Motor, cognitive, and behavioral decline in C9orf72 carriers over 6 months

Takeaway

  • In symptomatic C9orf72 carriers, decline in motor function correlates with worsening behavioral impairment; behavioral worsening is driven by negative behaviors in those with amyotrophic lateral sclerosis (ALS) or mixed ALS-frontotemporal dementia (ALS-FTD) and driven by disinhibition for those with FTD alone.

Why this matters

    As disease progression impacts both motor networks and behavioral networks for C9orf72 carriers, the ALS Functional Rating Scale (ALSFRS-R) alone may not be appropriate to measure disease impairment in clinical trials.