Table 3

Cognitive scoring of ALS cohort

Behavioural impairment		Cognitive assessment
ID	Overall ALSci (Y/N)	ALS Specific	ALS non-specific	ECAS total	Executive	Language	Fluency	Memory	Visuospatial	Behaviour
Case 1	N	92	33	125	42	28	22	22	11	4
Case 2 (NEK1)	N	86	30	116	38	28	20	18	12	2
Case 3 (SOD1)	N	87	31	118	41	28	18	19	12	2
Executive dysfunction		Cognitive assessment
ID	Overall ALSci (Y/N)	ALS specific	ALS non-specifc	ECAS total	Executive	Language	Fluency	Memory	Visuospatial	Behaviour
Case 4 (C9orf72)	Y	76	26	102	31	27	18	14	12	ND
Language dysfunction		Cognitive assessment
ID	Overall ALSci (Y/N)	ALS specific	ALS non-specifc	ECAS total	Executive	Language	Fluency	Memory	Visuospatial	Behaviour
Case 5 (C9orf72)	N	85	27	112	39	26	20	15	12	0
Case 6 (C9orf72)	Y	76	26	102	35	23	18	14	12	ND
Case 7	Y	81	20	101	37	26	18	8	12	ND
Case 8 (C9orf72)	Y	74	26	100	36	20	18	14	12	ND
Case 9	N	88	26	114	44	26	18	14	12	ND
Case 10	N	85	26	111	36	25	24	14	12	0
Fluency dysfunction		Cognitive assessment
ID	Overall ALSci (Y/N)	ALS specific	ALS non-specifc	ECAS total	Executive	Language	Fluency	Memory	Visuospatial	Behaviour
Case 11	Y	75	30	105	39	28	8	18	12	ND
Case 12	N	82	33	115	44	28	10	21	12	ND
Combined deficit: executive, language and fluency		Cognitive assessment
ID	Overall ALSci (Y/N)	ALS specific	ALS non-specifc	ECAS total	Executive	Language	Fluency	Memory	Visuospatial	Behaviour
Case 13	Y	65	26	91	32	25	8	15	11	0
Case 14	Y	62	28	90	32	22	8	16	12	ND
Unimpaired		Cognitive assessment
ID	Overall ALSci (Y/N)	ALS specific	ALS non-specifc	ECAS total	Executive	Language	Fluency	Memory	Visuospatial	Behaviour
Case 15 (C9orf72)	N	83	31	114	39	28	16	21	10	0
Case 16	N	87	33	120	41	28	18	21	12	ND
Case 17	N	97	35	132	47	28	22	23	12	ND
Case 18	N	93	32	125	45	28	20	20	12	1
Case 19 (NEK1)	N	88	30	118	42	28	18	18	12	ND
Case 20	N	85	29	114	39	28	18	17	12	ND
Case 21	N	86	30	116	40	28	18	18	12	ND
Case 22	N	82	33	115	35	27	20	21	12	0
Case 23(C9orf72)	N	86	32	118	40	28	18	20	12	0
Case 24	N	83	29	112	38	27	18	17	12	ND
Case 25	N	82	29	111	36	28	18	17	12	ND
Case 26(SOD1)	N	82	28	110	34	28	20	16	12	ND
Case 27	N	92	32	124	44	28	20	20	12	ND

Summary of ECAS scores for each patient in the cohort, subdivided by subdomain deficits.
ALS, amyotrophic lateral sclerosis; ALSci, cognitively impaired ALS; ECAS, Edinburgh Cognitive and Behavioural ALS Screen; ND, not done.