Table 1.

Demographics and clinical features of C9+ and control groups at baseline visit

	Healthy Controls	C9+ diagnosis subgroups
		Asymptomatic	ALS	ALS-FTD	bvFTD
Number at baseline	28	7	11	7	3
Age (years)	52.8 ± 9.1	42.8 ± 10.1*	52.4 ± 8.8	61.9 ± 9.8†	60.6 ± 5.1†
Male: Female	18:10	2:5	5:6	7:0	3:0
Education (yrs.)	16.3 ± 2.8	15.4 ± 2.8	14.8 ± 2.4	17.7 ± 1.4	14.7 ± 2.3
Disease Duration (months)	-	-	30.7 ± 23.9	37.5 ± 28.6	29.4 ± 26.5
MMSE	29.0 ± 1.2	29.0 ± 1.0	28.1 ± 2.1	25.4 ± 4.1*†	25.0 ± 4.4*†
ALSFRS-R	-	48 ± 0	36.0 ± 8.7†	41.1 ± 5.1	46.0 ± 2.6
FBI Score (%)	-	3.2 ± 3.3	6.7 ± 6.5	28.4 ± 11.0†	36.1 ± 24.2†
Letter Fluency Score	-	34.4 ± 16.6	32.6 ± 13.1	27.9 ± 16.8†	17.7 ± 5.5†
Follow-up scan interval (mos. from baseline)	3.0 ± 3.0 (n=23)	5.3 ± 0.5 (n=5)	5.6 ± 0.8 (n=6)	6.3 ± 0.5 (n=5)	6.2 ± 1.0 (n=3)
2nd follow-up scan interval (mos. from baseline)		16.3 ± 3.8 (n=4)	17.7 ± 0.4 (n=4)	18.9 ± 0.5 (n=2)	18.4 ± 2.1 (n=2)

^*differs from healthy controls (p < 0.05)

^†differs from C9+ asymptomatic subgroup (p < 0.05)

MMSE – mini-mental state exam; ALSFRS-R – revised ALS Functional Rating Scale

FBI –Frontal Behavioural Inventory, scored as % of possible