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.