Turns‐amplitude, number of small segments (NSS)‐activity and envelope‐activity clouds are 3 methods of electromyography (EMG) interference pattern analysis. Our objective was to evaluate the sensitivity and specificity of each individual cloud analysis and combined clouds analysis to compare with that of quantitative motor unit potential (QMUP) analysis.

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.

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 majority of patients with myasthenia gravis (MG) initially present with ocular symptoms but it is difficult to predict which cases will remain as ocular MG (OMG) or will progress to generalized MG. Here, we evaluated the serological profile of Japanese OMG and its relationship with clinical features.

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.

Diabetic peripheral neuropathy and metabolic syndrome (MetS) are both global health challenges with well‐established diagnostic criteria and significant impacts on quality of life. Clinical observations, epidemiologic evidence, and animal models of disease have strongly suggested MetS is associated with an elevated risk for cryptogenic sensory peripheral neuropathy (CSPN). MetS neuropathy preferentially

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.

We studied the performance of a 15‐item, health‐related quality‐of‐life polyneuropathy scale in a longitudinal study of patients with chronic inflammatory demyelinating polyneuropathy (CIDP).

Most amyotrophic lateral sclerosis (ALS) cases are considered sporadic, without a known genetic basis, and environmental exposures are thought to play a causal role. To learn more about sporadic ALS etiology, we recruited n = 188 ALS patients from northern New England and Ohio and matched controls 2:1 from the general population of the same regions. Questionnaires evaluated the association between

We previously reported a new quantitative analysis of single‐channel surface electromyography (EMG), the “clustering index method” (CI method), in the tibialis anterior muscle, which achieved sufficiently good sensitivity to detect neurogenic or myogenic abnormalities. The window width is a fundamental parameter of the CI method, and was arbitrarily set at 15 ms in that study. In this study, we searched

Nerve ultrasound has been used increasingly in clinical practice as a complementary test for diagnostic assessment of neuropathies, but nerve biopsy remains invaluable in certain cases. The aim of this study was to compare ultra–high‐frequency ultrasound (UHF‐US) to histologic findings in progressive polyneuropathies. Ten patients with severe, progressive neuropathies underwent ultrasound evaluation

See article on pages 633–637 in this issue.

Dystussia impacts the ability to protect the airway. Voluntary cough provides a metric of airway defense but differs from the reflexive response to aspiration during swallowing. Therefore, we evaluated relationships between voluntary and reflexive cough among individuals with amyotrophic lateral sclerosis (ALS) and a healthy cohort.

Ultrasound has revealed cross‐sectional nerve area (CSA) reduction in amyotrophic lateral sclerosis (ALS), but little is known about the sonographic nerve texture beyond CSA alterations. In a large cohort of 177 ALS patients and 57 control subjects, we investigated the covariance and disease‐specific signature of several sonographic texture features of the median and ulnar nerves and their relationship

We report a series of 10 patients with unilateral, dynamic, winged scapula (WS), without cause, that was diagnosed as voluntary winging of the scapula (VWS). We compared clinical, electrodiagnostic, and other examination data for 10 patients with VWS and 146 with dynamic WS‐related neuromuscular disorders, to establish a detailed pattern of the VWS subtype. In VWS, electrodiagnostic and other examinations

This series characterises nine patients with neurohistopathologically proven peripheral nerve neurolymphomatosis. A search of the hospital neuropathology database from 2002 to 2019 identified biopsy proven cases. Clinical data, investigation modalities, treatments, and outcomes were collated. Median age at neuropathy onset was 47 y, the neuropathy commonly as the initial lymphoma disease manifestation

Reported frequencies of cardiomyopathy in limb girdle muscular dystrophy R9 (LGMDR9) vary. We describe the frequency and age at onset of cardiomyopathy in an LDMDR9 cohort.

We aimed to determine the safety and tolerance of higher rates of infusion of intravenous immunoglobulin (IVIG) in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

First reported in December 2019 in Wuhan, China, novel coronavirus (SARS‐CoV‐2) has become a major challenge for health‐care systems around the world. It is estimated that about 17% of patients develop severe pneumonia, with a high incidence of acute respiratory distress syndrome (ARDS). Among the treatments for managing ARDS, prone positioning is used to improve ventilation.1 There are a few reports

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

Little is known about how different outlier estimation methods affect cut‐off limits for outliers in single fiber electromyography.

