Table 3

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 cortexGliosis2 (2, 3)1 (0.25, 2)0.022
Neuronal loss2 (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 cortexGliosis1 (0.5, 2)2 (1, 2)0.335
Neuronal loss2 (1, 2.5)2 (1, 2.75)0.662
TDP-433 (2, 3)3 (2.75, 3)0.575
Ubiquilin*2 (1, 2)1 (0, 1.75)0.050
Angular cortexGliosis1 (0.5, 1.5)0.5 (0, 2)0.688
Neuronal loss2 (1, 2)1 (1, 2)0.771
TDP-432 (1, 3)2 (1.25, 3)0.909
Ubiquilin*2 (1, 2)0 (0, 1.5)0.002
Cingulate cortexGliosis0 (0, 1.5)1 (0.25, 1.75)0.298
Neuronal loss1 (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
ThalamusGliosis1 (0, 1)0 (0, 0.75)0.198
Neuronal loss1 (0, 1)0 (0, 0.75)0.109
TDP-432 (0, 2)1 (0.25, 2.0)0.842
Ubiquilin0 (0, 0.5)0 (0, 0)0.286
Granule layer, cerebellumGliosis0 (0, 0)0 (0, 0)1.000
Neuronal loss0 (0, 0)0 (0, 0)1.000
TDP-43*0 (0, 0)0 (0, 0)0.317
Ubiquilin3 (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.