Isabelle Ledoux

Isabelle Ledoux
Institute of Myology

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25
Publications
4,398
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272
Citations

Publications

Publications (25)
Article
Full-text available
Objective To understand the natural disease upper limb progression over 3 years of ambulatory and non-ambulatory patients with Duchenne muscular dystrophy (DMD) using functional assessments and quantitative magnetic resonance imaging (MRI) and to exploratively identify prognostic factors. Methods Forty boys with DMD (22 non-ambulatory and 18 ambul...
Article
Full-text available
Objective The main aim was to explore the changes in hand-grip strength in patients with Duchenne muscular dystrophy (DMD) aged 5–29 years. Secondary aims were to test the effect of mutation, ambulatory status and glucocorticoid use on grip strength and its changes over time and to compute the number of subjects needed for a clinical trial to stabi...
Article
Full-text available
In myotonic dystrophy type 1, several studies have suggested causal relationships between CTG repeat length and the severity of symptoms, such as weakness or myotonia. We aimed to explore these relationships in a large population of 144 DM1 patients. All patients underwent clinical and functional assessments using a standardized test for grip stren...
Article
Background Adult polyglucosan body disease (APBD) is a progressive neurometabolic disorder caused by a deficiency of glycogen branching enzyme. We tested the efficacy of triheptanoin as a therapy for patients with APBD based on the hypothesis that decreased glycogen degradation leads to brain energy deficit. Methods and resultsThis was a two-site,...
Article
Aims: The non-ischaemic forearm exercise test (NIFET) is used as a diagnostic tool for the screening of patients with exercise intolerance and for the diagnosis of various metabolic muscle disorders. The production of lactate and ammonia are generally analysed to guide the diagnosis. The aim of this retrospective study was to determine the level o...
Article
Objective: To explore the value of nuclear magnetic resonance (NMR) and functional assessments for follow-up of ambulatory and nonambulatory patients with Duchenne muscular dystrophy (DMD). Methods: Twenty-five 53-skippable patients with DMD were included in this study; 15 were nonambulatory at baseline. All patients underwent clinical and funct...
Article
This was a retrospective study to assess the diagnostic value of the non-ischaemic forearm exercise test in detecting McArdle's disease. The study is a retrospective diagnostic study over 15 years (1999-2013) on a referred sample of patients suffering from exercise intolerance and various muscle complaints, generally with elevated creatine kinase (...
Article
Full-text available
Background Wrist movements become impaired with disease progression in various neuromuscular disorders. With the development of new therapies, thorough measurement of muscle strength is crucial to document natural disease progression and to assess treatment efficacy. We developed a new dynamometer enabling wrist flexion and extension torque measure...
Conference Paper
Myotonic dystrophy type 1 (DM1) is an autosomal dominant disease for which treatments are being developed. There is a need to define reliable and reproducible criteria to evaluate neuromuscular function in order to assess their effects. The main aim of our study consists of a follow-up of the natural history of DM1 patients over 3 years. Here, base...
Article
In the past fifteen years, more than 1200 patients underwent the non-ischemic forearm exercise test (NIFET) in our referral center as a first screening tool for the detection of metabolic myopathies. Finally 40 patients were diagnosed so far with a McArdle disease (glycogenosis type V). As a consequence of the absence of phosphorylase enzyme, no ri...
Article
Myotonic dystrophy is the most common adult muscle dystrophy. In view of emerging therapies, which use animal models as a proof of principle, the development of reliable outcome measures for in vivo longitudinal study of mouse skeletal muscle function is becoming crucial. To satisfy this need, we have developed a device to measure ankle dorsi- and...
Article
Full-text available
Background Ankle strength is often impaired in some of the most common neuromuscular disorders. Consequently, strength generated around this joint is important to assess, because it has a great impact on balance and gait. The objectives of this study were to establish normative data and predictive equations for both ankle dorsi- and plantar-flexion...
Article
Most of the neuromuscular diseases induce changes in muscle fibre characteristics. For example, Duchenne dystrophy is characterized by a specific loss of fast fibres, and an increase in small diameter fibres. These morphological changes may lead to large modifications in the distribution of fibre diameters, possibly producing bimodal distributions....
Article
Precise, sensitive muscle strength testing methods are needed to investigate muscle function in patients with neuromuscular disorders (NMD). Here, we describe an isokinetic knee flexor and extensor testing procedure using the Biodex 3's continuous passive motion (CPM) mode. The torque values recorded during passive isokinetic motion were subtracted...
Article
Action potentials travel along the muscle fibers with a specific conduction velocity that depends on their structural and functional properties. Only the estimation of muscle conduction velocity distribution (MCVD) may be able to depict this propagation heterogeneity. Based on the method proposed by Cummins et al. (Electroenceph Clin Neurophysiol,...

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