Biomedical subjects

J Y Hogrel

Publications and source records attributed to J Y Hogrel.

10 recordsLinked to original sources

PubMed2006

[Manual and quantitative muscle testing in neuromuscular disorders. How to assess the consistency of strength measurements in clinical trials?].

Evaluation of functional capacities of patients suffering of neuromuscular disorders, particularly muscle strength, is a critical issue for their diagnosis and follow-up. Within the framework of the natural history of any given disease, such an evaluation may improve the clinician's knowledge of the pathophysiological processes involved, and may help to anticipate and sometimes prevent deleterious consequences as the disease progresses. It is also helpful for identifying correlation between the severity of organic damage and the functional impact of the disease. The measurement of functional capacities must be done with accuracy, sensitivity and reliability, essentially when used as an outcome measure for therapeutic trials. Several evaluation tools for measuring muscle strength are available. They are usually classified into two groups: manual muscle testing (MMT) methods and quantified muscle testing (QMT) methods. In this article, we present the principles of strength measurements, and the different tools and materials that are commonly used in clinical settings. Their limitations and drawbacks are illustrated through several examples. Although QMT is theoretically and potentially more consistent than MMT to precisely follow the muscle capacities of the patients, precise and robust procedures must be elaborated and validated for each tested muscle function. Strength measurements must be performed by trained and experimented clinical evaluators. This issue is critical in the follow up of multicentric therapeutic trials. Inter-rater reliability must be assessed to guarantee the statistical power of the trial.

Clinical Trials as Topic↗

PubMed2005

[About a phase I gene therapy clinical trial with a full-length dystrophin gene-plasmid in Duchenne/Becker muscular dystrophy].

This is the first gene transfer trial in Duchenne/Becker patients. The aim of the study was to provide evidence on transgene expression and safety of the intramuscular administration of a plasmid containing a full-length dystrophin CDNA. Nine Duchenne/Becker patients, distributed in three cohorts of three patients, were injected into their radialis muscles either once with 200 microg (first cohort) or 600 microg (second cohort) or twice, two weeks apart, with 600 microg plasmidic DNA (third cohort). The patients were enrolled sequentially upon evaluation of the data by an independant pilot committee. In the biopsies taken three weeks after the initial injection from the injected site, the plasmid was detected in all patients. An exogenous dystrophin expression was found in 6/9 patients. The level of expression was low, up to 6 % of weak complete sarcolemmal labelling, and up to 26% of partial sarcolemmal staining. Dystrophin in RNAs were detected by nested RT-PCR in five out of the six biopsies with exogenous dystrophin-positive fibers. Interestingly, neither humoral or cellular antidystrophin responses were observed. No local or general adverse effects were seen. This paves the way for future developments in gene therapy in hereditary muscle diseases, and specifically in Duchenne/Becker myopathies.

Biopsy↗

PubMed2003

Histopathological differences of myotonic dystrophy type 1 (DM1) and PROMM/DM2.

Muscle biopsy findings in DM2 have been reported to be similar to those in DM1. The authors used myosin heavy chain immunohistochemistry and enzyme histochemistry for fiber type differentiation on muscle biopsies. Their results show that DM2 patients display a subpopulation of type 2 nuclear clump and other very small fibers and, hence, preferential type 2 fiber atrophy in contrast to type 1 fiber atrophy in DM1 patients.

Adult↗

PubMed2003

Automatic identification of motor unit action potential trains from electromyographic signals using fuzzy techniques.

