The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) in frontotemporal dementia

Figures & data

Table 1 Demographics Characteristics.

	Patients		Controls		p-Value
	ALS-FTD	bvFTD
Number	20	23	30
Gender, M:F	12:8	14:9	9:21	χ^2 = 6.58	0.04
Age, mean (SD)	65 (8)	60 (7)	59 (8)	F = 3.89	0.03^
Years of education, mean (SD)	13 (3)	12 (3)	14 (3)	F = 3.86	0.03*
Duration, mean years (SD)	3 (1)	5 (4)	n/a	t = 2.6	0.02
Dementia severity^1	9.5 (2.8)	9.3 (2.8)	n/a	t = 0.26	0.80

^Differences lie between ALS-FTD and control (post-hoc Bonferroni test p = 0.03). *Differences lie between bvFTD and control (post-hoc Bonferroni test p = 0.02). Other comparisons non-significant.

¹ Modified Clinical Dementia Rating, mean number of boxes (14).

Table 2 Group comparisons of ECAS domains.

	Patients		Controls	Statistic	Group analysis
	ALS-FTD	bvFTD
	Median (range)	Median (range)	Median (range)	Kruskall–Wallis	p-Value
Language/28	21 (5–28)	20 (1–28)	28 (25–28)	35.3	<0.001^a,b
Verbal fluency/24	3 (0–14)	8 (0–18)	20 (6–24)	42.8	<0.001^a,b
Executive/48	19.5 (4–43)	23.5 (4–42)	40.5 (30–45)	32.9	<0.001^a,b
Memory/24	7 (0–16)	10 (0–21)	18 (12–22)	34.5	<0.001^a,b
Visuospatial/12	11 (3–12)	11 (0–12)	12 (11–12)	21.2	<0.001^a,b
ALS total/100	45 (12–81)	55.5 (14–87)	89 (72–95)	40.0	<0.001^a,b
Non-ALS total/36	19 (6–27)	16 (0–32)	30 (24–34)	36.7	<0.001^a,b
ECAS total/136	65 (18–98)	78 (15–119)	118 (100–127)	40.3	<0.001^a,b

Post-hoc Mann–Whitney test significant at p < 0.001 between ^aALS-FTD and controls and ^bbvFTD and controls. No significant differences between ALS-FTD and bvFTD were observed.

Table 3 ECAS subtests: FTD patients (ALS-FTD + bvFTD) compared to controls.

Test	FTD		Controls		Mann–Whitney		p-Value	Effect size
	Median	Range	Median	Range	U	z
ALS-specific
Naming/8	6	1–8	8	7–8	177.0	−5.5	<0.001	−0.65
Comprehension/8	8	0–8	8	8–8	375.0	−4.0	<0.001	−0.47
Spelling/12	8	0–12	12	10–12	199.5	−5.0	<0.001	−0.60
Fluency letter S/12	1	0–10	10	4–12	60.0	−6.4	<0.001	−0.78
Fluency letter T/12	0	0–10	10	4–12	73.0	−6.3	<0.001	−0.77
Reversed digits/12	4	0–8	8	4–10	151.5	−5.5	<0.001	−0.65
Alternation/12	6	0–12	12	5–12	222.0	−4.7	<0.001	−0.56
Sentence completion/12	4.5	0–11	11	4–12	127.0	−5.7	<0.001	−0.68
Social cognition/12	6	0–12	12	6–12	277.5	−3.9	<0.001	−0.47
ALS nonspecific
Immediate recall/10	2	0–7	7	4–10	105.0	−6.1	<0.001	−0.71
Retention over delay/10	3	0–10	9	4–10	316.0	−3.6	<0.001	−0.42
Delayed recognition/4	1	0–4	2	0–4	270.5	−3.9	<0.001	−0.47
Dot counting/4	4	0–4	4	3–4	437.5	−2.9	0.004	−0.34
Cube counting/4	3.5	0–4	4	3–4	315.0	−4.2	<0.001	−0.50
Number location/4	4	0–4	4	3–4	450.0	−2.7	0.007	−0.32

Table 4 Percentage of patients showing impairment.

Criterion	Below 5th percentile of control scores		Using 2014 cutoffs (1)		Using age and education adjusted cutoffs (9)
Domain	ALS-FTD	bvFTD	ALS-FTD	bvFTD	ALS-FTD	bvFTD
Language	80	74	85	78	65	61
Verbal fluency	95	70	100	70	95	78
Executive	90	74	90	74	80	70
Memory	75	61	80	78	60	61
Visuospatial	40	44	40	44	35	43
ALS-specific	90	78	95	87	85	70
ALS nonspecific	80	65	80	74	60	57
Total/136	100	83	100	91	85	74

Table 5 Inter-correlations between domains in ALS-FTD and bvFTD (Spearman’s rho).

	Verbal fluency	Executive	Memory	Visuo-spatial
ALS-FTD	–	–	–	–
Language	0.51	0.35	0.13	0.25
Verbal Fluency	–	0.75**	0.19	0.48
Executive	–	–	−0.06	0.39
Memory	–	–	–	−0.11
bvFTD	–	–	–	–
Language	0.74 ***	0.77***	0.78***	0.66***
Fluency	–	0.71***	0.70***	0.40
Executive	–	–	0.62**	0.67***
Memory	–	–	–	0.43*

***p < 0.001; **p < 0.01; *p < 0.05.

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Data availability

The data that support the findings of this study are available from the corresponding author upon reasonable request.