activities of daily living profile (MG-ADL) to evaluate baseline disease severity and treatment respon ... Full text Link to item Cite

Does change in neuromuscular jitter predict or correlate with clinical change in MG?

Journal Article Muscle 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 ... 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

Randomized Trial of Thymectomy in Myasthenia Gravis.

Journal Article N Engl J Med · August 11, 2016 BACKGROUND: Thymectomy has been a mainstay in the treatment of myasthenia gravis, but there is no conclusive evidence of its benefit. We conducted a multicenter, randomized trial comparing thymectomy plus prednisone with prednisone alone. METHODS: We compa ... Full text Link to item Cite

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

Journal Article Neurology · 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 ... 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

Presynaptic Disorders: Lambert-Eaton Myasthenic Syndrome and Botulism.

Journal Article Semin Neurol · August 2015 Lambert-Eaton myasthenic syndrome (LEMS) and botulism are acquired presynaptic nerve terminal disorders of the neuromuscular junction. Lambert-Eaton myasthenic syndrome is an idiopathic or paraneoplastic autoimmune syndrome in which autoantibodies of the P ... Full text Link to item Cite

A genome-wide association study of myasthenia gravis.

Journal Article JAMA 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 ... Full text Link to item 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

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

Pregnancy and myasthenia gravis.

Journal Article Continuum (Minneap Minn) · February 2014 PURPOSE OF REVIEW: Myasthenia gravis (MG) is an acquired autoimmune disorder characterized by fluctuating ocular, limb, or oropharyngeal muscle weakness due to an antibody-mediated attack at the neuromuscular junction. The female incidence of MG peaks in t ... 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

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

Association between performance on Neurology In-Training and Certification Examinations.

Journal Article Neurology · January 8, 2013 OBJECTIVE: This study analyzed the relationship between performance on the American Academy of Neurology Residency In-Service Training Examination (RITE) and subsequent performance on the American Board of Psychiatry and Neurology (ABPN) Certification Exam ... Full text Link to item Cite

2011 Annual report of the American Board of Psychiatry and Neurology, Inc.

Journal Article American Journal of Psychiatry · August 1, 2012 Full text Cite

INCREASED OCCURRENCE OF LATE ONSET MYASTHENIA GRAVIS

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

Reply from the authors

Journal Article Neurology · May 10, 2011 Full text Cite

Trends in American Board of Psychiatry and Neurology specialties and neurologic subspecialties.

Journal Article Neurology · September 21, 2010 OBJECTIVE: To review the current status and recent trends in the American Board of Psychiatry and Neurology (ABPN) specialties and neurologic subspecialties and discuss the implications of those trends for subspecialty viability. METHODS: Data on numbers o ... 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

Cervical dystonia

Journal Article Future Neurology · December 1, 2009 Cervical dystonia is a common disorder with significant associated morbidity. Although limited benefit can be derived from oral pharmacologic agents, the advent of botulinum neurotoxin (BoNT) injection has provided a valuable tool in the treatment of this ... Full text Cite

Long-term treatment with botulinum toxin type A in cervical dystonia has low immunogenicity by mouse protection assay.

Journal Article Mov Disord · July 30, 2008 To evaluate the immunogenicity of botulinum toxin type A (BoNTA; BOTOX) in cervical dystonia (CD). Subjects diagnosed with CD for > or =1 year and previously naïve to BoNTs were treated with BoNTA in a prospective, open-label, multicenter study. Serum samp ... 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

Clinical features of myasthenia gravis.

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

Certification in neuromuscular medicine: a new neurologic subspecialty.

Journal Article Neurology · April 3, 2007 Featured Publication Information is provided concerning the new subspecialty certificate in neuromuscular medicine of the American Board of Psychiatry and Neurology and the eligibility requirements for such certification of practicing neurologists and child neurologists. The A ... Full text Link to item Cite

Certification in neuromuscular medicine: a new neurologic subspecialty.

