MND progression linked to metabolic overdrive

30 April 2018



Increased energy use in the cells of patients with motor neurone disease (MND) has been linked to faster disease progression and reduced lifespan.

A University of Queensland study revealed that patients who used more energy at rest - called hypermetabolism - were more than twice as likely to die within 12 months of assessment.

MND, also known as amyotrophic lateral sclerosis (ALS) or Lou Gehrig’s disease, is a rapidly progressing neurological disorder that attacks the nerve cells in the brain and spinal cord that control speech, movement, eating and breathing. The loss of these nerve cells (motor neurons) leads to progressive muscle weakness and wasting, and death. Currently, there is no cure.

Dr Shyuan Ngo, Scott Sullivan MND Research Fellow at QBI and AIBN, said the findings will help manage and potentially treat the fatal disease.

“It helps manage disease progression in the clinic, and opens up new avenues for improving patient outcomes through treatments that specifically target the cellular process of energy use.”

Metabolic overdrive could help explain MND progression

One of the hallmarks of MND is that cellular metabolism in muscle and nerve cells goes ‘haywire’, a phenomena that could impact on whole body energy use.

The research showed this could contribute to the progression of disease, potentially manifesting as fatigue, weakness and atrophy.

Co-investigator, Dr Frederik Steyn from UQ's Centre for Clinical Research, said the findings revealed important information about how the body responded to MND.

“Following diagnosis, patients have an average life expectancy of two to five years, but there is a great deal of variability from person to person,” Dr Steyn said.  

“It is extremely difficult for clinicians to identify which patients will progress rapidly and which won’t.

“We now know that how the body responds from a metabolic perspective can have a big impact on a person’s progression and survival.”

There is no cure for MND, in part, because the genetic and cellular mechanisms that lead to the disease are not yet fully understood.

The study is published in the Journal of Neurology, Neurosurgery, and Psychiatry.