A technique is proposed that allows automatic decomposition of electromyographic (EMG) signals into their constituent motor unit action potential trains (MUAPTs). A specific iterative algorithm with a classification method using fuzzy-logic techniques was developed. The proposed classification method takes into account imprecise information, such as waveform instability and irregular firing patterns, that is often encountered in EMG signals. Classification features were determined by the combining of time position and waveform information. Statistical analysis of inter-pulse intervals and spike amplitude provided an accurate estimation of features used in the classification step. Algorithm performance was evaluated using simulated EMG signals composed of up to six different discharging motor units corrupted with white noise. The algorithm was then applied to real signals recorded by a high spatial resolution surface EMG device based on a Laplacian spatial filter. On six groups of 20 simulated signals, the decomposition algorithm performed with a maximum and an average mean error rate of 2.13% and 1.37%, respectively. On real surface EMG signals recorded at different force levels (from 10% to 40% of the maximum voluntary contraction), the algorithm correctly identified 21 MUAPTs, compared with the 29 MUAPTs identified by an experienced neurophysiologist. The efficiency of the decomposition on surface EMG signals makes this method very attractive for non-invasive investigation of physiological muscle properties. However, it can also be used to decompose intramuscularly recorded EMG signals.

Action Potentials↗

PubMed2002

Motor unit conduction velocity distribution estimation: assessment of two short-term processing methods.

Muscle fibre conduction velocity (MFCV) can be used as an index of the structural and/or functional modifications that can occur during fatigue or pathological processes. Current evaluation of MFCV from surface electromyography (SEMG) classically produces an average value. However, a single mean value is not sufficient when modifications affect only a small part of the conduction velocity distribution. In such a case, an estimation of the whole motor unit conduction velocity distribution (MUCV) would be advantageous. The aim of this study was the evaluation of the quality of two short-term methods based on cross-correlation (CC) and peak-to-peak (PP) estimation. A comprehensive simulation program was used to generate signals with known MUCV distributions. The Dmax statistic of Kolmogorov-Smirnov was used as an error criterion to quantify the estimation error and to optimise the MUCV distribution computation algorithms. The minimum error was observed for an analysing window of 10ms for PP and 15ms for CC. Dmax was significantly lower for PP (0.195+/-0.054) than for CC (0.343+/-0.073). Various simulations showed the strong effect of the variance of the true distribution on the features of the estimated ones. Clinical data measured on the abductor pollicis brevis were studied. MUCV was estimated on a healthy subject (3.63+/-0.87ms(-1)), a patient suffering from a myopathy (2.73+/-0.51ms(-1)) and one suffering from a neuropathy (4.38+/-0.23ms(-1)). The results demonstrate the overall superiority of a peak-to-peak approach.

Adult↗

PubMed2001

A non-ischemic forearm exercise test for the screening of patients with exercise intolerance.

BACKGROUND: The forearm exercise test is a common investigation that allows detection of some metabolic myopathies. It is not completely standardized and, when performed in ischemic conditions, may induce rhabdomyolysis in patients with glycogenosis. OBJECTIVE: To develop a standardized non-ischemic exercise test for a safe screening of patients with exercise intolerance. METHODS: Twenty-six healthy subjects and 32 patients with exercise intolerance performed an isometric exercise at 70% of the maximal voluntary contraction during 30 seconds in non-ischemic conditions. Blood concentrations of creatine kinase, lactate, and ammonia were analyzed. RESULTS: A nearly fourfold lactate rise was induced by exercise in healthy subjects. All patients with normal muscle biopsy showed values similar to those of healthy subjects. No significant lactate increase was observed in six patients with a myophosphorylase defect and one with a debrancher defect. Disparate lactate responses were observed in 14 patients with a mitochondrial myopathy. The blood lactate level at rest was abnormally high in four of these patients. The lactate surface normalized by the mechanical energy production was above the normal range in eight patients. CONCLUSIONS: The authors propose a standardized non-ischemic grip test that overcomes the main drawbacks of the classic ischemic forearm exercise test. It provides a specific, efficient, and safe screening test for patients with exercise intolerance. Its sensitivity was very good for patients with a glycogenolysis defect but remains partial in patients with a mitochondrial disorder.

Adult↗

PubMed2001

Age-related morphological changes of the deltoid muscle from 50 to 79 years of age.

