Screening for cognition and behaviour changes in ALS

Figures & data

Table I. Normative data on the ECAS.

	Mean (SD)	Range	Abnormality cut-off
Executive functions (max 48)	40.48 (3.54)	33–46	33
Language functions (max 28)	27.63 (0.70)	26–28	26
Fluency (max 24)	19.85 (2.50)	14–24	14
ALS-Specific functions (max 100)	87.95 (4.98)	75–97	77
Memory functions (max 24)	18.68 (2.73)	12–23	13
Visuospatial functions (max 12)	11.85 (0.48)	10–12	10
ALS Non-specific functions (max 36)	30.53 (2.96)	22–35	24
ECAS Total score (max 136)	118.48 (6.64)	99–128	105

Max: maximum score; ALS-Specific functions consists of the total score of Executive, Language and Fluency scores. ALS Non-specific functions consist of the total score of Memory and Visuospatial functions. ECAS Total score is the combined score for ALS-Specific and ALS Non-specific functions. Cut-off is based on 2 SD from the mean. A score at or below this value indicates impairment.

Table II. Characteristics of ALS patients and healthy controls.

	ALS patients	Healthy controls
Age	59.98 (11.36) 34–86	59.20 (12.57) 37–87
Years of education	11.54 (2.15) 10–18	12.28 (2.52) 9–20
Duration of illness (months)	25 5–180
ALSFRS	34.93 (8.51) 13–48

ALSFRS: ALS Functional Rating Scale. Mean (SD) and range are reported other than for duration for which median and range are provided.

Figure 1. ECAS subdomains: frequency of abnormal performance in ALS patients across each cognitive domain.

Table III. ECAS scores: frequency of abnormal performance in ALS group.

	Frequency of abnormality	Percentage of Total ALS Group falling below cut-off
ALS-Specific functions	14	29.17%
ALS Non-specific functions	3	6.25%
ECAS Total score	14	29.17%

Figure 2. Behaviour domains: frequency of behavioural impairment.