T. Bradley Willingham and Kevin K. McCully, Front. Physiol., 14 September 2017 doi:10.3389/fphys.2017.00689
The ability to sustain submaximal exercise is largely dependent on the oxidative capacity of mitochondria within skeletal muscle, and impairments in oxidative metabolism have been implicated in many neurologic and cardiovascular pathologies. Here we review studies which have demonstrated the utility of Near-infrared spectroscopy (NIRS) as a method of evaluating of skeletal muscle mitochondrial dysfunction in clinical human populations.
In FRDA, NIRS measures of mitochondrial capacity in the forearm were inversely correlated with feelings of low energy, suggesting that mitochondrial function may be related to fatigue in persons with FRDA across the spectrum of symptom severity (Bossie et al., 2016). These findings lend support to the use of NIRS measures of mitochondrial capacity as measure of muscle dysfunction in persons with FRDA during interventions or over the course of disease progression.
There are currently no physiological evaluation tools available for clinicians diagnosing and treating patients with mitochondrial disease, and the application of NIRS may offer a relevant, in vivo measure of mitochondrial function in this population. To date, Friedreich's ataxia is the only mitochondrial disease to be evaluated using NIRS, and establishing the usefulness of NIRS in evaluating mitochondrial dysfunction in persons with mitochondrial disease warrants further investigation.
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