Table 1

Demographics table for symptomatic and at-risk family members

	Symptomatic patients					At-risk family members
	Total	C9orf72	GRN	MAPT	Contrasts	Preclinical*	Non-carrier*	Contrasts*	Preclinical†	Non-carrier†	Contrasts†
N	185	87	65	33		317	320		294	294
Handedness					P=0.02‡			P=0.16‡			P=0.14‡
Right	174	80	65	29		282	298		275	262
Left	9	5	0	4		31	20		17	28
Ambidextrous	2	2	0	0		4	2		2	4
Sex					X²=6.2, p=0.045			X²=0.90, p=0.34			X²=0.86, p=0.35
Male	108	57	30	21		123	136		112	123
Female	77	30	35	12		194	184		182	171
Genotype								X²=0.21, p=0.90			X²=0.58, p=0.75
C9orf72						117	115		104	103
GRN						144	144		138	132
MAPT						56	61		52	59
Maximum number of visits
1						121	118		124	122
2						80	98		80	95
3						72	58		60	38
4						30	27		22	23
5						10	15		7	16
6						4	4		1	0
Diagnosis
bvFTD		62	33	31
PPA		4	28	0
FTD-ALS		9	0	0
ALS		6	0	0
PSP		1	0	0
CBS		0	2	1
AD		0	1	0
Dementia-NOS		3	1	1
Other		2	0	0
Time interval for change score (SD)						2.6 (1.4)(n=196)	2.5 (1.5)(n=202)	t(394.7)=−0.6, p=0.54	2.5 (1.3)(n=170)	2.4 (1.5)(n=172)	t(340)=−0.7, p=0.49
Age (SD)	62.3 (8.5)	63.7 (8.3)	63.5 (6.9)	56.2 (9.5)	F(2,184)=11.5, p<0.001§ C9orf72>MAPT GRN>MAPT	44.0 (11.8)	46.3 (14.0)	t(619)=2.3, p=0.03	44.0 (11.9)	46.7 (14.1)	t(570.1)=2.6, p=0.01
Age at onset (SD)	58.1 (8.8)	58.8 (9.0)	60.6 (7.2)	51.1 (7.7)	F(2,184)=11.5, p<0.001§ C9orf72>MAPT GRN>MAPT
Education, years (SD)	12.2 (4.0)	12.6 (4.0)	11.2 (4.0)	13.2 (3.6)	F(2,184)=3.5, p=0.03§ MAPT>GRN (p=0.065)	14.3 (3.3)	13.9 (3.6)	t(635)=−1.5, p=0.13	14.3 (3.3)	13.9 (3.6)	t(586)=−1.58, p=0.1
Years from expected symptom onset (SD)¶						−14.4 (11.8)	−13.2 (14.1)	t(618.5)=1.17, p=0.24	−14.5 (12.0)	−12.9 (14.2)	t(569.3)=1.51, p=0.13

Fisher’s exact/χ² tests (if expected cell count was <5), independent sample t-tests or one-way analysis of variance were used to discern group differences for relevant variables.
*At-risk participants from 248 families. Participants completed the GENFI symptom list.
†At-risk participants from 228 families. Participants completed the CBI-R Questionnaire.
‡Fisher’s exact test was used.
§Bonferroni correction applied.
¶Years from expected onset was calculated by subtracting the participant’s age at the time of participation from the mean age of symptom onset within the family.
AD, Alzheimer's Disease;; ALS, amyotrophic lateral sclerosis; bvFTD, behavioural variant frontotemporal dementia; CBI-R, Cambridge Behavioural Inventory Questionnaire-Revised; CBS, corticobasal syndrome; C9orf72, chromosome 9 open reading frame 72; GENFI, Genetic Frontotemporal Dementia Initiative; GRN, progranulin; MAPT, microtubule-associated protein tau; NOS, not otherwise specified; PPA, primary progressive aphasia; PSP, progressive supranuclear palsy.