Journal Article Muscle Nerve · April 2007 Information is provided concerning the new subspecialty certificate in neuromuscular medicine of the American Board of Psychiatry and Neurology and the eligibility requirements for such certification of practicing neurologists and child neurologists. The A ... Full text Link to item Cite

Peripheral neuropathy in Krabbe disease: electrodiagnostic findings.

Journal Article Neurology · July 25, 2006 Featured Publication 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 ... Full text Link to item Cite

Peripheral neuropathy in Krabbe disease: effect of hematopoietic stem cell transplantation.

Journal Article Neurology · July 25, 2006 Featured Publication 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 ... Full text Link to item Cite

Electromyographic assessment of peripheral nerve diseases: An overview

Journal Article Handbook of Clinical Neurophysiology · January 1, 2006 Full text Cite

MuSK-antibody positive myasthenia gravis: clinical and electrodiagnostic patterns.

Journal Article Clin Neurophysiol · September 2005 Featured Publication 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 ... Full text Link to item Cite

Autoimmune Myasthenia Gravis: Recommendations for Treatment and Immunologic Modulation.

Journal Article Curr Treat Options Neurol · January 2005 Featured Publication 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

Myasthenia gravis and pregnancy.

Journal Article Neurol Clin · November 2004 Featured Publication Treatment considerations for women who have MG and are of childbearing age are complicated. When possible, before pregnancy, establishing a plan for therapy is ideal, recognizing the potential concerns for the patient and the fetus. Decisions about treatme ... Full text Link to item Cite

The management of myasthenia gravis in pregnancy.

Journal Article Semin Neurol · March 2004 As with several autoimmune diseases, myasthenia gravis (MG) occurs frequently in young women in their childbearing years. The treatment of MG in women therefore poses unique and challenging issues to neurologists, obstetricians, and neonatologists as the s ... Full text Link to item Cite

Severity and impact of xerostomia in patients treated with botulinum toxin type b for cervical dystonia: Observations on the quality of life of patients with xerostomia.

Journal Article Curr Ther Res Clin Exp · March 2004 BACKGROUND: Although dry mouth (xerostomia) has been reported with botulinum toxin type B used as treatment for cervical dystonia, the impact of this adverse effect (AE) on patients' activities of daily living (ADLs) has not been assessed. t OBJECTIVE: The ... Full text Link to item Cite

Preface

Journal Article Supplements to Clinical Neurophysiology · January 1, 2004 Full text Cite

Mycophenolate mofetil for myasthenia gravis: an analysis of efficacy, safety, and tolerability.

Journal Article Neurology · November 25, 2003 Featured Publication 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 ... Full text Link to item Cite

Clinical aspects of MuSK antibody positive seronegative MG.

Journal Article Neurology · June 24, 2003 Featured Publication 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 ... Full text Link to item Cite

Can we eliminate placebo in ALS clinical trials?

Journal Article Amyotroph Lateral Scler Other Motor Neuron Disord · April 2003 BACKGROUND: Previous studies concluded that the decline in strength in patients with amyotrophic lateral sclerosis (ALS) is a linear function. If so, a patient's natural history might serve as the control, instead of placebo, in a clinical trial. METHODS: ... Full text Link to item Cite

Effect of thymectomy on human peripheral blood T cell pools in myasthenia gravis.

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

Mycophenolate mofetil for myasthenia gravis: an open-label pilot study.

Journal Article Neurology · January 9, 2001 Featured Publication 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 ... Full text Link to item Cite

Predictive value of single-fiber electromyography in the extensor digitorum communis muscle of patients with ocular myasthenia gravis: a retrospective study.

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

Retrospective analysis of the use of cyclosporine in myasthenia gravis.

Journal Article Neurology · August 8, 2000 Featured Publication 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 ... Full text Link to item Cite

Lambert-Eaton myasthenic syndrome: electrodiagnostic findings and response to treatment.

