Table 2.

Cognitive and neuropsychiatric markers in pre-symptomatic and symptomatic mutation carriers versus controls.

Cognitive domain	Cognitive tests	Controls (N=33) (SD; range)	Pre-symptomatic mutation carriers (N=12) (SD; range)	Symptomatic mutation carriers (N=15) (SD; range)
Global cognitive function	MMSE (mean points)	29.4 (0.7; 27 – 30)	29.8 (0.6; 28 – 30)	26.7 (3.8; 16 – 30)**
Memory	WMS (mean Memory quotient)	124.9 (10.8; 105 – 143)	121.6 (11.5; 103 – 137)	110.5 (24.9; 72 – 143)*
	HVLT immediate recall	28.2 (4.3; 16 – 35)	28.0 (2.8; 24 – 32)	21.4 (7.5; 7 – 31)*
	HVLT delayed recall	10.4 (2.3; 6 – 19)	9.8 (1.7; 7 – 12)	6.8 (3.7; 0 – 12)**
Psychomotor speed	TMT A (mean sec)	30.3 (10.7; 15 – 53)	26.6 (8.8; 16 – 44)	54.8 (21.7; 29 – 96)**
Executive function	TMT B (mean sec)	59.9 (22.5; 30 – 105)	54.5 (13.4; 39 – 85)	161.2 (127.1; 67 – 540)**
	DSST (mean nr correct symbols)	77.1 (15.5; 46 – 110)	84.0 (11.8; 62 – 101)	47.3 (17.3; 18 – 79)**
	Clock drawing (median points)	3.0 (0.2; 2 – 3)	3.0 (0.5; 2 – 3)	2.0 (0.6; 1 – 3)**
Language	Letter fluency (mean nr of correct words)	33.9 (9.1; 13 – 50)	32.9 (11.8; 19 – 54)	23.5 (7.9; 12 – 38)*
	Category fluency (mean nr of correct words)	22.2 (5.6; 14 – 40)	23.0 (6.0; 14 – 32)	15.3 (5.5; 3 – 23) *
	BNT (mean nr of correct items)	28.6 (1.0; 27 – 30)	28.3 (1.1; 26 – 30)	23.0 (6.6; 4 – 29)**
Apathy	Apathy scale of Starkstein (mean points)	9.4 (3.7; 2 – 15)	8.9 (5.2; 2 – 20)	8.9 (4.9; 2 – 18)
Anxiety	HADS subscale anxiety (mean points)	3.5 (2.7; 0 – 10)	4.0 (2.8; 0 – 9)	5.5 (3.8; 0 – 13)*
Depression	HADS subscale depression (mean points)	1.9 (2.3; 0 – 9)	1.8 (3.0; 0 – 10)	4.1 (3.2; 0 – 11) *

^*=p<0.05 and

^**=p<0.001; controls vs pre-symptomatic carriers and controls vs symptomatic carriers