scholarly journals Application Value of the Motor Unit Number Index in Patients With Kennedy Disease

2021 ◽  
Vol 12 ◽  
Author(s):  
Shuo Zhang ◽  
Xin Yang ◽  
Yingsheng Xu ◽  
Yongmei Luo ◽  
Dongsheng Fan ◽  
...  
Keyword(s):  
Motor Unit ◽  
Rating Scale ◽  
Muscular Atrophy ◽  
Sum Score ◽  
Objective Parameter ◽  
Compound Muscle Action Potentials ◽  
Size Index ◽  
Axonal Collateral ◽  
Abductor Digiti Minimi ◽  
Sum Scores

The aim of this study was to evaluate the usefulness of the motor unit number index (MUNIX) technique in Kennedy disease (KD) and test the correlation between the MUNIX and other clinical parameters. The MUNIX values of the bilateral deltoid, abductor digiti minimi (ADM), quadriceps femoris (QF), and tibialis anterior (TA) were determined and compared with the course of the disease. The MUNIX sum score was calculated by adding the MUNIX values of these 8 muscles. Disability was evaluated using the spinal and bulbar muscular atrophy functional rating scale (SBMAFRS). The MUNIX scores of patients with KD were negatively correlated with the course of the disease (p < 0.05), whereas their motor unit size index (MUSIX) scores were positively correlated with the course the of disease (p < 0.05). MUNIX sum scores were correlated with SBMAFRS scores (r = 0.714, p < 0.05). MUNIX was more sensitive than compound muscle action potentials or muscle strength as an indicator of neuron loss and axonal collateral reinnervation. The MUNIX sum score is an objective and a reliable indicator of disease progression, and it is a potential choice for therapeutic clinical trials. The MUNIX can assess the functional loss of motor axons and is correlated with disability. The MUNIX sum score may be especially suitable as an objective parameter.

scholarly journals Motor unit number estimation versus compound muscle action potential in the evaluation of motor unit loss in amyotrophic lateral sclerosis

2021 ◽  
Vol 57 (1) ◽  
Author(s):  
Arwa Khalid Ibrahem ◽  
Akram Mohammed Al-Mahdawi ◽  
Farqad Bader Hamdan
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Unit ◽  
Rating Scale ◽  
Compound Muscle Action Potential ◽  
Motor Units ◽  
Case Detection ◽  
Muscle Action ◽  
Compound Muscle Action Potentials ◽  
Rate Of Decline ◽  
Lateral Sclerosis

Abstract Introduction Motor neuron loss is the primary pathologic feature of amyotrophic lateral sclerosis (ALS). An estimate of the number of surviving motor units (MUs) represents a direct measure of the disease state in ALS. The objective of the study is to compare MU number estimation (MUNE) using the multipoint stimulation method (MPS) and compound muscle action potentials (CMAP) amplitude in patients with ALS. Methods Twenty-eight patients with ALS with a disease duration of 3–48 months were studied. Nerve conduction study of the median, ulnar, tibial, common peroneal, and sural nerves were done. Besides, electromyography (EMG) of cranio-bulbar, cervical, thoracic and lumbosacral muscles, and MPS-MUNE. Results MUNE is decreased in patients with ALS. MUNE was positively correlated with CMAP amplitude, medical research council (MRC) scale, and ALS functional rating scale (ALS-FRS). On the contrary, MUNE was negatively correlated with MUAP duration. Case detection by the MUNE methods was high as compared to that of CMAP amplitude. Conclusions MUNE is highly specific and more sensitive than CMAP amplitude in detecting the neurophysiologic abnormalities in patients with ALS. Case detection by MUNE is three times more than CMAP amplitudes. The rate of decline of motor units using the MPS-MUNE is more sensitive than the MRC score and ALSFRS-R when expressed as the percentage change from baseline.

scholarly journals Safety, Tolerability, and Effect of Nusinersen Treatment in Ambulatory Adults With 5q-SMA

2021 ◽  
Vol 12 ◽  
Author(s):  
Bakri Elsheikh ◽  
Steven Severyn ◽  
Songzhu Zhao ◽  
David Kline ◽  
Matthew Linsenmayer ◽  
...  
Keyword(s):  
Adverse Events ◽  
Motor Unit ◽  
Rating Scale ◽  
Muscular Atrophy ◽  
Compound Muscle Action Potential ◽  
Motor Unit Potential ◽  
Serious Adverse Events ◽  
Significant Treatment ◽  
Single Motor Unit ◽  
Isometric Muscle Contraction