Journal Article Neurology · June 13, 2000 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 ... Full text Link to item Cite

Leukemia inhibitory factor, oncostatin M, IL-6, and stem cell factor mRNA expression in human thymus increases with age and is associated with thymic atrophy.

Journal Article J Immunol · February 15, 2000 The roles that thymus cytokines might play in regulating thymic atrophy are not known. Reversing thymic atrophy is important for immune reconstitution in adults. We have studied cytokine mRNA steady-state levels in 45 normal human (aged 3 days to 78 years) ... Full text Link to item Cite

A randomized trial of 3,4-diaminopyridine in Lambert-Eaton myasthenic syndrome.

Journal Article Neurology · February 8, 2000 Featured Publication 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 ... Full text Link to item Cite

Domestic violence in neurologic practice.

Journal Article Arch Neurol · June 1999 Full text Link to item Cite

Changes in thymic function with age and during the treatment of HIV infection.

Journal Article Nature · December 17, 1998 The thymus represents the major site of the production and generation of T cells expressing alphabeta-type T-cell antigen receptors. Age-related involution may affect the ability of the thymus to reconstitute T cells expressing CD4 cell-surface antigens th ... Full text Link to item Cite

Botulinum toxin type B: a double-blind, placebo-controlled, safety and efficacy study in cervical dystonia.

Journal Article Neurology · September 1997 We enrolled and treated 122 patients with idiopathic cervical dystonia in a double-blind, placebo-controlled safety and efficacy study of botulinum toxin type B (BotB). Both A-responsive and A-resistant patients were enrolled. Patients received intramuscul ... Full text Link to item Cite

Acquired myasthenia gravis.

Journal Article Neurol Clin · August 1997 Featured Publication Myasthenia gravis, an antibody-mediated disorder of neuromuscular transmission that produces clinical weakness, may be ocular or generalized. Clinical diagnostic evaluation may be supplemented by electrophysiologic studies and antibody testing. Therapeutic ... Full text Link to item Cite

Seronegative myasthenia gravis

Journal Article Neurology · April 1, 1997 Cite

Treatment of acquired myasthenia gravis

Journal Article Neurology · January 1, 1997 Full text Cite

A placebo-controlled trial of recombinant human ciliary neurotrophic (rhCNTF) factor in amyotrophic lateral sclerosis. rhCNTF ALS Study Group.

Journal Article Ann Neurol · February 1996 Preclinical investigations indicated that recombinant human ciliary neurotrophic factor (rhCNTF) may have potential as therapy for amyotrophic lateral sclerosis (ALS). We evaluated the safety and efficacy of rhCNTF in a prospective, double-blind, placebo-c ... Full text Link to item Cite

Treatment of spasmodic torticollis with intramuscular phenol injection.

Journal Article J Neurol Neurosurg Psychiatry · February 1995 Full text Link to item Cite

Race, sex, and puberty influence onset, severity, and outcome in juvenile myasthenia gravis.

Journal Article Neurology · July 1994 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 ... Full text Link to item Cite

The electrodiagnosis of myasthenia gravis and the Lambert-Eaton myasthenic syndrome.

Journal Article Neurol Clin · May 1994 Featured Publication 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 ... Link to item Cite

Single fiber EMG and repetitive stimulation of the same muscle in myasthenia gravis.

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

Acetylcholine receptor antibodies in juvenile myasthenia gravis.

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

Single fiber electromyography in myasthenia gravis during pregnancy.

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

Botulinum toxin therapy for neurologic disorders.

Journal Article Postgrad Med · May 1, 1992 In the last 20 years, the therapeutic uses of botulinum toxin, a potent neurotoxin, have been investigated. The agent produces chemical denervation of muscle, thereby causing atrophy and weakness. Studies have shown that injection of this agent is an effec ... Full text Link to item Cite

Myotonic dystrophy.

Journal Article Semin Neurol · September 1991 Featured Publication Full text Link to item Cite

Single-fiber EMG demonstrates reinnervation dynamics after nerve injury.

