Clinical applications of surface electromyography in neuromuscular disorders

JY Hogrel - Neurophysiologie Clinique/Clinical Neurophysiology, 2005 - Elsevier
Surface electromyography (SEMG) is still rarely used in clinical settings for the detection and
analysis of myoelectric signals. The electromyographic signal detected on the skin surface …

Update on outcome assessment in myositis

LG Rider, R Aggarwal, PM Machado… - Nature Reviews …, 2018 - nature.com
The adult and juvenile myositis syndromes, commonly referred to collectively as idiopathic
inflammatory myopathies (IIMs), are systemic autoimmune diseases with the hallmarks of …

[HTML][HTML] Long-term microdystrophin gene therapy is effective in a canine model of Duchenne muscular dystrophy

C Le Guiner, L Servais, M Montus, T Larcher… - Nature …, 2017 - nature.com
Duchenne muscular dystrophy (DMD) is an incurable X-linked muscle-wasting disease
caused by mutations in the dystrophin gene. Gene therapy using highly functional …

Supersonic shear imaging provides a reliable measurement of resting muscle shear elastic modulus

L Lacourpaille, F Hug, K Bouillard… - Physiological …, 2012 - iopscience.iop.org
The aim of the present study was to assess the reliability of shear elastic modulus
measurements performed using supersonic shear imaging (SSI) in nine resting muscles (ie …

First test of a “high-density injection” protocol for myogenic cell transplantation throughout large volumes of muscles in a Duchenne muscular dystrophy patient …

D Skuk, M Goulet, B Roy, V Piette, CH Côté… - Neuromuscular …, 2007 - Elsevier
A 26-years old Duchenne muscular dystrophy (DMD) patient received normal muscle-
precursor cells, proliferated in vitro and implanted in a thenar eminence, biceps brachii, and …

[HTML][HTML] Grip strength measured by high precision dynamometry in healthy subjects from 5 to 80 years

JY Hogrel - BMC musculoskeletal disorders, 2015 - Springer
Background Grip strength is a variable which may be important to measure and follow in
various populations. A new dynamometer with high accuracy and sensitivity has recently …

[HTML][HTML] Prospective and longitudinal natural history study of patients with type 2 and 3 spinal muscular atrophy: baseline data NatHis-SMA study

A Chabanon, AM Seferian, A Daron, Y Péréon… - PLoS …, 2018 - journals.plos.org
Spinal muscular atrophy (SMA) is a monogenic disorder caused by loss of function
mutations in the survival motor neuron 1 gene, which results in a broad range of disease …

[HTML][HTML] Quantitative muscle MRI as an assessment tool for monitoring disease progression in LGMD2I: a multicentre longitudinal study

TA Willis, KG Hollingsworth, A Coombs, ML Sveen… - PloS one, 2013 - journals.plos.org
Background Outcome measures for clinical trials in neuromuscular diseases are typically
based on physical assessments which are dependent on patient effort, combine the effort of …

[HTML][HTML] Forelimb treatment in a large cohort of dystrophic dogs supports delivery of a recombinant AAV for exon skipping in Duchenne patients

C Le Guiner, M Montus, L Servais, Y Cherel… - Molecular Therapy, 2014 - cell.com
Duchenne muscular dystrophy (DMD) is a severe muscle-wasting disorder caused by
mutations in the dystrophin gene, without curative treatment yet available. Our study …

Effect of ascorbic acid in patients with Charcot–Marie–Tooth disease type 1A: a multicentre, randomised, double-blind, placebo-controlled trial

J Micallef, S Attarian, O Dubourg, PM Gonnaud… - The Lancet …, 2009 - thelancet.com
Summary Background Charcot–Marie–Tooth disease type 1A (CMT1A) is a hereditary
peripheral neuropathy that affects roughly one in 5000 births. No specific therapy currently …