TABLE 1.
Autopsy cohort
| LATE-NC w/o AD | AD w/o LATE-NC | AD + LATE-NC | |
|---|---|---|---|
| N | 7 | 23 | 20 |
| Age at death (years) | 87.6 ± 5.6 | 80.4 ± 8.2 | 82.5 ± 8.1 |
| Sex (M/F) | 5/2 | 15/8 | 14/6 |
| aMCI/ADD | 3/4 | 1/22 | 1/19 |
| Interval diagnosis to death | 1.6 ± 1.6 | 1.7 ± 1.4 | 2.6 ± 2.2 |
| Interval FDG-PET to death | 3.6 ± 3.5 | 3.1 ± 2.3 | 3.8 ± 2.4 |
| A, 0/1/2/3 | 0/3/4/0 | 0/0/1/22 | 0/0/0/20 |
| B, 0/1/2/3 | 0/6/1/0 | 0/0/1/22 | 0/0/1/19 |
| C, 0/1/2/3 | 4/3/0/0 | 1/0/2/20 | 0/1/1/18 |
| TDP-43 stage, 1/2/3 | 0/5/2 | N/A | 2/13/5 |
| Lewy body pathology, 0/1/2/3/4, %pos | 3/1/0/3/0, 57% | 12/1/0/5/5, 48% | 7/0/1/7/5, 65% |
| FTD-related TDP-43 (FTDTDP) | 0/7, 0% | 0/23, 0% | 1/20, 5% |
| Argyrophilic grain disease (FTDTAU-5) | 5/7, 71% | 0/23, 0% | 4/20, 20% |
| Other 4R tauopathy (FTDTAU-6) | 3/7, 43% | 5/23, 22% | 9/20, 45% |
| Tangle dominant disease (FTDTAU-9) | 1/7, 14% | 0/23, 0% | 0/20, 0% |
| Other 3R/4R tauopathy (FTDTAU-10) | 1/7, 14% | 1/23, 4% | 1/20, 5% |
Abbreviations: aMCI, amnestic mild cognitive impairment; ADD, Alzheimer’s disease dementia; F, female; FTD, frontotemporal degeneration; LATE-NC, limbic age-related TDP-43 encephalopathy neuropathologic change; M, male; N, sample size.
Notes: Average values are reported as mean ± standard deviation. A, B, C correspond to AD neuropathological change scores reflecting Thal amyloid phase (A), Braak neurofibrillary tangle stage (B), and Consortium to Establish a Registry for Alzheimer’s Disease (CERAD) density of neuritic plaques (C). A/B/C scales are scored on a common semiquantitative 4-point scale from absent (0) to low (1), intermediate (2), and high (3).25 TDP-43 staging followed an adapted version of the simplified staging system proposed by Nelson et al.:3 1 – TDP-43 in amygdala only; 2 – TDP-43 in hippocampus or entorhinal/inferior temporal cortex, but not frontal neocortex; 3 – TDP-43 in frontal neocortex and any of the other regions (see supporting information for more detailed information on TDP-43 assessment and individual staging). Scoring of Lewy body pathology follows consensus criteria from McKeith et al.:47 1 – brainstem predominant; 2 – limbic transitional, 3 – diffuse neocortical, 4 – amygdala predominant. FTDTDP and FTDTAU-X codes in parentheses refer to the respective pathologic designations in the Neuropathology Data Form of the National Alzheimer Coordinating Center (v10; https://www.alz.washington.edu/NONMEMBER/NP/npform10.pdf).