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

Extubation after transsternal thymectomy for myasthenia gravis: a prospective analysis.

Journal Article South Med J · June 1991 Recommendations concerning postoperative extubation after thymectomy for myasthenia gravis are presently based upon retrospective chart reviews. We present the results of a prospective investigation of time to extubation after thymectomy for 14 patients ov ... Full text Link to item Cite

Electromyography in disorders of neuromuscular transmission.

Journal Article Semin Neurol · March 1990 Featured Publication Electrophysiologic tests in neuromuscular transmission disorders are valuable aids in the clinical assessment of patients with suspected disease. The techniques of RNS and SFEMG are most reliable when the electromyographer is aware of their pitfalls and wh ... Full text Link to item Cite

Animal production industry in the year 2000 A.D.

Journal Article J Reprod Fertil Suppl · 1990 One can easily envision that, in the very near future, all bulls being progeny tested will be screened for genomic markers linked to economic traits and females may also be screened if enrolled as donors in a nuclear transfer programme. The concept of prod ... Link to item Cite

Vascular headache from extracranial venous malformation.

Journal Article Headache · October 1989 Featured Publication Headache associated with intracranial venous anomalies is well described but headache associated with extracranial venous malformation previously has not been recognized. A case of extracranial venous malformation producing vascular headache is presented. ... Full text Link to item Cite

QUANTITATIVE EMG MYOTONIC-DYSTROPHY

Journal Article MUSCLE & NERVE · September 1, 1989 Link to item Cite

Moyamoya disease associated with polycystic kidney disease and eosinophilic granuloma.

Journal Article Stroke · August 1989 Featured Publication Moyamoya disease has been associated with renal artery stenosis, cerebral hemorrhage, and multiple cranial traumas. We report a unique case of moyamoya disease associated with polycystic kidney disease and eosinophilic granuloma. Although the etiology of m ... Full text Link to item Cite

Reproductive performance in mares subjected to examination by diagnostic ultrasound.

Journal Article Theriogenology · July 1989 Mares were subjected to frequent examination by diagnostic ultrasound and data were compiled with respect to reproductive efficiency. The data were collected over a 3-yr period on 1032 light horse mares. The cummulative pregnancy rate at 35 d post-ovulatio ... Full text Link to item Cite

Management of facial spasm with Clostridium botulinum toxin.

Journal Article Arch Otolaryngol Head Neck Surg · July 1989 Featured Publication Full text Link to item Cite

The effect of cholinesterase inhibitors of SFEMG in myasthenia gravis.

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

Evaluation of an automatic method of measuring features of motor unit action potentials.

Journal Article Muscle Nerve · February 1989 Featured Publication 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 ... Full text Link to item Cite

Automatic analysis of the electromyographic interference pattern using the turns: amplitude ratio.

Journal Article Electroencephalogr Clin Neurophysiol · December 1988 Featured Publication 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 ... Full text Link to item Cite

SINGLE FIBER ELECTROMYOGRAPHY IN MYASTHENIA-GRAVIS DURING PREGNANCY

Journal Article ELECTROENCEPHALOGRAPHY AND CLINICAL NEUROPHYSIOLOGY · October 1, 1988 Link to item Cite

SERIAL QUANTITATIVE EMG STUDIES IN MOTOR NEURON DISEASE

Journal Article ELECTROENCEPHALOGRAPHY AND CLINICAL NEUROPHYSIOLOGY · October 1, 1988 Link to item Cite

QUANTITATIVE EMG IN THE POSTPOLIO MUSCULAR-ATROPHY SYNDROME

Journal Article MUSCLE & NERVE · September 1, 1988 Link to item Cite

AN AUTOMATED-METHOD OF MEASURING MOTOR UNIT ACTION-POTENTIAL FEATURES - FINDINGS IN NERVE AND MUSCLE DISEASES

Journal Article ELECTROENCEPHALOGRAPHY AND CLINICAL NEUROPHYSIOLOGY · November 1, 1987 Link to item Cite

