Differentiation Between Amyotrophic Lateral Sclerosis and Mimics Using Quantitative Analysis of Fsciculation with Muscle Ultrasound.

Objective To determine the diagnostic accuracy of the intensity of fasciculation evaluated by muscle ultrasound in the differential diagnosis of amyotrophic lateral sclerosis (ALS). Methods We prospectively recruited patients who had ALS and neuropathy-radiculopathy attending Peking Union Medical College Hospital from 2017 to 2020. Healthy adults from a community were recruited as healthy controls.

Clinical recommendations for perioperative immunotherapy-induced adverse events in patients with non-small cell lung cancer.

Perioperative adjuvant treatment has become an increasingly important aspect of the management of patients with non-small cell lung cancer (NSCLC). In particular, the success of immune checkpoint inhibitors, such as antibodies against PD-1 and PD-L1, in patients with lung cancer has increased our expectations for the success of these therapeutics as neoadjuvant immunotherapy. Neoadjuvant therapy is widely used in patients with resectable stage IIIA NSCLC and can reduce primary tumor and lymph node stage, improve the complete resection rate, and eliminate microsatellite foci; however, complete pathological response is rare.

A Comprehensive Analysis of 2013 Dystrophinopathies in China: A Report From National Rare Disease Center.

Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are X-linked recessive neuromuscular disorders caused by mutations in . A high-quality database of DMD/BMD is essential not only for clinical practice but also for fundamental research. Here, we aimed to build the largest Chinese national dystrophinopathy database using the National Rare Diseases Registry System of China.

Effectiveness of repetitive transcranial magnetic stimulation (rTMS) after acute stroke: A one-year longitudinal randomized trial.

Aims: To evaluate the effectiveness of repetitive transcranial magnetic stimulation (rTMS) on motor recovery after stroke using a prospective, double-blind, randomized, sham-controlled study.

Methods: Patients with unilateral subcortical infarction in the middle cerebral artery territory within 1 week after onset were enrolled. The patients were randomly divided into an rTMS treatment group and a sham group.

The Awaji criteria increases the diagnostic sensitivity of the revised El Escorial criteria for amyotrophic lateral sclerosis diagnosis in a Chinese population.

Objectives: The accurate and early diagnosis of amyotrophic lateral sclerosis (ALS) is important for extending the life expectancy of patients. However, previous studies that have assessed the diagnostic sensitivities of the Awaji criteria (AC) and the revised El Escorial criteria (rEEC) in patients with ALS have been inconsistent, most of them were consensual regarding the advantage of Awaji over conventional criteria. Our study sought to compare the roles of AC and rEEC in the diagnosis of ALS.

Cerebral Functional Reorganization in Ischemic Stroke after Repetitive Transcranial Magnetic Stimulation: An fMRI Study.

Aims: Our study aimed to figure out brain functional reorganization evidence after repetitive transcranial magnetic stimulation (rTMS) using the resting-state functional magnetic resonance imaging (rsfMRI).

Methods: Twelve patients with unilateral subcortex lesion in the middle cerebral artery territory were recruited. Seven of them received a 10-day rTMS treatment beginning at about 5 days after stroke onset.

13,14-seco-Withanolides from Physalis minima with Potential Anti-inflammatory Activity.

Four new 13,14-seco-withanolides, minisecolides A - D (1 - 4), together with three known analogues 5 - 7, were isolated from the whole plants of Physalis minima. The structures of new compounds were determined on the basis of spectroscopic analysis, including (1) H-, (13) C-NMR, 2D-NMR (HMBC, HSQC, ROESY), and HR-ESI-MS. Evaluation of all isolates for their inhibitory effects on nitric oxide (NO) production was conducted on lipopolysaccaride-activated RAW264.

Rapidly Progressive Polyneuropathy in a Patient With Monoclonal Gammopathy: A Case Report of POEMS Syndrome and Beyond.

Neuropathy, the dominant clinical feature of POEMS syndrome, is typically distal, symmetric, and slowly progressive with demyelinating changes. After a gradual proximal spread, it usually results in severe muscle weakness and functional disabilities. Cases characterized by acute onset polyneuropathy are rarely described.

Quantitating Changes in Jitter and Spike Number Using Concentric Needle Electrodes in Amyotrophic Lateral Sclerosis Patients.

Background: Single-fiber electromyography (SFEMG) has been suggested as a quantitative method for supporting chronic partial denervation in amyotrophic lateral sclerosis (ALS) by the revised EI Escorial criteria. Although concentric needle (CN) electrodes have been used to assess jitter in myasthenia gravis patients and healthy controls, there are few reports using CN electrodes to assess motor unit instability and denervation in neurogenic diseases. The aim of this study was to determine whether quantitative changes in jitter and spike number using CN electrodes could be used for ALS studies.

Pattern Differences of Small Hand Muscle Atrophy in Amyotrophic Lateral Sclerosis and Mimic Disorders.

Background: Amyotrophic lateral sclerosis (ALS) and some mimic disorders, such as distal-type cervical spondylotic amyotrophy (CSA), Hirayama disease (HD), and spinobulbar muscular atrophy (SBMA) may present with intrinsic hand muscle atrophy. This study aimed to investigate different patterns of small hand muscle involvement in ALS and some mimic disorders.

Methods: We compared the abductor digiti minimi/abductor pollicis brevis (ADM/APB) compound muscle action potential (CMAP) ratios between 200 ALS patients, 95 patients with distal-type CSA, 88 HD patients, 43 SBMA patients, and 150 normal controls.

