Effects of short-term unloading and active recovery on human motor unit properties, neuromuscular junction transmission and transcriptomic profile.

Electrophysiological alterations of the neuromuscular junction (NMJ) and motor unit potential (MUP) with unloading are poorly studied. We aimed to investigate these aspects and the underlying molecular mechanisms with short-term unloading and active recovery (AR). Eleven healthy males underwent a 10-day unilateral lower limb suspension (ULLS) period, followed by 21-day AR based on resistance exercise.

Shoulder palsy following SARS-CoV-2 infection: two cases of typical Parsonage-Turner syndrome.

Background And Purpose: Coronavirus disease 2019 (COVID-19) is now known to cause neurological complications in both the central and the peripheral nervous system. Two new cases of typical neuralgic amyotrophy or Parsonage-Turner (PT) syndrome following coronavirus 2 infection (SARS-CoV-2) are reported here with explicit electrophysiological and imaging pathological features, underlining the possible association between COVID-19 and PT syndrome.

Case Reports: Case 1 was a 45-year-old schoolteacher presenting with acute pain in the right shoulder a few days after SARS-CoV-2 infection, with shoulder abduction and elbow flexion weakness.

Clinical features of Pompe disease with motor neuronopathy.

Pathological studies on rodent models and patients with Pompe disease have demonstrated the accumulation of glycogen in spinal motor neurons; however, this finding has rarely been evaluated clinically in patients with Pompe disease. In this study, we analyzed seven patients (age, 7-11 years) with Pompe disease who received long-term enzyme replacement therapy. In addition to traditional myopathy-related clinical and electrophysiological features, these patients often developed bilateral foot drop, distal predominant weakness of four limbs, and hypo- or areflexia with preserved sensory function.

Muscle thickness measured by ultrasound is reduced in neuromuscular disorders and correlates with clinical and electrophysiological findings.

Introduction: Nerve imaging has a limited role in axonal and muscle fiber loss. In this study, we sought to explore the utility of standardized muscle ultrasound (US) assessment in these clinical scenarios.

Methods: We performed a prospective study from March to August 2018 of patients attending the neuromuscular clinic.

Electrophysiologic evaluation of facial nerve functions after oxaliplatin treatment.

Purpose: This study analyzes the effect of oxaliplatin treatment on the facial nerve. The facial nerve is the most commonly paralyzed cranial motor nerve because it advances through a long, curved bone canal. Electroneurography and blink reflex are the electrophysiological measurements used for evaluating facial nerve function.

Tone-Inhibiting Insoles Enhance the Reciprocal Inhibition of Ankle Plantarflexors of Subjects With Hemiparesis After Stroke: An Electromyographic Study.

Background: Spasticity is a common sequela of upper motor neuron pathology, such as cerebrovascular diseases and cerebral palsy. Intervention for spasticity of the ankle plantarflexors in physical therapy may include tone-inhibiting casting and/or orthoses for the ankle and foot. However, the physiological mechanism of tone reduction by such orthoses remains unclarified.

Motor Unit Number Index (MUNIX) detects motor neuron loss in pre-symptomatic muscles in Amyotrophic Lateral Sclerosis.

Objective: Motor Unit Number Index (MUNIX) is a quantitative neurophysiological measure that provides an index of the number of lower motor neurons supplying a muscle. It reflects the loss of motor neurons in patients with Amyotrophic Lateral Sclerosis (ALS). However, it is unclear whether MUNIX also detects motor unit loss in strong, non-wasted muscles.

Lower motor neuron dysfunction in ALS.

In the motor system there is a complex interplay between cortical structures and spinal cord lower motor neurons (LMN). In this system both inhibitory and excitatory neurons have relevant roles. LMN loss is a marker of motor neuron disease/amyotrophic lateral sclerosis (MND/ALS).

The neuromuscular impact of symptomatic SMN restoration in a mouse model of spinal muscular atrophy.

