Table 1.

Patient Characteristics

	bvFTD patients (n=104)
Age at disease onset– yr	54.8 ± 9.5
Age at first evaluation – yr	60.8 ± 8.2
Male sex – no. (%)	63 (60.6)
White – no. (%)	94 (90.4)
Right handedness – no. (%)	93 (89.4)
Education – yr	16.8 ± 9.0
MMSE at initial evaluation	22.5 ± 7.1
CDR at initial evaluation	1.4 ± 0.7
CDRSOB at initial evaluation	7.3 ± 3.5
FTLD-CDRSOB at initial evaluationa	9.9 ± 4.2
Mutation carriers – no. (%)b	27 (27.6)
C9ORF72 – no. (%)	15 (55.6)
GRN – no. (%)	6 (22.2)
MAPT – no. (%)	6 (22.2)
GRN T allele frequency – no. (%)	41 (31.1)c
Tau H1 allele frequency – no. (%)	107 (81.1)

Abbreviations: bvFTD = behavioral variant frontotemporal dementia; CDR = Clinical Dementia Rating; CDRSOB = CDR Sum of Boxes; C9ORF72 = chromosome 9 open reading frame 72 hexanucleotide expansions; FTLD-CDRSOB = Frontotemporal Lobar Degeneration - modified CDRSOB; GRN = progranulin; MAPT microtubule-associated protein tau; MMSE = Mini Mental State.

Age, MMSE, CDR, CDRSOB, and FTLD-CDRSOB, at initial evaluation represent patient characteristics measured within one year of first presentation. Values for age, education, MMSE, CDR, CDRSOB, and CDR-FTLD, are mean ± standard deviations. Race or ethnic group was self-reported. Scores on the MMSE range from 0 to 30, with higher scores denoting better cognitive function. Scores on the CDR, CDRSOB, and FTLD-CDRSOB range from 0 to 3, 0 to 18 and 0 to 24, respectively, with higher scores denoting more disability.

^an=89 bvFTD patients had FTLD-CDRSOB recorded at their first evaluation.

^bpercentages are calculated for the patients who underwent genetic testing (n=98).

^cP=.054, compared to allele frequency of normal population using Chi-square test.