Scholars@Duke
Lisa Deneen Hobson-Webb

Lisa Deneen Hobson-Webb

Professor of Neurology
Neurology, Neuromuscular Disease
lisa.hobsonwebb@duke.edu
Duke Box 3403, Durham, NC 27710
Dept of Medicine, Division of Neurology, Durham, NC 27710

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

Rotational 3D shear wave elasticity imaging: Effect of knee flexion on 3D shear wave propagation in in vivo vastus lateralis.

Journal Article J Mech Behav Biomed Mater · February 2024 Skeletal muscle is a complex tissue, exhibiting not only direction-dependent material properties (commonly modeled as a transversely isotropic material), but also changes in observed material properties due to factors such as contraction and passive stretc ... Full text Link to item Cite

Neuromuscular ultrasound standardized scanning techniques and protocols: Expert panel recommendations.

Journal Article Muscle Nerve · October 2023 Neuromuscular ultrasound has become an integral part of the diagnostic workup of neuromuscular disorders at many centers. Despite its growing utility, uniform standard scanning techniques do not currently exist. Scanning approaches for similar diseases var ... 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

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

Ultrasound-guided-electromyography in plegic muscle: Usefulness of nerve stimulation.

Journal Article Muscle Nerve · March 2023 INTRODUCTION/AIMS: In traumatic nerve lesions (TNLs), motor unit potentials (MUPs) may be difficult to detect in early injury. Ultrasound-guided electromyography (US-EMG) can aid in identifying areas of muscle activation, but its sensitivity can be improve ... Full text Link to item Cite

Carpal tunnel syndrome: updated evidence and new questions.

Journal Article Lancet Neurol · March 2023 Carpal tunnel syndrome is the most common entrapment neuropathy, affecting quality of life for many people. Although it is a well recognised condition, new insights into epidemiology, diagnosis, and treatment have emerged in the past 6 years. The availabil ... Full text Link to item Cite

Repeatability of Rotational 3-D Shear Wave Elasticity Imaging Measurements in Skeletal Muscle.

Journal Article Ultrasound Med Biol · March 2023 Shear wave elasticity imaging (SWEI) usually assumes an isotropic material; however, skeletal muscle is typically modeled as a transversely isotropic material with independent shear wave speeds in the directions along and across the muscle fibers. To captu ... Full text Link to item Cite

A scoping review of methods used in musculoskeletal soft tissue and nerve shear wave elastography studies.

Journal Article Clin Neurophysiol · August 2022 This scoping review of shear wave elastography (SWE) articles in musculoskeletal soft tissue and nerve research demonstrates methodological heterogeneity resulting from a lack of standardized data collection and reporting requirements. Seven literature dat ... Full text Link to item Cite

Classical Complement Pathway Inhibition in a "Human-On-A-Chip" Model of Autoimmune Demyelinating Neuropathies.

Journal Article Adv Ther (Weinh) · June 2022 Chronic autoimmune demyelinating neuropathies are a group of rare neuromuscular disorders with complex, poorly characterized etiology. Here we describe a phenotypic, human-on-a-chip (HoaC) electrical conduction model of two rare autoimmune demyelinating ne ... Full text Link to item Cite

Expert consensus on the combined investigation of carpal tunnel syndrome with electrodiagnostic tests and neuromuscular ultrasound.

Journal Article Clin Neurophysiol · March 2022 Expert consensus was sought to guide clinicians on the use of electrodiagnostic tests (EDX) and neuromuscular ultrasound (NMUS) in the investigation of suspected carpal tunnel syndrome (CTS). Consensus was achieved using the Delphi method via three consecu ... Full text Link to item Cite

Full Characterization of in vivo Muscle as an Elastic, Incompressible, Transversely Isotropic Material Using Ultrasonic Rotational 3D Shear Wave Elasticity Imaging.

