Table 1.
Case ID | Neuropathological diagnosis | Neurological diagnosis (last ante mortem) | Age at death [years] |
PMI [hours] | Sex | Disease duration [years] | Brain weight [g] | Tau isoform# [IHC: 4R/3R] | AGD | Braak&Braak (NFT) | CERAD-plaque density | Thal-Phase | Aβ42 (4G8) | α-synuclein (Braak; LBs) | TDP-43 | FUS | |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
PSP1 | PSP | PSP-RS | 68 | 38 | Male | 6.0 | 1460 | ± | − | 1 | 0 | 0 | No | 0 | neg | neg | |
PSP2 | PSP | PSP-ns | 77 | 78 | Female | 2.5 | 1112 | ± | − | 0 | 0 | 0 | No | 0 | neg | neg | |
PSP3 | PSP | PSP-ns | 78 | 42 | Male | 9.0 | NA | ± | + | 1 | 0 | 1* | Yes* | 0 | neg | neg | |
PSP4 | PSP | bvFTD-tauopathy | 68 | 7 | Female | 7.0 | 1005 | ± | − | 0 | 0 | 0 | No | 0 | neg | neg | |
CBD1 | CBD | PSP-RS | 52 | 14 | Female | 4.5 | 1260 | ± | − | 0 | 0 | 0 | No | 0 | neg | neg | |
CBD2 | CBD | bvFTD | 56 | 44 | Male | 2.5 | NA | ± | + | 1 | 0 | 0 | No | 0 | neg | neg | |
CBD3 | CBD | bvFTD-CBS | 59 | 23 | Female | 7.0 | 1220 | ± | − | 1 | – | 1* | Yes* | 0 | pos | neg | |
CBD4 | CBD | bvFTD-MND | 59 | 54 | Male | 1.5 | 1330 | ± | + | 1 | 0 | 3* | Yes* | 0 | neg | neg | |
C1 | Ctrl | − | 58 | 22 | Male | − | NA | −/− | − | 1 | − | 0 | − | 0 | pos | − | |
C2 | Ctrl | − | 82 | 27 | Male | − | 1230 | −/− | − | 2 | − | 1* | − | 0 | neg | neg | |
C3 | Ctrl | − | 86 | 20 | Female | − | 1035 | −/− | − | 1 | − | 0 | − | 0 | − | − | |
C4 | Ctrl | − | 64 | 33 | Female | − | NA | −/− | − | 1 | − | 0 | − | 0 | neg | neg | |
C5 | Ctrl | − | 53 | 29 | Male | − | 1792 | −/− | − | 0 | − | 2* | − | 0 | neg | neg |
Aβ42 amyloid beta 42, AGD Argyrophilic Grain Disease, bvFTD behavioral variant Frontotemporal Dementia, CBS Corticobasal Syndrome, CERAD Consortium to Establish a Registry for Alzheimer's Disease, Ctrl control, IHC immunohistochemistry, LBs Lewy bodies, MND motor neuron disease, NA not assessable, neg negative, NFT neurofibrillary tangles, ns not specified, PMI postmortem interval, RS Richardson Syndrome. See also comprehensive data (Supplementary Data, T0, online resource)
#In study-relevant region. *No 4G8/Aβ42-positivity or Aβ-plaques in the study-relevant region