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). Here, we report results of a long‐term survival analysis of participants in CENTAUR.

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.

Specific force, that is the amount of force generated per unit of muscle tissue, is reduced in patients with facioscapulohumeral muscular dystrophy (FSHD). The causes of reduced specific force and its relation with FSHD disease severity are unknown.

The report of Cacciavillani and colleagues contributes to the discussion of the possibility of peripheral nerve involvement in coronavirus disease 2019 (COVID‐19) and adds to our observation of neuralgic amyotrophy following infection with severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2).1, 2 Their case viewed in conjunction with a growing body of evidence underscores a relevant pathophysiological

Muscle ultrasound is a valuable addition to the neuromuscular toolkit in both the clinic and research settings, with proven value and reliability. However, it is currently not fulfilling its full potential in the diagnostic care of patients with neuromuscular disease. This review highlights the possibilities and pitfalls of muscle ultrasound as a diagnostic tool and biomarker, and discusses challenges

The muscle relaxant methocarbamol is widely used for the treatment of muscle spasms and pain syndromes. To elucidate molecular mechanisms of its action, we studied its influence on neuromuscular transmission, on isometric muscle force and on voltage‐gated Na+ channels.

It is unclear whether the heart is affected in pediatric patients with milder forms of spinal muscular atrophy (SMA). Therefore, we aimed to determine the presence of any cardiac abnormalities in these patients.

Smooth muscle dysfunction in Duchenne muscular dystrophy (DMD) has been rarely studied. A cross‐sectional study was conducted to estimate the prevalence of smooth muscle dysfunction (vascular, upper gastrointestinal, and bladder smooth muscle) in children with DMD using questionnaires (Pediatric Bleeding Questionnaire, Pediatric Gastroesophageal Symptom Questionnaire, and Dysfunctional Voiding Symptom

We read with great interest the case report on neuralgic amyotrophy following infection with severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2).1 Respiratory symptoms are the primary manifestations of SARS‐Cov‐2 infection. Reports of several neurologic manifestations have most commonly involved the central nervous system.2 In addition to olfactory dysfunction, peripheral nervous system involvement

Childhood onset chronic inflammatory demyelinating polyneuropathy (CIDP) often requires long‐term immunomodulatory therapy. We report a comprehensive review of our treatment of pediatric CIDP with a focus on high‐dose weekly corticosteroids (“pulse oral corticosteroids”), a treatment method that is not commonly reported. We retrospectively reviewed medical records of pediatric patients with CIDP treated

We aimed to examine the influence of different speeds of stretching on denervation‐induced skeletal muscle fibrosis.

We aimed at evaluating the differential involvement of large myelinated Aβ‐fibers, small myelinated Aδ‐ and unmyelinated C‐fibers in patients with diabetic polyneuropathy and how they contribute to neuropathic pain.

When performing postexercise facilitation (PEF) as part of the repetitive nerve stimulation (RNS) test in Lambert‐Eaton myasthenic syndrome (LEMS), it is important to avoid any influence of the previous exercise or RNS test on the compound muscle action potential (CMAP) amplitude.

Antibodies against ganglioside complexes (GSCs) are associated with various clinical features and subtypes of Guillain‐Barré syndrome (GBS).

Size index (SI) is a motor unit potential (MUP) parameter in concentric needle electromyography calculated from amplitude and area/amplitude, which can sensitively discriminate between control and neurogenic MUPs. In this study, we investigated the application of SI to myogenic MUPs based on expanded data.

