The relationship between primary progressive aphasia and amyotrophic lateral sclerosis


  • Amyotrophic lateral sclerosis (ALS) was identified in 12% of individuals with primary progressive aphasia (PPA), and frontotemporal lobar degeneration (FTLD) was identified in all PPA-ALS cases.

Why this matters

  • It is important to assess for ALS in those with PPA, and currently identified gene mutations associated with neurodegenerative disease do not fully explain onset of familial PPA-ALS.