Table 1: CLINICAL CHARACTERISTICS OF ILE416THR CARRIERS AND NON-CARRIERS.

Age at evaluation and age at onset are given as Mean and Standard Deviation. NPI-Q: Cummings Neuropsychiatric Inventory-Questionnaire IR: interquartile range CDR: clinical dementia rating. MCI mild cognitive impairment

	Non-carriers	Ile416Thr carriers
	Total n= 67	Total n= 26	Asymptomatic n=14	MCI n=4	Dementia n=8
Age at onset	NA	NA	NA	47.6 (5.8)	51.6 (5.0)
Age at evaluation	36.5 (14.3)	46.0 (14.4)	36.5 (12.0)	50.8 (3.8)	59.1 (8.7)
CDR
0 (None)	63 (94%)	14 (53,9%)	14 (100%)	0	0
0.5 (Questionable)	4 (6%)	4 (15.4%)	0	4 (100%)	0
1 (Mild)	0	3 (11.5%)	0	0	3 (37.5%)
2 (Moderate)	0	2 (7.7%)	0	0	2 (25%)
3 (Severe)	0	3 (11.5%)	0	0	3 (37.5%)
Depression symptoms	7 (10.4%)	8 (30,8%)	1 (7.1%)	1 (25%)	6 (75%)
Anxiety symptoms	0	6 (23,1%)	1 (7.1%)	1 (25%)	4 (50%)
Delusions	0	3 (11.5%)	0	1 (25%)	2 (25%)
Hallucinations	0	3 (11.5%)	0	1 (25%)	2 (25%)
Insomnia	0	4 (15.4%)	0	1 (25%)	3 (37.5%)
NPI-Q median (IR)	0 (4)	2,00 (10,50)	0,00 (6,25)	9,5 (21,00)	41(NA)
Myoclonus	0	5 (19.2%)	0	0	5 (62.5%)
Seizures	0	4 (15.4%)	0	0	4 (50%)
Traumatic Brain Injury	9 (13,4%)	2 (7.7%)	1 (7.1%)	1 (25%)	0
Headache	0	4 (15.4%)	1 (7.1%)	2 (50%)	1 (12.5%)
Alcoholism	2 (3%)	4 (15.4%)	1 (7.1%)	0	3 (37.5%)
Tobacco use	14 (20,9%)	11 (42.3%)	5 (35.7%)	2 (50%)	4 (50%)
Drug abuse	6 (9%)	2 (7.7%)	2 (14.3%)	0	0
Learning difficulties	11 (16.4%)	4 (15.4%)	3	1	0