Table 1.

Clinical features of Patients P1–P22

The level of impairment in domains is denoted as follows: 0, preserved domain; +, mildly impaired domain; ++, severely impaired domain. #, Patient with GRN mutation.

AD = Alzheimer pathology; DLBD = diffuse Lewy body disease; G = agrammatic PPA; L = logopenic PPA by the 2011 classification; L* = logopenic PPA without repetition impairment and therefore unclassifiable by the 2011 guidelines; M = mixed PPA characterized by combined impairment of grammar and comprehension; NA = information not available; Ph = phonemic paraphasia; Se = semantic paraphasia; TDP-A = frontotemporal lobar degeneration with transactive response DNA-binding protein 43 precipitates of type A; ? = the patient was not able to produce sufficient verbal or written language to judge that domain.