Objective: To determine the safety and tolerability of nusinersen treatment in ambulatory adults with spinal muscular atrophy (SMA) and investigate the treatment effect on muscle strength, physical function, and motor unit physiology.Methods: Individuals aged 18 years or older with genetically confirmed 5q SMA, three or more copies of the SMN2 gene, and the ability to ambulate 30 feet were enrolled. Safety outcomes included the number of adverse events and serious adverse events, clinically significant vital sign or laboratory parameter abnormalities. Outcome assessments occurred at baseline (prior to the first dose of nusinersen) and then 2, 6, 10, and 14 months post-treatment.Results: Six women, seven men (mean age: 37 ± 11, range: 18–59 years) were included for analyses. The most common side effects were headache and back pain, but overall procedures and treatments were well-tolerated. No serious adverse events were reported. Maximal Voluntary Isometric Muscle Contraction Testing (MVICT) and 6-min walk test (6MWT) both showed overall stability with significant increases at 2, 6, and 10 months for the 6MWT. More consistent significant treatment effects were noted on the Hammersmith Functional Motor Scale Expanded, SMA-Functional Rating Scale, and forced vital capacity. Treatment resulted in progressively increased ulnar compound muscle action potential and average single motor unit potential amplitudes, but motor unit number estimation remained stable.Conclusions: Nusinersen treatment is safe and well-tolerated in ambulatory adults with SMA. Treatment resulted in improved motor function and electrophysiological findings suggest that this improvement may be occurring via improved motor unit reinnervation capacity.

Psychological networks in clinical populations: investigating the consequences of Berkson's bias

2019 ◽  
pp. 1-9 ◽  
Author(s):  
Jill de Ron ◽  
Eiko I. Fried ◽  
Sacha Epskamp
Keyword(s):  
Network Structure ◽  
Binary Data ◽  
Ordinal Data ◽  
Graphical Model ◽  
Rating Scale ◽  
Network Models ◽  
Simulation Studies ◽  
Potential Threat ◽  
Sum Score ◽  
Research Populations

Abstract Background In clinical research, populations are often selected on the sum-score of diagnostic criteria such as symptoms. Estimating statistical models where a subset of the data is selected based on a function of the analyzed variables introduces Berkson's bias, which presents a potential threat to the validity of findings in the clinical literature. The aim of the present paper is to investigate the effect of Berkson's bias on the performance of the two most commonly used psychological network models: the Gaussian Graphical Model (GGM) for continuous and ordinal data, and the Ising Model for binary data. Methods In two simulation studies, we test how well the two models recover a true network structure when estimation is based on a subset of the data typically seen in clinical studies. The network is based on a dataset of 2807 patients diagnosed with major depression, and nodes in the network are items from the Hamilton Rating Scale for Depression (HRSD). The simulation studies test different scenarios by varying (1) sample size and (2) the cut-off value of the sum-score which governs the selection of participants. Results The results of both studies indicate that higher cut-off values are associated with worse recovery of the network structure. As expected from the Berkson's bias literature, selection reduced recovery rates by inducing negative connections between the items. Conclusion Our findings provide evidence that Berkson's bias is a considerable and underappreciated problem in the clinical network literature. Furthermore, we discuss potential solutions to circumvent Berkson's bias and their pitfalls.

scholarly journals POS0258 REAL-TIME VERSUS STATIC SCORING IN MUSCULOSKELETAL ULTRASONOGRAPHY IN PATIENTS WITH INFLAMMATORY HAND OSTEOARTHRITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 351.2-352
Author(s):  
L. Van de Stadt ◽  
F. Kroon ◽  
M. Reijnierse ◽  
D. Van der Heijde ◽  
F. Rosendaal ◽  
...  
Keyword(s):  
Real Time ◽  
Doppler Signal ◽  
Hand Osteoarthritis ◽  
Scoring Methods ◽  
Research Support ◽  
Patient Level ◽  
Sum Score ◽  
Absolute Agreement ◽  
Sum Scores ◽  
Real Time Ultrasonography

