MAPT and early-onset autosomal dominant Alzheimer disease: However, there is not complete concordance between phenotype and pathology: svPPA can more rarely be caused by tau or Alzheimer pathologies [22, 37•, 40]; nfvPPA is sometimes caused by Alzheimer’s disease, or TDP-43 (type A or B) [22, 37•, 41, 42]; lvPPA has been associated with dementia with Lewy bodies[43] and TDP-43 (type A) [41]; and an lvPPA-like phenotype has been consistently identified in people with mutations in the progranulin gene [44, 45].