Increased metabolic use in patients with amyotrophic lateral sclerosis (ALS) has been linked to faster disease progression and reduced lifespan, University of Queensland researchers report.
The finding, according to the UQ Centre for Clinical Research’s Dr. Frederik Steyn, reveals important information about how the body responds to ALS, and could hold clues to new approaches to treatment.
“Patients who used more energy at rest were more than twice as likely to die within 12 months of assessment. 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. 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."
Dr. Steyn said.
Hypermetabolism, an increased use of energy, refers to a significant increase in measured resting energy expenditure compared to to predicted resting energy expenditure. Previous research of hypermetabolism in ALS shows it is prevalent in from 25% to 68% of sporadic ALS patients.
In familial ALS, hypermetabolism is reported to occur in all patients.
[caption id=“attachment_95387” align=“aligncenter” width=“680”] Survival probability of hypermetabolic versus normometabolic patients with amyotrophic lateral sclerosis (ALS) relative to time of metabolic assessment.
Credit: Frederik J Steyn et al CC-BY[/caption]
ALS, also known as motor neurone disease (MND), is a fatal neurological degenerative disease that progresses rapidly.
“There is no cure, in part because the genetic and cellular mechanisms that lead to the disease are not yet fully understood. 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,"
Dr. Steyn said.
The research showed this could contribute to the progression of disease, potentially manifesting as fatigue, weakness and atrophy. Funding support was provided by Wesley Medical Research.
Steyn FJ, Ioannides ZA, van Eijk RPA, et al Hypermetabolism in ALS is associated with greater functional decline and shorter survival J Neurol Neurosurg Psychiatry Published Online First: 29 April 2018. doi: 10.1136/jnnp-2017-317887
Top Image: University of Queensland