Background:Ultrasound (US) is used in rheumatic musculoskeletal diseases (RMDs) such as hand osteoarthritis (OA) as outcome measure. Traditionally scoring is performed real-time, but central reading of static US images could avoid issues of inter-rater reliability. However, agreement between real-time and static assessment has not been studiedObjectives:To study the agreement between real-time and static scoring of US in inflammatory hand OA.Methods:Ultrasound was performed of 30 joints obtained in 75 patients with hand osteoarthritis, treated with prednisolone or placebo in a randomized double-blind trial. Hand joints were assessed for synovial thickening, effusion, Doppler signal and osteophytes by ultrasound (score 0-3 per joint) at baseline and after treatment. Two ultrasonographers blinded for clinical data scored the live images together (simultaneously) in real-time. A consensus score for each joint was recorded. Representative images stored during scanning were scored by one ultrasonographer minimally 6 months after real-time scoring. For each patient, images of each visit were scored paired, with known chronological order.Agreement between scoring methods was studied at joint level with quadratic weighted kappa. At patient level, intra-class correlations (ICC; mixed effect model, absolute agreement, with clustering taken into account) were calculated at both timepoints. ICCs were also calculated for the delta of sum scores. Responsiveness of scoring methods was analyzed with generalized estimating equations (GEE) with treatment as independent and ultrasonography findings as dependent variable.Results:Thirty-nine patients (52%) were treated with prednisolone and 36 (48%) were treated with placebo. Patient characteristics were well-balanced between treatment groups.All patients had signs of synovial thickening and osteophytes as assessed by real-time ultrasonography, and almost all signs of effusion (99%) or a positive Doppler signal (95%) in at least one joint. Total ultrasonography sum score for osteophytes was high (mean 45 ±SD 12), whereas sum score was low for positive Doppler signal (mean 5.9 ±SD 4.4), with intermediate sum scores for synovial thickening and effusion (mean 16 ±SD 6.3 and 11 ±SD 6.0 respectively). Static sum scores were overall slightly higher (osteophytes mean 48 ±SD 10; Doppler mean 6.9 S±D 5.0; synovial thickening mean 20 ±SD 7.0 and effusion 13 ±SD 6.5)Agreement at baseline was good to excellent at joint level (kappa 0.72-0.88) and moderate to excellent at patient level (ICC 0.59-0.86). Agreement for delta sum scores was poor to fair for synovial thickening and effusion (ICC 0.18 and 0.34 respectively), but excellent for Doppler signal (ICC 0.80) (Table 1).Real-time ultrasonography showed responsiveness to prednisolone with a mean between-group difference of synovial thickening sum score of -2.5 (CI:-4.7 to-0.3). Static ultrasonography did not show a decrease in synovial thickening (Figure 1). No difference in ultrasonography scores was seen for the other ultrasonography features, neither with real-time nor static scoring.Conclusion:While cross-sectional agreement between real-time and static ultrasonography was good, agreement of delta sum scores was not and paired static ultrasonography measurement of synovial thickening did not show responsiveness to prednisone therapy where real-time ultrasonography did. Therefore, when using ultrasonography in clinical trials, real-time dynamic scoring should remain the standard.Table 1.Agreement on patient levelBaselineWeek 6Delta W6-BLICC (95% CI)ICC (95% CI)ICC (95% CI)Synovitis0.59 (0.26-0.76)0.58 (0.24-0.77)0.18 (0 - 0.40)Effusion0.84 (0.66-0.92)0.84 (0.75-0.89)0.34 (0.12-0.53)Osteophytes0.82 (0.50-0.92)0.78 (0.56-0.88)NDDoppler0.86 (0.75-0.92)0.91 (0.85-0.94)0.80 (0.70 -0.87)ICC: intra-class correlation coefficient linear mixed model (random patient, fixed rating), absolute agreement. ND: Not DerterminedDisclosure of Interests:Lotte van de Stadt: None declared, Féline Kroon: None declared, Monique Reijnierse Grant/research support from: Dutch Arthritis Foundation, Désirée van der Heijde Consultant of: bbVie, Amgen, Astellas, AstraZeneca, Bayer, BMS, Boehringer Ingelheim, Celgene, Cyxone, Daiichi, Eisai, Eli-Lilly, Galapagos, Gilead, Glaxo-Smith-Kline, Janssen, Merck, Novartis, Pfizer, Regeneron, Roche, Sanofi, Takeda, UCB Pharma, Frits Rosendaal: None declared, Naghmeh Riyazi: None declared, R. de Slegte: None declared, Jende van Zeben: None declared, Cornelia Allaart: None declared, Margreet Kloppenburg Consultant of: Abbvie, Pfizer, Levicept, GlaxoSmithKline, Merck-Serono, Kiniksa, Flexion, Galapagos, Jansen, CHDR, Grant/research support from: MI-APPROACH, Marion Kortekaas: None declared

scholarly journals Burnout and Its Relationship with Demographic and Job-Related Variables among Dentists in Lithuania: A Cross-Sectional Study

