We evaluated the functional consequences of diaphragm involvement in patients with inclusion body myositis (IBM).

We utilized the Pooled Resource Open‐Access Clinical Trials (PRO‐ACT) database to investigate whether melatonin use among patients with amyotrophic lateral sclerosis (ALS) was associated with slower disease progression and prolonged survival.

Despite use of qualitative laryngeal electromyography (LEMG) guided botulinum toxin A (BoNT‐A) injection for treatment of adductor spasmodic dysphonia (AdSD), unsatisfactory injections and complete “misses” remain problematic. We aimed to determine if the quantitative LEMG measure of number of small segments (NSS) correlates with voice outcomes following (BoNT‐A injection for AdSD.

A newly introduced term, “axonal conduction block,” brought a confusion in the electrodiagnostic diagnosis of Guillain‐Barrè syndrome (GBS). I am proposing the term “nodal conduction block” for “axonal conduction block.” This unifying concept of nodal conduction block will accommodate both the traditional concept of demyelination as well as the new concept of nodopathy in the “axonal form of GBS,”

In this study we explored walking activity in a large cohort of boys with Duchenne muscular dystrophy (DMD).

GNE myopathy is widely regarded as a distal myopathy. Involvement of proximal musculature in this condition has not been systematically studied.

Fasciculations are most commonly seen in the biceps brachii muscle in amyotrophic lateral sclerosis (ALS). In this study we have explored the association between fasciculation frequency in a single location—biceps brachii and brachialis muscles (BB), and disease burden and activity.

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.

This study examines the correlation, and clinical meaningfulness, between reachable workspace outcome and reported activities of daily living (ADL) function of individuals with facioscapulohumeral dystrophy (FSHD).

Erythropoietin (EPO) promotes myelination and functional recovery in rodent peripheral nerve injury (PNI). While EPO receptors (EpoR) are present in Schwann cells, the role of EpoR in PNI recovery is unknown because of the lack of EpoR antagonists or Schwann cell‐specific EpoR knockout animals.

The purpose of this study was to estimate the risk of death by suicide for those with amyotrophic lateral sclerosis (ALS) seeking care within the Veteran Health Administration (VHA).

The clinical course of neuromuscular disorders (NMDs) can be affected by infections, both in immunocompetent individuals, and in those with reduced immunocompetence due to immunosuppressive/immunomodulating therapies. Infections and immunizations may also trigger NMDs. There is a potential for reduced efficacy of immunizations in patients with reduced immunocompetence. The recent vaccination program

Quantitative muscle MRI as a sensitive marker of early muscle pathology and disease progression in adult‐onset myotonic dystrophy type 1

Amyloidosis refers to an etiologically heterogeneous group of protein misfolding diseases, pathologically characterized by extracellular amyloid fibrils producing congophillic amorphous deposits in organs and tissues, which may lead to severe organ dysfunction and mortality. Clinical presentations vary and are often nonspecific, depending on what organs or tissues are affected. In systemic amyloidosis

Precise placement of stimulating and recording electrodes is vital when performing nerve conduction studies (NCSs). In this study, we aimed to determine whether ultrasonography (US) was more precise in localizing the superficial radial nerve (SRN), dorsal ulnar cutaneous nerve (DUCN), ulnar nerve (UN) crossing the cubital tunnel, and radial nerve (RN) crossing the spiral groove (SG) compared to conventional

The Boston Carpal Tunnel Questionnaire (BCTQ) is a patient‐reported outcome measure (PROM) used to measure symptom severity and function in carpal tunnel syndrome (CTS). Despite its wide usage, investigation of its measurement properties using modern psychometric methodologies is limited.

The most common subtypes of Guillain‐Barré syndrome (GBS) are acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). In the first days after the onset of weakness, standard nerve conduction studies (NCS) may not distinguish GBS subtypes. Reduced nerve excitability may be an early symptom of nerve dysfunction, which can be determined with the compound muscle

We aimed to describe the electrophysiological progression rate of chronic idiopathic axonal polyneuropathy (CIAP) and look into the potential role of human leukocyte antigen (HLA) genetic susceptibility in its development.

Myotonic dystrophy type 2 (DM2) is a neuromuscular disorder characterized by myotonia and muscle weakness, with no medical treatment to prevent a decline in decline. It is unknown whether exercise training is effective in DM2. The aim of this study was to investigate the effect of exercise training on functional capacity and body composition in these patients.