Journal Article IEEE Trans Med Imaging · January 2022 Using a 3D rotational shear wave elasticity imaging (SWEI) setup, 3D shear wave data were acquired in the vastus lateralis of a healthy volunteer. The innate tilt between the transducer face and the muscle fibers results in the excitation of multiple shear ... Full text Link to item Cite

An Open-Source Radon-Transform Shear Wave Speed Estimator with Masking Functionality to Isolate Different Shear-Wave Modes

Conference IEEE International Ultrasonics Symposium, IUS · January 1, 2022 The Radon Transform (RT) approach is a common method used to estimate shear wave trajectory and speed in ultrasound shear wave elasticity imaging (SWEI). The RT calculates the sum of 2D spatiotemporal data amplitude under each potential linear trajectory t ... Full text 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

Expert consensus on the combined investigation of ulnar neuropathy at the elbow using electrodiagnostic tests and nerve ultrasound.

Journal Article Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology · September 2021 The addition of ultrasound (US) to electrodiagnostic (EDX) tests can significantly enhance the accuracy of testing for ulnar neuropathy at the elbow (UNE). We aimed to obtain expert consensus to guide clinicians on the combined use of EDX and US in UNE inv ... Full text Cite

Quantitative muscle ultrasound and electrical impedance myography in late onset Pompe disease: A pilot study of reliability, longitudinal change and correlation with function.

Journal Article Mol Genet Metab Rep · September 2021 BACKGROUND/OBJECTIVES: Late-onset Pompe disease (LOPD) is slowly progressive, making it difficult to assess clinical change and response to interventions. In this study, quantitative muscle ultrasonography (QMUS) and electrical impedance myography (EIM) we ... Full text Link to item Cite

Tongue weakness and atrophy differentiates late-onset Pompe disease from other forms of acquired/hereditary myopathy.

Journal Article Mol Genet Metab · July 2021 Late-onset Pompe disease (LOPD) is an inherited autosomal recessive progressive metabolic myopathy that presents in the first year of life to adulthood. Clinical presentation is heterogeneous, differential diagnosis is challenging, and diagnostic delay is ... Full text Open Access Link to item Cite

Neuromuscular ultrasound competency assessment: Consensus-based survey.

Journal Article Muscle Nerve · May 2021 Neuromuscular ultrasound is a rapidly evolving specialty with direct application for patient care. Competency assessment is an essential standard needed to ensure quality for practitioners, particularly for those newly acquiring skills with the technique. ... Full text Link to item Cite

The Role of Imaging for Disorders of Peripheral Nerve.

Journal Article Clin Geriatr Med · May 2021 Peripheral nerve imaging is a helpful and sometimes essential adjunct to clinical history, physical examination, and electrodiagnostic studies. Advances in imaging technology have allowed the visualization of nerve structures and their surrounding tissues. ... 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

Group Shear Wave Speed Viscoelastic Analysis using 3D Rotational Volumetric Shear Wave Imaging in Relaxed and Contracted in vivo Muscle

Conference IEEE International Ultrasonics Symposium, IUS · January 1, 2021 To characterize muscle tissue as a viscoelastic transversely isotropic material, we use rotational volumetric shear wave elasticity imaging (SWEI) and group speed viscoelastic analysis. We performed these measurements in vivo in the vastus lateralis of two ... Full text Cite

Quantification of Skeletal Muscle Fiber Orientation in 3D Ultrasound B-Modes

Conference IEEE International Ultrasonics Symposium, IUS · January 1, 2021 Skeletal muscle exhibits transverse isotropy with a symmetry axis in the muscle fiber direction. Characterization of muscle mechanical properties with ultrasound shear wave elasticity imaging requires an accurate measurement of the 3D muscle fiber orientat ... Full text Cite

Factors Affecting in vivo SH and SV Mode Wave Propagation in vastus lateralis Muscle at Varying Knee Flexion Angles Using Ultrasonic Rotational 3D SWEI

Conference IEEE International Ultrasonics Symposium, IUS · January 1, 2021 We previously have demonstrated the use of ultrasonic rotational 3D SWEI to measure all three mechanical properties necessary to fully characterize the vastus lateralis muscle in vivo as an incompressible transversely isotropic material: transverse shear m ... Full text Cite

Respiratory muscle training in late-onset Pompe disease: Results of a sham-controlled clinical trial.