2021 ◽  
Vol 18 (8) ◽  
pp. 3968
Author(s):  
Eglė Slabšinskienė ◽  
Andrej Gorelik ◽  
Aistė Kavaliauskienė ◽  
Apolinaras Zaborskis
Keyword(s):  
Job Satisfaction ◽  
Emotional Exhaustion ◽  
Maslach Burnout Inventory ◽  
Cross Sectional Study ◽  
Personal Accomplishment ◽  
Cross Sectional ◽  
Satisfaction Index ◽  
Sum Score ◽  
Sum Scores ◽  
High Level

Although burnout has been described as a serious hazard for personal and professional lives and has been surveyed among dentists in many countries, no study has been published regarding burnout among dentists in Lithuania. This study aimed to evaluate the burnout level among Lithuanian dentists and its association with demographic variables, job satisfaction, and other job-related variables. The data were collected among dentists online or during professional conferences while using an anonymous questionnaire (n = 380). The Maslach Burnout Inventory (MBI) was used to evaluate the burnout level. A Poisson regression was applied for the analysis of relationships between variables. We observed that 42.3% of the respondents had a high emotional exhaustion (EE) (95% confidence interval (CI): 37.4–42.3%), while 18.7% (95% CI: 15.0–22.9%) and 28,2% (95% CI: 23.4–32.6%) had high depersonalization (DP) and low personal accomplishment (PA), respectively. Nonetheless, 15.3% (95% CI: 11.8–18.9%) of the study population experienced a high level of overall burnout. An original job satisfaction index was elaborated. It was significantly associated with sum scores of all burnout dimensions: with the EE sum score (Ratio of Sum Score Means (RSSM) 1.54; 95% CI: 1.46–1.62), DP sum score (RSSM 1.59; 95% CI: 1.45–1.74), and PA sum score (RSSM 0.88; 95% CI: 0.84–0.92). It was concluded that Lithuanian dentists can be characterised by high burnout intensity and high prevalence of burnout, being especially evident in emotional exhaustion. The dentist with low job satisfaction appeared to be the most vulnerable to all burnout dimensions.

scholarly journals Clinical Determinants of Disease Progression in Amyotrophic Lateral Sclerosis—A Retrospective Cohort Study

2021 ◽  
Vol 10 (8) ◽  
pp. 1623
Author(s):  
Maria Viktoria Requardt ◽  
Dennis Görlich ◽  
Torsten Grehl ◽  
Matthias Boentert
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
Disease Progression ◽  
Rating Scale ◽  
Rapid Decline ◽  
Chronic Obstructive ◽  
Obstructive Pulmonary Disease ◽  
Hazard Ratios ◽  
Sum Score ◽  
Lateral Sclerosis

Background: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that is ultimately fatal but characterized by substantial phenotypic heterogeneity, which is known to impact long-term course and survival. This study investigated clinical determinants of disease progression and outcome in a large cohort of patients with ALS. Methods: Retrospective analysis included comprehensive data from 625 patients who attended a tertiary ALS centre at least twice. Patients were stratified according to five distinct clinical phenotypes: classical ALS; bulbar ALS; ALS with frontotemporal dementia (ALS-FTD); upper motor neuron predominant (UMNP); and lower motor neuron predominant (LMNP). Results: This study confirmed higher age at symptom onset, shorter latency to diagnosis and more rapid decline in the revised ALS Functional Rating Scale sum score as predictors of poor prognosis. Hazard ratios for shorter survival were higher in patients with ALS-FTD versus classical ALS, and in patients with versus without chronic obstructive pulmonary disease (COPD). Mean survival was longest in the UMNP phenotype group. Conclusions: This study confirmed established predictors of shorter survival in ALS and showed that concomitant COPD in particular relates to poor outcome.

scholarly journals Motor Unit Number Estimation in Neuromuscular Disease

2008 ◽  
Vol 35 (2) ◽  
pp. 153-159 ◽  
Author(s):  
Omid Rashidipour ◽  
K. Ming Chan
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Unit ◽  
Neuromuscular Disease ◽  
Muscular Atrophy ◽  
Neuromuscular Diseases ◽  
Neuronal Loss ◽  
Response To Treatment ◽  
Motor Unit Number Estimation ◽  
Electrophysiological Method ◽  
Lateral Sclerosis