In adult males, cross‐sectional area (CSA) for type II muscle fibers is generally larger than for type I fibers. In this cross‐sectional study the aim was to compare sex‐related CSAs of various muscle fiber types during childhood‐to‐adulthood transition.

Skeletal muscle inflammation and oxidative stress are associated with aging‐related loss of muscle mass and may be attributable to alterations in the number and types of leukocytes in skeletal muscle. Here, we tested the hypothesis that aging changes the number and composition of leukocyte subsets in skeletal muscle tissue.

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. Our aim was to survey experts' opinions regarding physician competency assessment of neuromuscular ultrasound and to identify minimal competency

Collagen VI related myopathies are congenital diseases of variable phenotype. The severe phenotype is referred to as Ullrich congenital muscular dystrophy. In this study, we describe analoguos clinical signs and histopathological alterations in Landseer dogs.

Thymectomy is required for the treatment of thymoma‐associated myasthenia gravis (MG). However, MG may develop only after thymectomy, a condition known as post‐thymectomy MG. This study aimed to investigate the risk factors for post‐thymectomy MG in patients with thymoma.

Anti‐cytosolic 5′‐nucleotidase 1A (cN1A) antibodies are commonly detected in patients with sporadic inclusion body myositis (sIBM). However, their pathogenic role has not been established. Moreover, efforts toward identifying sIBM distinct clinicopathologic characteristics associated with these antibodies have yielded conflicting results.

Several studies of patients with polyneuropathy failed to show differences between patients with and without pain. In the current study, we aimed to explore the association between neuropathic symptoms, mainly pain, and polyneuropathy characteristics.

The present study assesses the contributions of axonal degeneration and demyelination in leprosy nerve damage. New clinical strategies can emerge from an in‐depth understanding of the pathogenesis of neural leprosy (NL).

We aim to describe 12‐mo functional and motor outcome performance in a cohort of participants with congenital myotonic dystrophy (CDM).

Identification and treatment of immune‐mediated polyneuropathies may lead to improved strength and function. We studied the clinical and laboratory features, and treatment response, in patients with motor‐sensory axonal polyneuropathies who were found to have C5b‐9 complement staining on endoneurial microvessels.

Carpal tunnel syndrome (CTS) may be associated with structural lesions or anatomical variations at the wrist, especially in patients whose symptoms are more severe in, or limited to, the nondominant hand. The aims of this study were to identify the type and frequency of structural abnormalities and anatomical variations, and to demonstrate the contribution of ultrasound in this subgroup of CTS patients

There is increasing evidence that calcitonin gene–related peptide (CGRP) plays a role in the development of neuropathic pain, a common feature of peripheral neuropathy. Although clinical studies have shown that anti‐CGRP monoclonal antibodies are highly efficacious for migraine headache prophylaxis, their effects on nonheadache chronic pain conditions, including neuropathic pain, in humans are unknown

There is a great demand for accurate non‐invasive measures to better define the natural history of disease progression or treatment outcome in Duchenne muscular dystrophy (DMD) and to facilitate the inclusion of a large range of participants in DMD clinical trials. This review aims to investigate which MRI sequences and analysis methods have been used and to identify future needs. Medline, Embase,

To collect preliminary data on the effects of mexiletine on cortical and axonal hyperexcitability in sporadic amyotrophic lateral sclerosis (ALS) in a phase 2 double‐blind randomized controlled trial.

Erratum for P. Seror et al. “Voluntary winging of the scapula: proposed diagnostic criteria”. Muscle and Nerve, November 2020; Volume 62. Issue 5. Pages 611–616. https://doi.org/10.1002/mus.27047 There was an error in the author list. MK Alexandra Roren, PhD, should read Alexandra Roren, PhD. We apologize for this error.

Prognostic uncertainty in amyotrophic lateral sclerosis (ALS) confounds clinical management planning, patient counseling, and trial stratification. Fasciculations are an early clinical hallmark of disease and can be quantified noninvasively. Using an innovative analytical method, we correlated novel fasciculation parameters with a predictive survival model.

Diagnosing cervical radiculopathy (CR) can be difficult because of symptomatic overlap with peripheral neuropathies. In this retrospective observational study, we aimed to determine whether short‐tau inversion recovery (STIR) magnetic resonance imaging (MRI) sequences are useful for detecting signs of denervation in the multifidus muscles in patients with CR.

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.

