OBJECTIVE: To characterize in vivo signatures of pathological diagnosis in a large cohort of @entity1 with @entity35869 ( @entity35869 ) variants defined by current diagnostic classification. METHODS: Extensive clinical, cognitive, neuroimaging, and neuropathological data were collected from 69 @entity1 with sporadic @entity35869 , divided into 29 semantic (svPPA), 25 non-fluent (nfvPPA), 11 logopenic (lvPPA), and 4 mixed @entity35869 . Patterns of grey matter (GM) and white matter (WM) @entity194 at presentation were assessed and tested as predictors of pathological diagnosis using support vector machine (SVM) algorithms. RESULTS: A clinical diagnosis of @entity35869 was associated with @entity747 ( @entity747 ) with @entity4943 inclusions in 40.5%, @entity747 -tau in 40.5%, and @entity195 ( @entity195 ) pathology in 19% of cases. Each variant was associated with one typical pathology: 24/29 (83%) svPPA showed @entity747 , 22/25 (88%) nfvPPA showed @entity747 -tau, and all 11 lvPPA had @entity195 . Within @entity747 -tau, 4R-tau pathology was commonly associated with nfvPPA, whereas @entity18309 was observed in a minority of subjects across all variants except for lvPPA. Compared with pathologically typical cases, svPPA-tau showed significant extrapyramidal signs, greater executive impairment, and severe @entity62 and WM @entity194 . nfvPPA- @entity4943 @entity1 lacked general motor symptoms or significant WM @entity194 . Combining GM and WM volumes, SVM analysis showed 92.7% accuracy to distinguish @entity747 -tau and @entity747 - @entity4943 pathologies across variants. INTERPRETATION: Each @entity35869 clinical variant is associated with a typical and most frequent cognitive, neuroimaging, and neuropathological profile. Specific clinical and early anatomical features may suggest rare and atypical pathological diagnosis in vivo. This article is protected by copyright. All rights reserved.

Typical and atypical pathology in XXXX variants.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity747 :: ('MESH:D057174', 'Disease') :: ['frontotemporal lobar degeneration', 'FTLD', 'FTLD-TDP type C'] @entity194 :: ('MESH:D001284', 'Disease') :: ['atrophy'] @entity35869 :: ('MESH:D018888', 'Disease') :: ['PPA', 'primary progressive aphasia'] @entity195 :: ('MESH:D000544', 'Disease') :: ['AD', "Alzheimer's disease"] @entity4943 :: ('MESH:D057180', 'Disease') :: ['TDP'] @entity62 :: ('MESH:D056784', 'Disease') :: ['striatal and frontal GM'] @entity18309 :: ('MESH:D020774', 'Disease') :: ["Pick's disease"]
@entity35869 :: (MESH:D018888,Disease) :: ['primary progressive aphasia']
