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. Author manuscript; available in PMC: 2019 Sep 1.
Published in final edited form as: Acta Neuropathol. 2018 Jun 20;136(3):389–404. doi: 10.1007/s00401-018-1878-z

Table 3: Clinicopathologic characteristics compared by clinical diagnoses.

CBS
N = 69
PSP
N = 77
AD
N = 13
FTD
N = 12
Others
N = 15
P value
Male, No. (%) 31 (45%) 39 (51%) 3 (23%) 7 (58%) 9 (60%) 0.285
Age at death, years 71 ± 7 68 ± 8 75 ± 10* 67 ± 7 72 ± 8 0.010
Disease duration, years 7 ± 2 6 ± 3 7 ± 3 7 ± 2 5 ± 3 0.081
Diagnostic physician type 6.8×10−8
 Movement disorder specialist 40/65 (62%) 35/71 (49%) 0/13 (0%) 1/12 (8%) 6/13 (46%)
 Dementia specialist 6/65 (9%) 4/71 (6%) 2/13 (15%) 5/12 (42%) 3/13 (23%)
 General neurologist 18/65 (28%) 31/71 (44%) 8/13 (62%) 6/12 (50%) 1/13 (8%)
 Non-neurologist 1/65 (2%) 1/71 (1%) 3/13 (23%) 0/12 (0%) 3/13 (23%)
Asymmetric rigidity/apraxia 57/61 (93%) 23/41 (56%) 0/4 (0%) 3/9 (33%) 3/7 (43%) 8.1×10−9
Downward gaze palsy 17/61 (28%) 50/67 (83%) 1/9 (11%) 1/11 (9%) 3/12 (25%) 1.5×10−9
Initial presentations 5.6×10−8
 Motor symptoms 44/58 (76%) 28/61 (46%) 1/6 (17%) 0/11 (0%) 4/12 (33%)
 Cognitive/behavioral
symptoms
5/58 (9%) 17/61 (28%) 5/6 (83%) 10/11 (91%) 6/12 (50%)
 Mixed 9/58 (16%) 16/61 (26%) 0/6 (0%) 1/11 (9%) 2/12 (17%)
Pathologic features
Brain weight, g 1100 ± 150 1140 ± 130 1000 ± 160* 1020 ± 90* 1170 ± 130 5.8×10−4
Thickness of corpus callosum,
mm
3.5 ± 1.0 3.6 ± 0.9 2.0 ± 0.7** 2.1 ± 0.5** 3.3 ± 1.2 3.2×10−7
TDP-43 pathology 1.5×10−5
 TDP-limited 17/69 (25%) 14/77 (18%) 4/13 (31%) 5/12 (42%) 4/15 (27%)
 TDP-severe 4/69 (6%) 32/77 (42%) 3/13 (23%) 1/12 (8%) 0/15 (0%)

Data are displayed as n (%) and mean ± SD. “Others” are atypical Parkinson’s disease, Parkinson’s disease with dementia or dementia with Lewy bodies (N = 8), primary progressive aphasia (N = 5), and multiple system atrophy (N = 2).

*

indicates P value <0.05 compared to PSP;

**

indicates P value <0.05 compared to PSP and CBS.