Table 2. Neuropsychological Scores of the Probands^a.

	Patient 003 From Family F297b		Patient 005 From Family F523c		Evaluation of Patient 010 From Family F480d	Patient 010 From Family FR1324e
	First Evaluation	Second Evaluation	First Evaluation	Second Evaluation		First Evaluation	Second Evaluation
Age at evaluation, y	67	68	54	56	74	62	63
Mini-Mental Status Examination12
Total score (30)	26	13	NA	NA	22	18	18
Orientation (10)	9	NA	NA	NA	10	NA	NA
Attention (5)	3	NA	NA	NA	1	NA	NA
Encoding (3)	3	NA	NA	NA	3	NA	NA
Recall (3)	3	NA	NA	NA	3	NA	NA
Language (8)	7	NA	NA	NA	5	NA	NA
Praxies (1)	1	NA	NA	NA	0	NA	NA
Mattis Dementia Rating Scale13
Total score (144)	NA	67	NA	74	101	108	114
Initiation (37)	NA	11	NA	6	18	20	22
Concept (39)	NA	16	NA	31	33	30	31
Attention (37)	NA	30	NA	28	32	32	32
Construction (6)	NA	4	NA	3	3	5	5
Memory (25)	NA	6	NA	6	15	11	14
Fluency tasks (2 min)14
Categories (animals)	NA	4	NA	NA	2	4	1
Letter (P)	NA	0	NA	NA	5	2	2
Frontal Assessment Battery15
Total score (18)	12	4	7	NA	3	7	8
Free and cued recall test16
Encoding (16)	NA	0	11	NA	16	0	0
Free recall (48)	NA	NA	2	NA	14	NA	NA
Total recall (48)	NA	NA	24	NA	31	NA	NA
Delayed free recall (16)	NA	NA	NA	NA	4	NA	NA
Delayed total recall (16)	NA	NA	NA	NA	12	NA	NA
No. of intrusions	NA	NA	3	NA	1	NA	NA
Rey’s figure copy (36)17	26	16	NA	NA	NA	31	29
Oral confrontation naming (80)18	NA	60	70	NA	76	65	65

Abbreviations: bvFTD, behavioral variant of frontotemporal dementia; NA, not available.

^aThe maximum score of each test and subtest is indicated in parentheses, unless otherwise specified.

^bAge at onset of bvFTD, 63 years.

^cAge at onset of bvFTD, 48 years.

^dAge at onset of bvFTD, 71 years.

^eAge at onset of bvFTD, 59 years.