Journal Article Neuromuscul Disord · November 2020 To address progressive respiratory muscle weakness in late-onset Pompe disease (LOPD), we developed a 12-week respiratory muscle training (RMT) program. In this exploratory, double-blind, randomized control trial, 22 adults with LOPD were randomized to RMT ... Full text Open Access Link to item Cite

Viscoelastic Characterization in Muscle using Group Speed Analysis and Volumetric Shear Wave Elasticity Imaging

Conference IEEE International Ultrasonics Symposium, IUS · September 7, 2020 Shear wave elasticity imaging (SWEI) typically assumes a homogeneous, isotropic, elastic material to relate shear wave speed (SWS) to stiffness; however muscle is anisotropic and viscoelastic (VE). To characterize this complex tissue, we combine 3D SWEI (a ... Full text Cite

Demonstration of Complex Shear Wave Patterns in Skeletal Muscle in vivo Using 3D SWEI

Conference IEEE International Ultrasonics Symposium, IUS · September 7, 2020 Shear wave elastography imaging (SWEI) of skeletal muscle has previously been used to identify speeds along and across the fibers, with limited success and low repeatability. In this work we show how a tilted configuration can be used to fully characterize ... Full text Cite

Placebo effect in chronic inflammatory demyelinating polyneuropathy: The PATH study and a systematic review.

Journal Article J Peripher Nerv Syst · September 2020 The Polyneuropathy And Treatment with Hizentra (PATH) study required subjects with chronic inflammatory demyelinating polyneuropathy (CIDP) to show dependency on immunoglobulin G (IgG) and then be restabilized on IgG before being randomized to placebo or o ... 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

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

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

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

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

Journal Article Clin 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 ... Full text 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

Emerging technologies in neuromuscular ultrasound.

Journal Article Muscle Nerve · June 2020 Neuromuscular ultrasound is an accepted and valuable element in the evaluation of peripheral nerve and muscle disease. However, ultrasound has several limitations to consider, including operator dependency and lack of a viable contrast agent. Fortunately, ... Full text Link to item Cite

On the Challenges Associated with Obtaining Reproducible Measurements Using SWEI in the Median Nerve.

Journal Article Ultrasound Med Biol · May 2020 This work discusses challenges we have encountered in acquiring reproducible measurements of shear wave speed (SWS) in the median nerve and suggests methods for improving reproducibility. First, procedural acquisition challenges are described, including ne ... Full text 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

Guidelines for neuromuscular ultrasound training.

Journal Article Muscle Nerve · October 2019 Neuromuscular ultrasound has become an essential tool in the diagnostic evaluation of various neuromuscular disorders, and, as such, there is growing interest in neuromuscular ultrasound training. Effective training is critical in mastering this modality. ... 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

ULTRASOUND IN THE DIAGNOSIS AND MONITORING OF AMYOTROPHIC LATERAL SCLEROSIS: A REVIEW.

Journal Article Muscle Nerve · August 2019 Neuromuscular ultrasound is complementary to electrodiagnostic (EDx) testing and is useful in enhancing the diagnosis of mononeuropathies, peripheral nerve trauma, and demyelinating polyneuropathies. There is increasing interest in using ultrasound both to ... 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

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Journal Article Mol Genet Metab · August 2019 INTRODUCTION:: Morbidity and mortality in adults with late-onset Pompe disease (LOPD) results primarily from persistent progressive respiratory muscle weakness despite treatment with enzyme replacement therapy (ERT). To address this need, we have developed ... 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

Thymectomy may not be associated with clinical improvement in MuSK myasthenia gravis.