Idiopathic small fiber neuropathy (iSFN) lacks broadly accepted diagnostic criteria, which hinders its timely diagnosis and treatment. A systematic literature review was performed to assess the published screening and diagnostic criteria for iSFN, excluding studies where SFN was of well‐established etiology. Eighty‐four clinical studies and seven guideline/review publications were included in this

Myasthenia gravis (MG) is an autoimmune disorder with bimodal age of presentation, occurring in young women of reproductive age and at an older age in men. Occasionally, MG is diagnosed during pregnancy. Management of MG includes symptomatic treatment with cholinesterase inhibitors and immunosuppressive therapy for controlling the disease activity. Treatment of MG in women of reproductive age, who

Anand et al1 described five patients with a pre‐existing diagnosis of myasthenia gravis (MG) and concurrent coronavirus disease 2019 (COVID‐19). Three developed hypoxemia requiring respiratory support, but none exhibited definite worsening weakness related to MG. At the time of the infection, four patients were in Myasthenia Gravis Foundation of America (MGFA) severity class I or in pharmacological

This study aimed to elucidate the longitudinal changes in nerve ultrasound parameters of adult Charcot–Marie–Tooth disease type 1A (CMT1A) patients.

Ulnar neuropathy at the elbow (UNE) is the second most common entrapment neuropathy. Our goal was to create and analyze a grading system for UNE electrodiagnostic severity.

Our aim was to assess key muscle imaging and contractility parameters in the Duchenne muscular dystrophy (DMD) rat model (Dmd‐KO rat), which have not yet been characterized sufficiently.

We thank Drs Galassi and Marchioni for their letter to the editor regarding our report on a series of patients with coronavirus disease‐2019 (COVID‐19) and myasthenia gravis (MG). We appreciate their suggestion that further diagnostic testing, such as chest computed tomography (CT), arterial blood gas sampling, and pulmonary function testing, may help to distinguish the effects of COVID‐19 from those

Duchenne muscular dystrophy (DMD) has been found to be associated with cognitive impairment. However, few studies have addressed cognitive impairment among mothers of children with DMD. In the present study, the neuropsychological profiles of both carrier mothers (C‐Ms) and noncarrier mothers (NC‐Ms) were examined, and the findings were compared with healthy control mothers (HC‐Ms). There were 90 participants

Serial muscle biopsies within clinical trials for Duchenne muscular dystrophy (DMD) are critical to document therapeutic responses. Less invasive means of sampling muscle are needed. We analyzed a retrospective consecutive case‐series cohort of vacuum‐assisted core needle muscle biopsy procedures performed on healthy and dystrophic individuals at a single institution assessing for safety and reliability

See article on pages 351–357 in issue MUS 62:3.

See article on pages 492–501 in this issue.

Guillain‐Barré syndrome (GBS) is an inflammatory polyradiculoneuropathy associated with numerous viral infections. Recently, there have been many case reports describing the association between coronavirus disease‐2019 (COVID‐19) and GBS, but much remains unknown about the strength of the association and the features of GBS in this setting. We reviewed 37 published cases of GBS associated with COVID‐19

Inotersen, an antisense oligonucleotide inhibitor of transthyretin (TTR) protein production, demonstrated significant benefit versus placebo in the modified Neuropathy Impairment Score (NIS) +7 neurophysiologic tests (mNIS+7) in patients with hereditary TTR‐mediated amyloidosis (hATTR) with polyneuropathy. This analysis assessed the mNIS+7 components by anatomic location and the lower limb function

Hereditary transthyretin‐mediated amyloidosis (hATTR) manifests as multisystem dysfunction, including progressive polyneuropathy. Inotersen, an antisense oligonucleotide, improved the course of neuropathic impairment in patients with hATTR in the pivotal NEURO‐TTR study (NCT01737398). To determine inotersen's impact on symptoms and patients’ neuropathy experience, we performed a post hoc analysis of

We evaluated the association between muscle ultrasound, number of motor units, and clinical parameters, and assessed their utility for distinguishing amyotrophic lateral scleorisis (ALS) patients from healthy individuals.

Evidence‐based information about cerebrospinal fluid (CSF) levels of biomarkers in patients with amyotrophic lateral sclerosis (ALS) is limited.

In this study, we aimed to investigate the progression of peripheral nervous system involvement in xeroderma pigmentosum group A (XP‐A).

MRI is a helpful tool for monitoring disease progression in late‐onset Pompe disease (LOPD). Our study aimed to evaluate if muscle diffusion tensor imaging (mDTI) shows alterations in muscles of LOPD patients with <10% fat‐fraction. We evaluated 6 thigh and 7 calf muscles (both legs) of 18 LOPD and 29 healthy controls (HC) with muscle diffusion tensor imaging (mDTI), T1w, and mDixonquant sequences