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Table 1 Demographic, clinicopathological and genetic profile of cases

From: Distinct TDP-43 inclusion morphologies in frontotemporal lobar degeneration with and without amyotrophic lateral sclerosis

 

FTLD

FTLD-ALS

ALS

N (% male)

23 (52%)

23 (57%)

15 (53%)

Age at death (year)

65 ± 8a

67 ± 8a

71 ± 8

Age at onset (year)

59 ± 8a

63 ± 8a

70 ± 7

Disease duration (year)

6 ± 4a

4 ± 3

2 ± 2

Postmortem delay (hours)

25 ± 23

25 ± 17

22 ± 10

bvFTD/SD/PNFA/FTD unspecified/AD (n)

21/0/0/1/1

17/2/2/1/1

N/A

C9ORF72 carrier % (n)

48% (11)a

39% (9)a

0% (0)

GRN carrier % (n)

35% (8)a

0% (0)b

0% (0)b

  1. DD Disease duration (years), DO Disease onset (years), PMD postmortem delay (hours), bvFTD behavioral variant frontotemporal dementia, SD semantic dementia, PNFA progressive non-fluent aphasia, AD Alzheimer’s disease, N/A not applicable.a p < 0.05 compared to ALS, b p < 0.05 compared to FTLD