Impaired oxygen extraction in metabolic myopathies: detection and quantification by near‐infrared spectroscopy

B Grassi, M Marzorati, F Lanfranconi… - Muscle & Nerve …, 2007 - Wiley Online Library
Patients with mitochondrial myopathies (MM) or myophosphorylase deficiency (McArdle's
disease, McA) show impaired capacity for O2 extraction, low maximal aerobic power, and …

Metabolic myopathies: functional evaluation by analysis of oxygen uptake kinetics.

B Grassi, S Porcelli, M Marzorati… - Medicine and science …, 2009 - europepmc.org
Purpose The aim was to identify additional noninvasive tools allowing to detect and to
quantify the metabolic impairment in patients with mitochondrial myopathies (MM) or …

Exercise testing as a diagnostic entity in mitochondrial myopathies

M Tarnopolsky - Mitochondrion, 2004 - Elsevier
Exercise intolerance is one of the most common symptoms in patients with mitochondrial
myopathies (MM). At the whole body level, this is characterized by a reduction in maximal …

Cycle ergometry is not a sensitive diagnostic test for mitochondrial myopathy

T Dysgaard Jeppesen, D Olsen, J Vissing - Journal of neurology, 2003 - Springer
Cycle exercise has repeatedly been used to diagnose patients suspected of having
mitochondrial myopathy (MM), in whom exercise intolerance and lactic acidosis are …

An oxidative defect in metabolic myopathies: diagnosis by noninvasive tissue oximetry

W Bank, B Chance - Annals of Neurology: Official Journal of …, 1994 - Wiley Online Library
Metabolic myopathies due to a variety of enzymatic deficiencies are well recognized. The
dynamics of oxygen delivery and utilization during exercise have not been observed …

Genetic disease of mitochondrial function evaluated by NMR and NIR spectroscopy of skeletal tissue

B Chance, W Bank - Biochimica et Biophysica Acta (BBA)-Molecular Basis …, 1995 - Elsevier
Bioenergetic sufficiency can be quantitatively assayed by nuclear magnetic resonance
spectroscopy (MRS) and on a relative basis by tissue optical spectroscopy (NIRS). Nuclear …

31P-MRS of skeletal muscle is not a sensitive diagnostic test for mitochondrial myopathy

TD Jeppesen, B Quistorff, F Wibrand, J Vissing - Journal of neurology, 2007 - Springer
Clinical phenotypes of persons with mitochondrial DNA (mtDNA) mutations vary
considerably. Therefore, diagnosing mitochondrial myopathy (MM) patients can be …

The spectrum of exercise tolerance in mitochondrial myopathies: a study of 40 patients

T Taivassalo, T Dysgaard Jensen, N Kennaway… - Brain, 2003 - academic.oup.com
Impaired skeletal muscle oxidative phosphorylation in patients with severe mitochondrial
respiratory chain defects results in disabling exercise intolerance that is associated with a …

Bioenergetics of skeletal muscle in mitochondrial myopathy

DJ Taylor, GJ Kemp, GK Radda - Journal of the neurological sciences, 1994 - Elsevier
Abstract 31 Phosphorus nuclear magnetic resonance spectroscopy was used to examine
skeletal muscle in 29 patients with mitochondrial myopathy, 9 male and 20 female …

Principles, insights, and potential pitfalls of the noninvasive determination of muscle oxidative capacity by near-infrared spectroscopy

A Adami, HB Rossiter - Journal of Applied Physiology, 2018 - journals.physiology.org
Skeletal muscle oxidative capacity is highly plastic, strongly associated with whole body
aerobic capacity (16, 18) and state of health. Loss of muscle oxidative capacity is associated …