Background: Significant advances in the development of SMN-restoring therapeutics have occurred since 2010 when very effective biological treatments were reported in mouse models of spinal muscular atrophy. As these treatments are applied in human clinical trials, there is pressing need to define quantitative assessments of disease progression, treatment stratification, and therapeutic efficacy. The electrophysiological measures Compound Muscle Action Potential and Motor Unit Number Estimation are reliable measures of nerve function.

Electrophysiologic and clinico-pathologic characteristics of statin-induced muscle injury.

Objectives: In this study, we aimed at evaluation of electrophysiological and histopathalogical characteristics of statin-induced muscle injury as well as clinical features of patients who develop this condition in terms of frequency and pattern of evolution.

Materials And Methods: Forty patients (age 39-74 years) including 25 subjects with type 2 diabetes mellitus, 9 with cardiovascular diseases and 6 with hyperlipidemia, who were receiving atrovastatin 40 mg/day for variable period, were studied. Thirty three healthy subjects (age 31-74 years) served as control group.

Intestinal ischemia-reperfusion induced diaphragm contractility dysfunction: Electrophysiological and ultrastructural study in a neonatal rat model.

Aim: To evaluate the remote effect of intestinal ischemia reperfusion (IR) injury mediated by tumor necrosis factor alpha (TNF-α) on diaphragm contractility functions and whether administration of NAC may counteract the possible detrimental effects in an experimental neonatal rat model.

Methods: 40 Wistar rat pups were randomized into four groups; ten animals in each. Intestinal ischemia was conducted by obstructing mesentery of intestines by a silk loop.

Tracking motor neuron loss in a set of six muscles in amyotrophic lateral sclerosis using the Motor Unit Number Index (MUNIX): a 15-month longitudinal multicentre trial.

Background: Motor Unit Number Index (MUNIX) is a novel neurophysiological measure that provides an index of the number of functional lower motor neurons in a given muscle. So far its performance across centres in patients with amyotrophic lateral sclerosis (ALS) has not been investigated.

Objective: To perform longitudinal MUNIX recordings in a set of muscles in a multicentre setting in order to evaluate its value as a marker of disease progression.

Does quantitative EMG differ myotonic dystrophy type 2 and type 1?

Genetic testing is considered the only reliable diagnostic approach in myotonic dystrophy. However it has recently been reported that a considerable number of patients with genetically proven types of the disease have unusual phenotypic presentation. The aim of our study was to evaluate motor unit reorganization reflected by various electrophysiological abnormalities in myotonic dystrophies and to compare findings between type 1 (DM 1) and type 2 myotonic dystrophy (DM2).

Electrodiagnostic evaluation of myopathies.

Electrodiagnostic studies play an important role in the evaluation of patients suspected of having a myopathic disorder. They are used to exclude alternative diagnoses, confirm the presence of muscle disease, narrow down the differential, and identify an appropriate biopsy site. The most informative part of the electrodiagnostic study is needle electromyography.

Painful legs and moving toes syndrome: a 76-patient case series.

Objective: To better characterize the clinical features, electrophysiologic features, and treatment outcomes of painful legs and moving toes (PLMT) syndrome.

Design: Large case series.

Setting: Neurology outpatient clinic at a tertiary referral center, 1983-2011.

Evaluation of spinal and bulbar muscular atrophy by the clustering index method.

Introduction: A reliable electrophysiological marker for clinical trials is increasingly needed in spinal and bulbar muscular atrophy (SBMA). We previously developed a quantitative analysis method for surface electromyography (SEMG), the clustering index (CI) method. Our purpose was to test the utility of the CI method for evaluating lower motor neuron involvement in SBMA patients.

Electrophysiological analysis of a murine model of motoneuron disease.

Objective: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by loss of motoneurons of the primary motor cortex, the brainstem and the spinal cord, for which there are not effective treatments. Several transgenic mice that mimic motoneuron disease have been used to investigate potential treatments. The objective of this work is to characterize electrophysiologically the SOD1(G93A) transgenic mouse model of ALS, and to provide useful markers to improve early detection and monitoring of progression of the disease.

Suprascapular neuropathy in a shoulder referral practice.

