Pathological analysis of neurodegeneration in frontotemporal lobar degeneration (FTLD) with and without the C9orf72 repeat expansion
C9orf72 expansion positive FTLD (N=13) | C9orf72 expansion negative FTLD (N=12) | p Value | ||
---|---|---|---|---|
Mid-frontal cortex | Gliosis | 2 (2, 3) | 1 (0.25, 2) | 0.022 |
Neuronal loss | 2 (2, 3) | 2 (1, 2) | 0.005 | |
TDP-43* | 3 (2, 3) | 2 (1.25, 3) | 0.284 | |
Ubiquilin† | 2 (1, 2) | 0 (0, 1) | 0.001 | |
Superior to mid-temporal cortex | Gliosis | 1 (0.5, 2) | 2 (1, 2) | 0.335 |
Neuronal loss | 2 (1, 2.5) | 2 (1, 2.75) | 0.662 | |
TDP-43 | 3 (2, 3) | 3 (2.75, 3) | 0.575 | |
Ubiquilin* | 2 (1, 2) | 1 (0, 1.75) | 0.050 | |
Angular cortex | Gliosis | 1 (0.5, 1.5) | 0.5 (0, 2) | 0.688 |
Neuronal loss | 2 (1, 2) | 1 (1, 2) | 0.771 | |
TDP-43 | 2 (1, 3) | 2 (1.25, 3) | 0.909 | |
Ubiquilin* | 2 (1, 2) | 0 (0, 1.5) | 0.002 | |
Cingulate cortex | Gliosis | 0 (0, 1.5) | 1 (0.25, 1.75) | 0.298 |
Neuronal loss | 1 (1, 2) | 1 (1, 2) | 0.953 | |
TDP-43* | 2.5 (1.25, 3) | 3 (2, 3) | 0.328 | |
Ubiquilin* | 1.5 (1, 2) | 0 (0, 1) | 0.002 | |
Thalamus | Gliosis | 1 (0, 1) | 0 (0, 0.75) | 0.198 |
Neuronal loss | 1 (0, 1) | 0 (0, 0.75) | 0.109 | |
TDP-43 | 2 (0, 2) | 1 (0.25, 2.0) | 0.842 | |
Ubiquilin | 0 (0, 0.5) | 0 (0, 0) | 0.286 | |
Granule layer, cerebellum | Gliosis | 0 (0, 0) | 0 (0, 0) | 1.000 |
Neuronal loss | 0 (0, 0) | 0 (0, 0) | 1.000 | |
TDP-43* | 0 (0, 0) | 0 (0, 0) | 0.317 | |
Ubiquilin | 3 (1.5, 3) | 0 (0, 0) | 0.001 |
*Data missing for one case.
†Data missing for three cases.
TDP-43, TAR DNA-binding protein 43.