Journal Article Muscle Nerve · April 2019 INTRODUCTION: A randomized trial demonstrated benefit from thymectomy in nonthymomatous acetylcholine receptor (AChR)-antibody positive myasthenia gravis (MG). Uncontrolled observational and histologic studies suggest thymectomy may not be efficacious in a ... Full text Link to item Cite

Efficacy and safety of IVIG in CIDP: Combined data of the PRIMA and PATH studies.

Journal Article J Peripher Nerv Syst · March 2019 Intravenous immunoglobulin (IVIG) is a potential therapy for chronic inflammatory demyelinating polyneuropathy (CIDP). To investigate the efficacy and safety of the IVIG IgPro10 (Privigen) for treatment of CIDP, results from Privigen Impact on Mobility and ... Full text Link to item Cite

Restabilization treatment after intravenous immunoglobulin withdrawal in chronic inflammatory demyelinating polyneuropathy: Results from the pre-randomization phase of the Polyneuropathy And Treatment with Hizentra study.

Journal Article J Peripher Nerv Syst · March 2019 In patients with chronic inflammatory demyelinating polyneuropathy (CIDP), intravenous immunoglobulin (IVIG) is recommended to be periodically reduced to assess the need for ongoing therapy. However, little is known about the effectiveness of restabilizati ... 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

Neuromuscular ultrasound in clinical practice: A review.

Journal Article Clin Neurophysiol Pract · 2019 Neuromuscular ultrasound (NMUS) is becoming a standard element in the evaluation of peripheral nerve and muscle disease. When obtained simultaneously to electrodiagnostic studies, it provides dynamic, structural information that can refine a diagnosis or i ... Full text Link to item Cite

Point of Care Quantitative Assessment of Muscle Health in Older Individuals: An Investigation of Quantitative Muscle Ultrasound and Electrical Impedance Myography Techniques.

Journal Article Geriatrics (Basel) · December 16, 2018 Background: Muscle health is recognized for its critical role in the functionality and well-being of older adults. Readily accessible, reliable, and inexpensive methods of measuring muscle health are needed to advance research and clinical care. Methods: I ... 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

Indications for neuromuscular ultrasound: Expert opinion and review of the literature.

Journal Article Clin Neurophysiol · December 2018 Over the last two decades, dozens of applications have emerged for ultrasonography in neuromuscular disorders. We wanted to measure its impact on practice in laboratories where the technique is in frequent use. After identifying experts in neuromuscular ul ... Full text Link to item Cite

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

Validation of a simple disease-specific, quality-of-life measure for diabetic polyneuropathy: CAPPRI.

Journal Article Neurology · June 5, 2018 OBJECTIVE: We studied the performance of a 15-item, health-related quality-of-life polyneuropathy scale in the clinic setting in patients with diabetic distal sensorimotor polyneuropathy (DSPN). METHODS: Patients with DSPN from 11 academic sites completed ... Full text Link to item Cite

Neuroimaging findings in infantile Pompe patients treated with enzyme replacement therapy.

Journal Article Mol Genet Metab · February 2018 BACKGROUND: Recombinant human acid α-glucosidase (rhGAA) enzyme replacement therapy (ERT) has prolonged survival in infantile Pompe disease (IPD), but has unmasked central nervous system (CNS) changes. METHODS: Brain imaging, consisting of computed tomogra ... Full text Link to item Cite

The extrapolated reference values procedure: Theory, algorithm, and results in patients and control subjects.

Journal Article Muscle 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 ... 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

Ultrasound Imaging of Median Nerve Conduit in a Patient With Persistent Median Nerve Symptoms.