A quantitative analysis of deltoid muscle biopsy specimens was performed by light microscopy in 26 males and 25 females aged 50-79 years without known neuromuscular disease. Muscle fibre size, fibre type distribution and increase in mitochondrial content in muscle fibres were examined using a semi-automatic image analysis system. This study showed significant age- and gender-related differences. In females, there was marked atrophy of type II fibres with increasing age, specially of type IIb fibres, but no significant change in muscle fibre type distribution. In males, there was diminution in the relative proportion of type IIb fibres with increasing age but no significant muscle fibre atrophy. Mitochondrial aggregates increased with age, and this increase was observed earlier in females than in males. The gender-related morphological changes observed in the present study differ somewhat from those reported in the literature. The differences between males and females may be partially related to gender differences in muscular activity and may reflect an earlier decline in deltoid muscle strength in females. The existence of these age and gender changes should be taken into account in the interpretation of muscle biopsies of aged individuals.

Age Factors↗

PubMed2000

Fatigue of elbow flexors during repeated flexion-extension cycles: effect of movement strategy.

The objective of this work was to study the influence of movement strategy on fatigue of elbow flexors during repeated flexion-extension cycles in order to enlighten the fatigue process occurring during "pumping" in boardsailing. This dynamic exercise was performed by six high-level Olympic boardsailors according to two movement strategies with different amplitude and frequency. The parameters of the exercise were chosen to result in similar mechanical work at every instant for both modalities. Isometric evaluation was performed before and after dynamic exercise to quantify muscle fatigue. Analysis of physical parameters (maximum voluntary contraction, endurance time) and electromyographic parameters (in both temporal and frequency domains) emphasized the peripheral origin of muscle fatigue at the level of the biceps brachii and the brachioradialis. After considering the limitations of this type of study, the results are discussed in terms of synergy and differentiation between muscles, peripheral fatigue and movement strategy. Although the mechanical work and total physiological demands were similar for the two movement modalities, analysis of electromyographic parameters suggests that muscle fatigue mainly involves the biceps brachii when movements were slow and wide, and the brachioradialis when they were rapid and short. This study makes it possible to take these specificities into account in order to adjust the physical training program.

Adult↗

PubMed2000

A model of EMG generation.

Simulation models are unavoidable in experimental research when the point is to develop new processing algorithms to be applied on real signals in order to extract specific parameter values. Such algorithms have generally to be optimized by comparing true parameter values to those deduced from the algorithm. Only a simulation model can allow the user to access and control the actual process parameter values. This constraint is especially true when dealing with biomedical signals like surface electromyogram (SEMG). This work is an attempt to produce an efficient SEMG simulation model as a help for assessing algorithms related to SEMG features description. It takes into account the most important parameters which could influence these characteristics. This model includes all transformations from intracellular potential to surface recordings as well as a fast implementation of the extracellular potential computation. In addition, this model allows multiple graphically-programmable electrode-set configurations and SEMG simulation in both voluntary and elicited contractions.

Action Potentials↗

PubMed1998

Variability of some SEMG parameter estimates with electrode location.

Muscular action potential conduction velocity (CV) and mean power frequency (MPF) are commonly used parameters to describe the surface electromyographic signal (SEMG). The discrepancies concerning the behavior and interpretation of these main parameters in the literature have motivated this work. Our objective was to evaluate within- and between-individual reproducibility, sensitivity and variation of CV and MPF depending on the electrode location with respect to various contraction modalities. The results present evidence for significant influence of electrode location on CV and MPF, not only in their initial values but also in their changes during fatiguing efforts. This influence appears to be subject-dependent. This variability seems to be essentially due to the relative displacements of myotendinous and neuromuscular junctions with respect to the electrode set. Moreover, this study shows that MPF can be seen as force-dependent under certain conditions and that the CV-MPF relationship is strongly influenced by methodological factors. In conclusion, it seems irrelevant to derive reliable SEMG parameter estimates without considering electrode location. There is a strong need for proper standardization based on anatomical and methological aspects before attempting any individual characterization. Finally, we suggest a procedure for assessment of measurement quality.

Action Potentials↗