Cerebral degeneration in amyotrophic lateral sclerosis

Takeaway

  • People with aggressive amyotrophic lateral sclerosis (ALS), including those with rapidly progressive disease and significant upper motor neuron (UMN) signs, are more likely to display deterioration of the motor cortex over time than people with less aggressive ALS, and healthy controls.

Why this matters

  • ALS is a clinically heterogeneous condition, with varying rates of deterioration. It is difficult to identify phenotypically homogenous cohorts for the purpose of clinical research.

  • N-acetylaspartate (NAA) is a non-invasive biomarker of neuronal integrity that can be imaged and quantified using magnetic resonance spectroscopy (MRS).

  • The ratio NAA to other metabolites in the motor and pre-frontal cortices may be reduced in people with ALS, providing a means to track neurodegeneration over time.