THE EFFECT OF CHOLINESTERASE-INHIBITORS ON SINGLE FIBER ELECTROMYOGRAPHY IN MYASTHENIA-GRAVIS

Journal Article ELECTROENCEPHALOGRAPHY AND CLINICAL NEUROPHYSIOLOGY · November 1, 1987 Link to item Cite

SERIAL QUANTITATIVE EMG STUDIES IN MOTOR-NEURON DISEASE

Journal Article MUSCLE & NERVE · September 1, 1987 Link to item Cite

Single fiber electromyography in chronic progressive external ophthalmoplegia.

Journal Article Muscle Nerve · May 1987 Featured Publication 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 ... Full text Link to item Cite

A boy (a bleeder) and a bloody revolution.

Journal Article N C Med J · March 1987 Link to item Cite

Quantitative electromyography after poliomyelitis.

Journal Article Birth Defects Orig Artic Ser · 1987 Featured Publication Link to item Cite

Facial diplegia due to amyloidosis.

Journal Article South Med J · November 1986 In a 61-year-old man with facial diplegia and new onset of congestive heart failure (CHF), electroneuromyography indicated diffuse axonal degeneration, but sural nerve biopsy showed mild changes and no amyloid. Four months later, after the onset of diplopi ... Full text Link to item Cite

Forearm neuropathy and pruritus.

Journal Article South Med J · October 1986 Five adult patients (four of them men) had episodic brachioradial pruritus associated with forearm paresthesia and hypalgesia. No cervical, shoulder, or forearm trauma was known. Onset was variable, but two had had the condition for more than ten years. In ... Full text Link to item Cite

SINGLE FIBER ELECTROMYOGRAPHY IN CHRONIC PROGRESSIVE EXTERNAL OPHTHALMOPLEGIA

Journal Article ELECTROENCEPHALOGRAPHY AND CLINICAL NEUROPHYSIOLOGY · September 1, 1986 Link to item Cite

USE OF SINGLE FIBER ELECTROMYOGRAPHY (EMG) TO PREDICT THE COURSE OF OCULAR MYASTHENIA

Journal Article ELECTROENCEPHALOGRAPHY AND CLINICAL NEUROPHYSIOLOGY · September 1, 1986 Link to item Cite

Forearm neuropathy and pruritus

Journal Article Southern Medical Journal · January 1, 1986 Five adult patients (four of them men) had episodic brachioradial pruritus associated with forearm paresthesia and hypalgesia. No cervical, shoulder, or forearm trauma was known. Onset was variable, but two had had the condition for more than ten years. In ... Cite

Ergot, the "jerks," and revivals.

Journal Article Clin Neuropharmacol · 1984 Epidemics of epilepsy, a form of mass hysteria, were known in Eastern and Western cultures in the 17th and 18th centuries. A unique situation in the United States during the 19th centurey was the frontier religious movement, the setting in which the "jerks ... Full text Link to item Cite

Biliary excretion of glycolipid in induced or inherited glucosylceramide lipidosis.

Journal Article Biochim Biophys Acta · September 24, 1981 Metabolically inert L-[1-14C]glucosylceramide is stored predominantly in the liver after intravenous administration to mice. The half-time of this glycolipid analogue in the liver is 3.5 days and its clearance occurs predominantly via the bile. Within the ... Full text Link to item Cite

Migraine.

Journal Article South Med J · February 1980 Full text Link to item Cite

L-glucosylceramide: synthesis, properties, and resistance to catabolism by glucocerebrosidase in vitro.

Journal Article Proc Natl Acad Sci U S A · July 1979 Procedures for the synthesis and radioactive labeling of L-glucosylceramide are described. This compound is a stereoisomeric analogue of D-glucosylceramide which occurs in nature and accumulates in pathological quantity in the organs and tissues of patient ... Full text Link to item Cite

Crossed leg palsy in the elderly.

Journal Article South Med J · September 1978 Full text Link to item Cite