Journal Article J Clin Neurophysiol · January 2018 Peripheral nerve injury can be reconstructed using composite polymer nerve autografts, but the sonographic appearance of nerve wraps and conduits have not been well documented. This case report describes the sonographic findings in a 42-year-old woman with ... Full text Link to item Cite

Subcutaneous immunoglobulin for maintenance treatment in chronic inflammatory demyelinating polyneuropathy (PATH): a randomised, double-blind, placebo-controlled, phase 3 trial.

Journal Article Lancet Neurol · January 2018 BACKGROUND: Approximately two-thirds of patients with chronic inflammatory demyelinating polyneuropathy (CIDP) need long-term intravenous immunoglobulin. Subcutaneous immunoglobulin (SCIg) is an alternative option for immunoglobulin delivery, but has not p ... 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

Rituximab as treatment for anti-MuSK myasthenia gravis: Multicenter blinded prospective review.

Journal Article Neurology · September 5, 2017 OBJECTIVE: To evaluate the efficacy of rituximab in treatment of anti-muscle-specific kinase (MuSK) myasthenia gravis (MG). METHODS: This was a multicenter, blinded, prospective review, comparing anti-MuSK-positive patients with MG treated with rituximab t ... 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

Advancing neuromuscular ultrasound through research: Finding common sound.

Journal Article Muscle Nerve · September 2017 INTRODUCTION: Neuromuscular ultrasound (NMUS) is an accepted, but underutilized, diagnostic tool in the evaluation of neuromuscular disorders. The lack of uniform high-quality standards in published research and in clinical practice has been a limitation t ... 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

Is carpal tunnel syndrome present in acute stroke patients? An investigative study using clinical and imaging screening tools.

Journal Article J Clin Neurosci · May 2017 Carpal tunnel syndrome (CTS) is known to develop post-stroke. Median nerve ultrasound (US) is an inexpensive, effective means of screening. In this prospective feasibility study, we compared the ability of the physical exam, the Boston Carpal Tunnel Questi ... 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

The emerging phenotype of late-onset Pompe disease: A systematic literature review.

Journal Article Mol Genet Metab · March 2017 BACKGROUND: Pompe disease is an autosomal recessive disorder caused by deficiency of the lysosomal glycogen-hydrolyzing enzyme acid α-glucosidase (GAA). The adult-onset form, late-onset Pompe disease (LOPD), has been characterized by glycogen accumulation ... Full text Open Access 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

Standard median nerve ultrasound in carpal tunnel syndrome: A retrospective review of 1,021 cases.

Journal Article Clin Neurophysiol Pract · 2017 OBJECTIVE: Carpal tunnel syndrome (CTS) is diagnosed with electrodiagnostic (EDx) studies. Investigations have examined US cross sectional-area (CSA) and wrist to forearm ratio (WFR) cut-offs for screening EDx abnormalities in patients with suspected CTS. ... Full text 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

Therapeutic Implications of Peripheral Nerve Hyperexcitability in Muscle Cramping: A Retrospective Review.

Journal Article J Clin Neurophysiol · December 2016 PURPOSE: Muscle cramping due to peripheral nerve hyperexcitability (PNH) is poorly characterized. This retrospective study examines the prevalence of PNH and response to treatment. METHODS: The Duke EMG Database was queried to identify patients with muscle ... Full text Link to item Cite

Carpal tunnel syndrome: clinical features, diagnosis, and management.

Journal Article Lancet Neurol · November 2016 Carpal tunnel syndrome is the most common peripheral nerve entrapment syndrome worldwide. The clinical symptoms and physical examination findings in patients with this syndrome are recognised widely and various treatments exist, including non-surgical and ... Full text Link to item Cite

Ultrasound in EMG-Guided Biopsies: A Prospective, Randomized Pilot Trial.

Journal Article Muscle Nerve · October 2016 INTRODUCTION: At our institution, core muscle biopsies are performed on muscles selected using electromyography (EMG). Ultrasound (US) guidance is not used routinely. The aim of this study was to determine if US guidance of EMG selected muscles would incre ... Full text Link to item Cite

Construction and validation of the chronic acquired polyneuropathy patient-reported index (CAP-PRI): A disease-specific, health-related quality-of-life instrument.