Motor unit number estimation (MUNE) is an electrophysiological method designed to quantify motor unit loss in target muscles of interest. Most of the techniques are noninvasive and are therefore well suited for longitudinal monitoring. In this brief review, we describe the more commonly used techniques and their applications in amyotrophic lateral sclerosis, poliomyelitis, spinal muscular atrophy and hereditary sensorimotor neuropathies. Findings in some of these studies offer important pathophysiological insights. Since conventional electrophysiologic methods are not sensible measures of motor neuronal loss, MUNE could play a potentially important role in the diagnosis, monitoring of disease progression and response to treatment in neuromuscular diseases in which motor unit loss is a major feature.

scholarly journals Psychological Networks in Clinical Populations: A tutorial on the consequences of Berkson's Bias

2019 ◽  
Author(s):  
Jill de Ron ◽  
Eiko I Fried ◽  
Sacha Epskamp
Keyword(s):  
Network Structure ◽  
Binary Data ◽  
Ordinal Data ◽  
Graphical Model ◽  
Rating Scale ◽  
Network Models ◽  
Simulation Studies ◽  
Potential Threat ◽  
Sum Score ◽  
Research Populations

In clinical research, populations are often selected on the sum-score of diagnostic criteria such as symptoms. Estimating statistical models where a subset of the data is selected based on a function of the analyzed variables introduces Berkson’s bias, which presents a potential threat to the validity of findings in the clinical literature. The aim of the present paper is to investigate the effect of Berkson’s bias on the performance of the two most commonly used psychological network models: the Gaussian Graphical Model (GGM) for continuous and ordinal data, and the Ising Model for binary data. In two simulation studies, we test how well the two models recover a true network structure when estimation is based on a subset of the data typically seen in clinical studies. The network is based on a dataset of 2,807 patients diagnosed with major depression, and nodes in the network are items from the Hamilton Rating Scale for Depression (HRSD). The simulation studies test different scenarios by varying (1) sample size and (2) the cut-off value of the sum-score which governs the selection of participants. The results of both studies indicate that higher cut-off values are associated with worse recovery of the network structure. As expected from the Berkson’s bias literature, selection reduced recovery rates by inducing negative connections between the items. Our findings provide evidence that Berkson’s bias is a considerable and underappreciated problem in the clinical network literature. Furthermore, we discuss potential solutions to circumvent Berkson’s bias and their pitfalls.

scholarly journals Estimating Aggregate Environmental Risk Score in Psychiatry: The Exposome Score for Schizophrenia

2021 ◽  
Vol 12 ◽  
Author(s):  
Lotta-Katrin Pries ◽  
Gamze Erzin ◽  
Bart P. F. Rutten ◽  
Jim van Os ◽  
Sinan Guloksuz
Keyword(s):  
Risk Score ◽  
Environmental Risk ◽  
Meta Analysis ◽  
Population Based ◽  
Training Dataset ◽  
Comprehensive Overview ◽  
Sum Score ◽  
Spectrum Disorders ◽  
Sum Scores ◽  
Meta Analyses

To understand the role of environment in the pathoetiology of psychosis spectrum disorders, research has thus far mainly investigated the effects of single exposures in isolation, such as the association between cannabis use and schizophrenia. However, this approach fails to acknowledge the complexity of the exposome, which represents the totality of the environment involving many exposures over an individual's lifetime. Therefore, contemporary research adopting the exposome paradigm has aimed at capturing the combined effect of different environmental exposures by utilizing an aggregate environmental vulnerability score for schizophrenia: the exposome score for schizophrenia. Here, we attempt to provide a comprehensive overview of studies applying the exposome score for schizophrenia. First, we describe several approaches estimating exposomic vulnerability for schizophrenia, which falls into three categories: simple environmental sum scores (sum of dichotomized exposures), meta-analysis-based environmental risk score (sum scores weighted by estimates from meta-analyses), and the exposome score (sum score weighted by estimates from an analysis in an independent training dataset). Studies show that the exposome score for schizophrenia that assumes interdependency of exposures performs better than scores that assume independence of exposures, such as the environmental sum score and the meta-analysis-based environmental risk score. Second, we discuss findings on the pluripotency of the exposome score for schizophrenia and summarize findings from gene-environment studies using the exposome score for schizophrenia. Finally, we discuss possible scientific, clinical, and population-based applications of exposome score for schizophrenia, as well as limitations and future directions for exposome research to understand the etiology of psychosis spectrum disorders.

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