Unilateral diaphragmatic paralysis (UDP) has major clinical and etiological implications and, therefore, is important to diagnose. Lung function tests and invasive transdiaphragmatic pressure (Pdi) measurements are widely used to this end but, contrary to phrenic nerve conduction study (NCS), they require volitional maneuvers and/or may be poorly tolerated by patients. The purpose of this study was

Mutations in the GNE gene have been so far described as predominantly associated with distal lower‐limb myopathies. Recent reports describe mutations in this gene in patients with peripheral neuropathy and motor neuron disease.

A distal‐predominant demyelinating symmetric pattern is most frequent in patients with neuropathy associated with anti‐myelin‐associated glycoprotein (MAG) antibodies. The literature however lacks longitudinal data to describe whether this is consistent over time.

This two‐part study explored the safety, feasibility, and efficacy of a mild–moderate resistance isometric leg exercise program in ambulatory boys with Duchenne muscular dystrophy (DMD).

We propose the finger drop sign as a new clinical variant of acute motor axonal neuropathy (AMAN) defined by immunological and radiological evidence. We identified eight consecutive patients who had AMAN. All of them developed prominent involvement of the finger extensors. We performed magnetic resonance imaging (MRI) of the extremity muscles and serological assays for antiganglioside antibodies and

The aim of this study was to assess the relationship between ultrasonographic hand muscle thickness measurements and hand muscle strength in patients who underwent median or ulnar nerve reconstruction.

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 disease 2019 (COVID‐19) treatments on neuromuscular transmission, and a limited ability to fight off infection related to immunosuppressive treatments. Our goal

Regenerative peripheral nerve interfaces (RPNIs) transduce neural signals to provide high‐fidelity control of neuroprosthetic devices. Traditionally, rat RPNIs are constructed with ~150 mg of free skeletal muscle grafts. It is unknown whether larger free muscle grafts allow RPNIs to transduce greater signal.

See article on pages 40–45 in this issue.

An orally administered, fixed‐dose coformulation of sodium phenylbutyrate‐taurursodiol (PB‐TURSO) significantly slowed functional decline in a randomized, placebo‐controlled, phase 2 trial in ALS (CENTAUR). Herein we report results of a long‐term survival analysis of participants in CENTAUR. In CENTAUR, adults with ALS were randomized 2:1 to PB‐TURSO or placebo. Participants completing the 6‐month

Multiple mononeuropathy is a rare presentation of primary (AL) amyloidosis and nerve biopsy is usually needed for diagnosis. Conventional imaging is useful to identify proximal nerve involvement but may be inadequate. We report a patient with multiple mononeuropathy whose presentation was suggestive of AL amyloid neuropathy and in whom repeated tissue biopsies were negative for amyloid (including two

The split‐hand concept has highlighted the preferential wasting of the thenar side of the hand in amyotrophic lateral sclerosis (ALS). Our objective is to re‐explore pinch grip strength to assess whether it has the potential to be a practical biomarker of ALS.

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

The aim of this study was to establish reference jitter values for the voluntary activated sternocleidomastoid (SCM) muscle using a concentric needle electrode (CNE).

The goals of this study were to determine whether serum concentrations of endocannabinoids (eCB) and related lipids predict disease status in patients with amyotrophic lateral sclerosis (ALS) relative to healthy controls, and whether concentrations correlate with disease duration and severity.

Electrical impedance myography (EIM) provides insight into muscle composition and structure. We sought to evaluate its use in a mouse obesity model characterized by myofiber atrophy.

See article on pages E10 in this issue.

See article on pages E9–E10 in this issue.

This study aimed to examine the early experience of nusinersen for spinal muscular atrophy (SMA) from the patient and caregiver perspective.

One barrier to widespread adoption of neuromuscular ultrasound by clinical neurophysiologists is concern over how to identify and manage non‐neuromuscular findings. This review addresses this concern by describing the sonographic appearance of a variety of commonly observed pathologies and anatomic variants in dermal, subcutaneous, bony, glandular, lymphatic, vascular, and other superficial tissues

Several E2 (reference electrode) positions are described for fibular (peroneal) nerve conduction studies to tibialis anterior (TA).

We recently developed an inducible model of dysphagia using intralingual injection of cholera toxin B conjugated to saporin (CTB‐SAP) to cause death of hypoglossal neurons. In this study we aimed to evaluate tongue morphology and ultrastructural changes in hypoglossal neurons and nerve fibers in this model.