Journal Article Muscle Nerve · June 2016 INTRODUCTION: Generic health-related quality-of-life (HRQOL) patient-reported outcome measures have been used in patients with chronic immune-mediated polyneuropathies. We have created a disease-specific HRQOL instrument. METHODS: The chronic acquired poly ... Full text Link to item Cite

Ultrasound of Focal Neuropathies.

Journal Article J Clin Neurophysiol · April 2016 Focal neuropathies represent the most common indication for referral to electrodiagnostic laboratories. The etiologies range from chronic compression, as might be seen in carpal tunnel syndrome, to acute traumatic nerve injuries that require rapid interven ... Full text Link to item Cite

Can mycophenolate mofetil be tapered safely in myasthenia gravis? A retrospective, multicenter analysis.

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

Small-fiber neuropathy in pompe disease: first reported cases and prospective screening of a clinic cohort.

Journal Article Am J Case Rep · April 3, 2015 BACKGROUND: Prior autopsy reports demonstrate glycogen deposition in Schwann cells of the peripheral nerves in patients with infantile and late-onset Pompe disease (LOPD), but little is known about associated clinical features. CASE REPORT: Here, we report ... Full text Open Access 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

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

Ultrasound diagnosis of bony nerve entrapment: case series and literature review.

Journal Article Muscle Nerve · September 2013 INTRODUCTION: Nerve entrapment due to osseous callus formation is a rare complication after bone fracture. Electrodiagnostic studies and routine radiographic imaging often fail to demonstrate the pathology. The diagnosis is difficult and is often made inci ... Full text Link to item Cite

Neuromuscular ultrasound in polyneuropathies and motor neuron disease.

Journal Article Muscle Nerve · June 2013 Current standards for diagnosing polyneuropathies (PN) and motor neuron disease (MND) sometimes lack early sensitivity and result in delayed diagnosis and treatment. Neuromuscular ultrasound (NMUS), already established in the diagnosis of entrapment neurop ... Full text Link to item Cite

Oropharyngeal dysphagia may occur in late-onset Pompe disease, implicating bulbar muscle involvement.

Journal Article Neuromuscul Disord · April 2013 Late-onset Pompe disease (presenting after 12 months of age) often presents with limb-girdle and respiratory weakness, but oropharyngeal dysphagia has not been reported previously. A retrospective review of all late-onset Pompe disease patients evaluated i ... Full text Open Access Link to item Cite

Reporting the results of diagnostic neuromuscular ultrasound: an educational report.

Journal Article Muscle Nerve · April 2013 INTRODUCTION: Neuromuscular ultrasound (NMUS), an emerging diagnostic subspecialty field, has become an important extension of the electrodiagnostic examination. However, there are no formal guidelines on how to appropriately report NMUS results. METHODS: ... 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

Ultrasonography in the diagnosis of peripheral nerve disease.

Journal Article Expert Opin Med Diagn · September 2012 INTRODUCTION: High-resolution ultrasound (US) of the peripheral nerves is now a standard means of assessing neuromuscular disorders in many centers. Currently used in conjunction with electrodiagnostic (EDX) studies, nerve US is especially effective in the ... Full text Link to item Cite

Evidence-based guideline: neuromuscular ultrasound for the diagnosis of carpal tunnel syndrome.

Journal Article Muscle Nerve · August 2012 INTRODUCTION: The purpose of this study was to develop an evidence-based guideline for the use of neuromuscular ultrasound in the diagnosis of carpal tunnel syndrome (CTS). METHODS: Two questions were asked: (1) What is the accuracy of median nerve cross-s ... Full text Link to item Cite

Autopsy findings in late-onset Pompe disease: a case report and systematic review of the literature.

