Abstract

We reported amyotrophic lateral sclerosis (ALS) in 12% of a large cohort of patients with nonfluent variant primary progressive aphasia (nfvPPA) or semantic variant of primary progressive aphasia (svPPA) and demonstrated frontotemporal lobar and motor degeneration accompanied by TDP-43 inclusion pathology in all nfvPPA-ALS and svPPA-ALS cases that came to autopsy.1

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