Hypothesis: Suprascapular neuropathy (SSN) is considered a rare condition, and few studies have analyzed how commonly it is encountered in practice. Electrophysiologic studies are the gold standard for diagnosis; however, there is no consensus on diagnostic criteria. We hypothesized that SSN would be frequently diagnosed by electrophysiologic testing in a subset of patients with specific clinical and radiographic findings suggestive of the pathology.

Chronic inflammatory demyelinating polyneuropathy and respiratory failure due to phrenic nerve involvement.

In this article, 2 patients with chronic inflammatory demyelinating polyneuropathy (CIDP) are presented. These patients developed respiratory failure which could not be explained by any cardiac or pulmonary pathology. The first case had pure motor involvement and probable CIDP, and the second case had severe sensorial dysfunction and an ataxic picture in addition to motor symptoms.

Myokymia and neuromyotonia in 37 Jack Russell terriers.

The clinical and clinicopathological characteristics, treatment and outcome of vermicular muscle contractions (myokymia) and generalized muscle stiffness (neuromyotonia) in 37 Jack Russell terriers were evaluated retrospectively. Thirty dogs were affected by both disorders, whereas seven were presented with myokymia and never developed neuromyotonia. Clinical signs started at the mean age of 8 months.

Electrophysiological findings in patients with adult tethered cord syndrome.

Aim: This study was planned for detailed evaluation of electrophysiological findings in patients with adult TCS.

Material And Methods: Patients were retrospectively assessed for clinical, radiological and electrophysiological data between 1999 and 2008. Tibial somatosensorial evoked potentials, needle electromyography, nerve conduction studies and late responses (H reflex and F response) were studied in thirty (1 female, 29 males) TCS patients diagnosed by lumbar magnetic resonance imaging.

Observations of recurrent laryngeal nerve injury and recovery using a rat model.

Objectives/hypothesis: To evaluate standardized recurrent laryngeal nerve (RLN) injuries using a rat model via minimally invasive transoral electromyography (ToL EMG) and histologic studies.

Methods: Forty-two female Sprague Dawley rats weighing 200 g to 250 g underwent crush injury to the right RLN using a calibrated pressure clip (0.61 N or 1.

Spina bifida occulta. Foot deformities, enuresis and vertebral cleft: clinical picture and neurophysiological assessment.

Aim: The aim of the study was to investigate the relationship between the clinical evidence of foot deformities in spina bifida occulta and the associated neurophysio-logical damage.

Methods: The authors studied 47 patients with foot deformities (37 flat foot, 10 pes cavus) and vertebral cleft, variably associated with enuresis, midline cutaneous lesions, and further orthopaedic deformities. An electrophysiological evaluation was performed in an attempt to investigate the peripheral nervous system in greater detail, including conventional motor and sensory nerve conduction, F-wave recording and electromyogram (EMG) testing.

Clinical approach to the weak patient in the intensive care unit.

Motor weakness in a patient in the intensive care unit (ICU) may be related to (1) pre-existing neuromuscular disorder that leads to ICU admission, (2) new-onset or previously undiagnosed neurological disorder, or (3) complications of non-neuromuscular critical illness. Neuromuscular syndromes related to ICU treatment consist of critical illness polyneuropathy, critical illness myopathy, and prolonged neuromuscular blockade, and are now recognized as a frequent cause of newly acquired weakness in ICU patients. Clinical features include quadriparesis, muscle wasting, and difficulty weaning from the ventilator.

Approach to neuromuscular disorders in the intensive care unit.

Neuromuscular disorders increasingly are recognized as a complication in patients in the intensive care unit (ICU) and represent a common cause of prolonged ventilator dependency. The distinct syndromes of critical illness myopathy, prolonged neuromuscular blockade, and critical illness polyneuropathy (CIP) may arise as a consequence of sepsis, multi-organ failure, and exposure to various medications--notably, intravenous corticosteroids and neuromuscular blocking agents--but the pathophysiology of these disorders remains poorly understood. More than one syndrome may occur simultaneously, and the distinctions may be difficult in a particular patient, but a specific diagnosis usually can be established after careful clinical, electrodiagnostic, and, when necessary, histological evaluation.