Evidence of cerebellar TDP-43 loss of function in FTLD-TDP

Table 1 Characteristics of controls and FTLD-TDP cases

Variable	Controls N = 25	FTLD-TDP N = 95	P value
Sex			0.6644^a
Male	13 (52.0%)	54 (56.8%)
Female	12 (48.0%)	41 (43.2%)
Age at disease onset (years)	NA	63.6 (44.0, 83.0)
Disease duration (years)	NA	8.0 (2.0, 25)
Age at death (years)	81.9 (56.6, 99.0)	73.1 (52.4, 90.4)	0.0058^b
Genotype
No mutation	NA	33 (34.7%)
C9orf72 repeat expansion	NA	29 (30.6%)
GRN mutation	NA	33 (34.7%)
TDP-43 subtype
A	NA	68 (71.6%)
B	NA	8 (8.4%)
C	NA	15 (15.7%)
RIN	9.7 (7.1, 10)	9.4 (7.2, 10.0)	0.0375^b

The sample median (minimum, maximum) is given for continuous variables. Information was unavailable regarding age at disease onset and disease duration for 10 FTLD-TDP cases; age at death for 1 FTLD-TDP case; TDP-43 subtype for 4 FTLD-TDP cases. A Chi-squared test was used to test for differences in sex^a, and a Mann–Whitney test was used to test for differences in age at death and RIN^b between control and FTLD-TDP groups. P-values < 0.05 are considered statistically significant and are marked in bold

ISSN: 2051-5960