Structural brain changes and cognitive impairment in amyotrophic lateral sclerosis

Takeaway

  • Patients with cognitive and behavioral symptoms of amyotrophic lateral sclerosis (ALS) display widespread cerebral changes (specifically in the frontotemporal lobes) and progressive white matter involvement over time.

Why this matters

  • Up to 15% of patients with ALS develop frontotemporal dementia while another 35% have some degree of cognitive or behavioral impairment.

  • A repeat expansion in C9orf72 is the most common mutation in ALS and is associated widespread white and gray matter involvement.

  • Multimodal imaging studies could provide an anatomical characterization of neurodegeneration in ALS.