Overall, we identified both disparate and shared transcriptomic signatures associated with cortical atrophy in bvFTD with pathogenic variants in C9orf72, GRN, and MAPT. The spatial distribution of gene expression may contribute to selective vulnerability of macroscopic neuroanatomy, giving rise to the heterogeneity observed in bvFTD. Here, C9orf72 is linked to behavioral variant of frontotemporal dementia.