New Imaging Can Measure Cell Dysfunction in ALS Patients

More effective therapies could potentially be introduced thanks to a new magnetic resonance spectroscopy technique that can accurately measure how well the mitochondria are functioning in patients with Amyotrophic Lateral Sclerosis (ALS.) This non-invasive procedure may help measure the efficacy of treatments for ALS (also known as Motor Neuron Disease [MND]) patients.

Scientists from Sheffield Institute for Translational Neuroscience (SITraN) from the University of Sheffield in the United Kingdom tested how the 31-phosphorus magnetic resonance spectroscopy performed, publishing their findings in the January 13 issue of Brain.

Lead study author Dr. Matilde Sassani, a neurodegeneration researcher at Sheffield, said, “In this study, we found that phosphocreatine levels were depleted in the brain compared to healthy controls and, in this muscle, we found that inorganic phosphates were elevated in patients with MND. Both of these findings are consistent with mitochondrial dysfunction occurring in these people living with MND.”

People with ALS suffer from impaired mitochondrial function, so having a technique that can effectively and accurately measure mitochondrial activity could be critical to treatment.

In the study, the imaging technique was used to measure a chemical involved in the cell’s energy metabolism. ALS patients were compared to healthy age and gender-matched controls. The procedure resembles an MRI; the process allows investigators to capture a direct measurement of chemicals. This snapshot gives them the appropriate data needed to calculate a comprehensive view of a patient with MND.

Senior author Dr. Thomas Jenkins, a clinical senior lecturer at SITraN, said the newfound technique could pave the way for more effective MND treatments and potentially measure how effective medications are.

Jenkins said, “Treatments that aim to rescue mitochondrial function in MND are being investigated in labs around the world. This non-invasive tool can demonstrate whether medications in development are successfully targeting mitochondria, which is an important step in selecting treatments to take through clinical trials.”

Patients with other forms of neurodegenerative diseases might also benefit from this technique, though further research is needed.

To read additional information, refer to the article in the latest issue of Brain, a Journal of Neurology.

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