Asynchronization in Changes of Electrophysiology and Pathology of Spinal Cord Motor Neurons in Rats Following Middle Cerebral Artery Occlusion.

Background: Motor dysfunction is common in stroke patients. Clinical electrophysiological studies suggest that transsynaptic degeneration occurred in the lower motor neurons, while pathological evidence is lacked. This study aimed to combine the electrophysiological and pathological results to prove the existence of transsynaptic degeneration in the motor system after stroke.

Single-fiber Electromyography in the Extensor Digitorum Communis for the Predictive Prognosis of Ocular Myasthenia Gravis: A Retrospective Study of 102 Cases.

Background: Single-fiber electromyography (SFEMG) abnormality in the extensor digitorum communis (EDC) was reported in ocular myasthenia gravis (OMG), which indicated subclinical involvement beyond extraocular muscles in OMG patients. The relationship between the abnormal findings of SFEMG in EDC and the probability for OMG to develop generalized myasthenia gravis (GMG) is unknown. This retrospective study aimed to determine the predictive value of abnormality of SFEMG in EDC of OMG patients.

F Wave Study in Amyotrophic Lateral Sclerosis: Assessment of Segmental Motoneuronal Dysfunction.

Background: Dysfunctional spinal circuit may play a role in the pathophysiology of amyotrophic lateral sclerosis (ALS). The purpose of this study was to use F waves for assessment of segmental motoneuronal excitability following upper motor neuron (UMN) dysfunctions in ALS.

Methods: We studied the F waves of 152 ulnar nerves recorded from abductor digiti minimi in 82 patients with ALS.

Importance of sample size for the estimation of repeater F waves in amyotrophic lateral sclerosis.

Background: In amyotrophic lateral sclerosis (ALS), repeater F waves are increased. Accurate assessment of repeater F waves requires an adequate sample size.

Methods: We studied the F waves of left ulnar nerves in ALS patients.

Withanolides from Physalis minima and their inhibitory effects on nitric oxide production.

Six new withanolides (1-6), including two uncommon 1,10-seco withanolides (1 and 2), together with five known withanolides (7-11), were isolated from the whole plants of Physalis minima Linn.. The structures of new compounds were elucidated through spectroscopic methods, including (1)H, (13)C NMR, 2D-NMR, HRESIMS and circular dichroism (CD).

A clinical neurophysiology study of Hirayama disease.

Background: Hirayama disease is a rare disease characterized by juvenile-onset of asymmetric amyotrophy, of which etiology has not been clarified. The aim of our study was to investigate the clinical and neurophysiologic characteristics of Hirayama disease.

Methods: Neurophysiological tests, including nerve conduction studies (NCS), F-wave and routine electromyography (EMG), were performed in seventy-three patients with Hirayama disease.

[Value of anal sphincter electromyography, orthostatic hypotension and dizziness in diagnosing multiple system atrophy].

Objective: To explore the value of anal sphincter electromyography (ASEMG), orthostatic hypotension (OH), and dizziness in diagnosing multiple system atrophy (MSA).

Method: The characteristics of ASEMG and OH were compared among patients with dizziness (MSA and non-MSA), patients without OH (MSA and non-MSA), and patients with probable MSA (OH and non-OH).

Results: Totally 476 patients underwent ASEMG examinations.

[Clinical and electrophysiological characteristics of myasthenia gravis with thymoma: analysis of 72 patients].

Objective: To investigate the clinical and electrophysiological characteristics of myasthenia gravis with thymoma (MGT).

Methods: The clinical and repetitive nerve stimulation (RNS) data of 72 patients with MGT, 36 males and 36 females, and 63 patients with myasthenia gravis without thymoma (NMGT) were analyzed retrospectively.

Results: The onset age of 52 of the 72 MGT patients (72.

Effects of transcranial magnetic stimulation on motor cortical excitability and neurofunction after cerebral ischemia-reperfusion injury in rats.

Objective: To clarify the effects of repetitive transcranial magnetic stimulation (rTMS) on rat motor cortical excitability and neurofunction after cerebral ischemia-reperfusion injury.

Methods: After determined awake resting motor threshold (MT) and motor evoked potentials (MEPs) of right hindlimbs, 20 Sprague-Dawley rats were subjected to middle cerebral artery occlusion (MCAO) reperfusion injury, then rTMS were applied to rTMS group (n=10) at different time, while control group (n=10) received no stimulation. A week later, MT and MEPs were evaluated again, as well as neurological deficits and infarct volume.

Tumor necrosis factor-alpha polymorphism and secretion in myasthenia gravis.

Objective: To analyze the relationship between tumor necrosis factor-alpha (TNFalpha) gene promoter -308 polymorphism and myasthenia gravis (MG) in Chinese and analyze secretion of TNFalpha in peripheral blood mononuclear cells (PBMC) in MG patients.

Methods: A biallelic polymorphism at position -308 in the promoter of TNFalpha gene was screened by PCR amplification and NcoI recognition site. One hundred and twenty-three MG cases and 115 healthy controls were included in this study.

Single fiber electromyography in the diagnosis of ocular myasthenia gravis: report of 90 cases.

Background: The aim of this study was to evaluate single fiber electromyography (SFEMG) in the diagnosis of ocular myasthenia gravis (OMG), compared with repetitive nerve stimulation (RNS) and blood concentration of antibody to acetylcholine receptor (AchRAb).

Methods: SFEMG, RNS and AchRAb titration were measured in 90 patients with OMG (44 men, 46 women).

Results: Markedly increased jitter and ratio of block in the frontalis and the extensor digitorum communis (EDC) were observed (83.