Table 3.
Comparison of mean age at death and percentage hippocampal sclerosis positive, frontotemporal dementia positive, progressive non-fluent aphasia positive, and semantic dementia positive, between the current case series (bold) and prior case series with frontotemporal lobar dementia with aberrant TDP-43 (FTLD-TDP)
n | Mean death age in years (SD) | Hippocampal sclerosis pathology (%) | FTD or PNFA clinically (%) | Semantic dementia clinically (%) | |
---|---|---|---|---|---|
Current study | |||||
HS-Ageing (all) | 106 | 92 (7) | 100b | 0 | 0 |
HS-Ageing–TDPa | 71 | 94 (7) | 100b | 0 | 0 |
Rohrer et al. (2010) | |||||
FTLD–TDP type 1c | 9 | 59 (8) | 0 | 0 | 100 |
FTLD–TDP type 2c | 5 | 59 (11) | 20 | 100 | 0 |
FTLD–TDP type 3c | 10 | 57 (8) | 10 | 80 | 0 |
Josephs et al. (2009) | |||||
FTLD–TDP type 1d | 24 | 76 (10) | 75 | 100 | 0 |
FTLD–TDP type 2d | 9 | 74 (10) | 56 | 29 | 71 |
FTLD–TDP type 3d | 6 | 70 (8) | 67 | 100 | 0 |
Mackenzie et al. (2006)e | |||||
FTLD–TDP type 1d | 15 | 69 (5) | 93 | 93 | 7 |
FTLD–TDP type 2d | 9 | 70 (4) | 67 | 22 | 77 |
FTLD–TDP type 3d | 13 | 59 (11) | 100 | 100 | 0 |
Armstrong et al. (2009)f | |||||
FTLD–TDP sporadic | 52 | 71 (11) | 6 | ||
FTLD–TDP not sporadic | 42 | 70 (9) | 7 |
a 71/79 cases with hippocampal sclerosis evaluated for TDP-43 immunohistochemistry were positive.
b 100% is due entirely to inclusion criteria.
c According to the Sampathu scheme for FTLD-TDP typing (Sampathu et al, 2006).
d According to the Mackenzie scheme for FTLD-TDP typing (Mackenzie et al, 2006).
e Displayed also are data from the same patients in Davidson et al. (2007).
f Patients in this study all met clinical criteria for frontotemporal dementia syndrome.
FTD = frontotemporal dementia including behavioural and motor neurone disease variants; PNFA = progressive non-fluent aphasia.