Journal Article Mol Genet Metab · August 2012 BACKGROUND: Late-onset Pompe disease (LOPD) is a rare cause of declining proximal muscle strength and respiratory function that can also affect other organ systems. The development of enzyme replacement therapy has made it one of the few inherited muscle d ... Full text Link to item Cite

The clinical and electrodiagnostic characteristics of Pompe disease with post-enzyme replacement therapy findings.

Journal Article Clin Neurophysiol · November 2011 OBJECTIVE: Pompe disease is a neuromuscular disorder that was progressive and fatal prior to enzyme replacement therapy (ERT). The advent of treatment has made early recognition imperative. Electrodiagnostic (EDx) studies represent a valuable diagnostic to ... Full text 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

The electrodiagnostic characteristics of Glycogen Storage Disease Type III.

Journal Article Genet Med · July 2010 PURPOSE: Glycogen Storage Disease Type III, also known as debrancher deficiency or Cori disease, is an autosomal recessive disorder recognized for both its hepatic and muscle manifestations. The neuromuscular manifestations of Glycogen Storage Disease Type ... Full text Link to item Cite

Glycogen storage disease type III diagnosis and management guidelines.

Journal Article Genet Med · July 2010 PURPOSE: Glycogen storage disease type III is a rare disease of variable clinical severity affecting primarily the liver, heart, and skeletal muscle. It is caused by deficient activity of glycogen debranching enzyme, which is a key enzyme in glycogen degra ... 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

Median nerve ultrasonography in carpal tunnel syndrome: findings from two laboratories.

Journal Article Muscle Nerve · July 2009 Ultrasonographic (US) enlargement of the median nerve at the wrist is known to be consistent with carpal tunnel syndrome (CTS), although the effects of different measurement techniques, equipment, and patient populations remain unknown. The purpose of this ... Full text Link to item Cite

Case-control study of thromboembolic events associated with IV immunoglobulin.

Journal Article J Neurol · March 2009 Serious adverse events related to IVIg treatment are unusual, and interventions can be taken to reduce the risk of anaphylaxis, congestive heart failure and renal failure. Stroke and other thromboembolic (TE) events have also been associated with IVIg admi ... Full text Link to item Cite

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Journal Article Muscle and Nerve · September 1, 2008 Full text 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

The more the merrier?

Journal Article Muscle Nerve · May 2008 Full text Link to item Cite

Metronidazole: newly recognized cause of autonomic neuropathy.

Journal Article J Child Neurol · May 2006 Metronidazole is a commonly used antibiotic prescribed for the treatment of anaerobic and protozoal infections of the gastrointestinal and genitourinary tracts. It is associated with numerous neurologic complications, including peripheral neuropathy. Neuro ... Full text Link to item Cite

Facioscapulohumeral muscular dystrophy can be a cause of isolated childhood cognitive dysfunction.

Journal Article J Child Neurol · March 2006 Facioscapulohumeral muscular dystrophy is one of the most prevalent muscular dystrophies in the world, resulting from the deletion of tandem repeats on chromosome 4q35. Extramuscular associations include sensorineural hearing loss, mental retardation, and ... Full text Link to item Cite

Inflammatory neuropathies: an update on evaluation and treatment.

Journal Article Curr Rheumatol Rep · October 2005 Inflammatory neuropathies are a diverse group of illnesses sharing the pathologic characteristic of inflammation surrounding nerve fibers. They may be autoimmune, granulomatous, infectious, paraneoplastic, or paraproteinemic in origin. All can result in si ... Full text Link to item Cite

Oropharyngeal dysphagia

Journal Article Clinical Perspectives in Gastroenterology · January 1, 2000 Swallowing is a highly coordinated cascade of events, involving 30 pairs of muscles, six pairs of cranial/cervical nerves, and brain-stem and cortical swallowing centers, that results int he passage of ingested materials and oropharyngeal secretions into t ... Cite