From: Microglial burden, activation and dystrophy patterns in frontotemporal lobar degeneration
Pathological diagnosis | N | Genetic mutation (N) | Clinical diagnosis (N) | Sex (M/F) | Mean (range) age at symptom onset in years | Mean (range) age at death in years | Mean (range) disease duration in years | Mean (range) post-mortem delay (hours) |
---|---|---|---|---|---|---|---|---|
Controls | 5 | n/a | Control | 1/4 | n/a | 67.4 (38–80) | n/a | 45.7 (24.0–80.6) |
AD | 5 | n/a | Amnestic AD | 3/2 | 58.8 (49–65) | 69.2 (62.0–73.0) | 10.4 (5.0–17.0) | 45.8 (31.1–76.7) |
FTLD | 50 | Sporadic = 31 Genetic = 19: GRN = 5 C9orf72 = 8 MAPT = 5 TBK1 = 1 | bvFTD = 23 nfvPPA = 8 FTD-MND = 8 svPPA = 5 PSPS = 5 CBS = 1 | 29/21 | 57.8 (37–66) | 66.5 (51.3–84.0) | 8.7 (2.1–18.7) | 60.7 (10.8–157.6) |
FTLD subgroups | ||||||||
 FTLD-tau | 20 | Sporadic = 15 Genetic = 5 (MAPT) | bvFTD = 10 nfvPPA = 4 PSPS = 5 CBS = 1 | 13/7 | 58.2 (37–71) | 68.1 (52.4–84.0) | 9.9 (5.5–16.4) | 54.9 (24.0–103.3) |
 FTLD-CBD | 5 | All sporadic | nfvPPA = 3 PSPS =1 CBS = 1 | 2/3 | 60.2 (57–65) | 69.0 (64.8–73.8) | 8.8 (5.5–11.1) | 68.9 (37.2–103.3) |
 FTLD-MAPT | 5 | Genetic = 5 (all MAPT 10 + 16) | bvFTD = 5 | 2/3 | 48.0 (37–58) | 59.6 (52.4–68.4) | 11.6 (8.1–16.4) | 45.9 (24.0–64.0) |
 FTLD-Picks | 5 | All sporadic | bvFTD = 5 | 5/0 | 57.6 (52–64) | 68.1 (63.5–75.6) | 10.5 (5.5–15.6) | 47.8 (24.0–94.7) |
 FTLD-PSP | 5 | All sporadic | PSPS = 4 nfvPPA = 1 | 4/1 | 67.0 (62–71) | 75.6 (68.0–84.0) | 8.6 (6.0–13.0) | 57.2 (25.5–86.4) |
 FTLD-TDP | 26 | Sporadic = 12 Genetic = 14 C9orf72 = 8 GRN = 5 TBK1 = 1 | bvFTD = 9 nfvPPA = 4 FTD-MND = 8 svPPA = 5 | 12/14 | 59.4 (50–76) | 67.2 (53.1–78.6) | 7.8 (2.1–18.7) | 68.8 * (10.8–157.6) |
 FTLD-TDPA | 16 | Sporadic = 5 Genetic = 11 C9orf72 = 5 GRN = 5 TBK1 = 1 | bvFTD = 7 nfvPPA = 4 FTD-MND = 5 | 7/9 | 60.6 (50–76) | 67.0 (53.1–78.6) | 6.4 (2.1–10.3) | 77.3 (29.3–157.6) |
 FTLD-TDPB | 5 | Sporadic = 2 Genetic = 3 (C9orf72) | bvFTD = 2 FTD-MND = 3 | 2/3 | 56.2 (50–63) | 62.1 (56.2–67.2) | 5.9 (4.2–8.1) | 68.5 (10.8–96.0) |
 FTLD-TDPC | 5 | All sporadic | svPPA = 5 | 3/2 | 58.8 (52–64) | 73.0 (65.4–78.6) | 14.2 (10.3–18.7) | 43.8 (19.0–83.7) |
 FTLD-FUS (all aFTLD-U) | 4 | All sporadic | bvFTD = 4 | 4/0 | 45.4 (40–51) | 53.9 51.3–60.4 | 8.4 (5.5–11.3) | 39.0 (12.0–72.0) |
 FTLD cases used for genetic group analysis (all included in groups above) | 15 | GRN = 5 (all FTLD-TDPA) MAPT = 5 (all FTLD-MAPT) C9orf72 = 5 (all FTLD-TDPA) | bvFTD = 4 nfvPPA = 1 bvFTD = 5 FTD-MND = 3 nfvPPA = 2 | 2/3 2/3 1/4 | 58.0 (62–67) 48.0 (37–58) 60.8 (55–68) | 64.6 (55.3–74.2) 59.6 (52.4–68.4) 68.6 (62.7–75.1) | 6.6 (5.3–8.4) 11.6 (8.1–16.4) 7.8 (5.7–10.3) | 83.1 (29.3–157.6) 45.9 (24.0–64.0) 78.7 (51.9–107.1) |