Table 1.

Huntington’s disease gene-carrier demographics and clinical characteristics

Variable					Missing data No (% all participants)
					Baseline	Visit 2	Visit 3	Visit 4
Total number of participants	229				–
Male, n (%)	104 (45%)				0
Age at baseline, mean (SD)	44.9 (10.2)				0
CAG, median (min–max)	43 (39–59)				0
DBS at baseline, mean (SD)	333.2 (74.2)				0
Clinical status at baseline, n (%)	Pre-manifest 115 (50%)		Motor manifest 114 (50%)		0
Education Level, median (min—max)	4 (1, 6)				0
Occupational cognitive demands, Mean (SD)	3.70 (0.87)				10 (4%)
Verbal IQ, median (min–max)	UK: 33 (4–47), CA: 34 (11–48) FR: 34 (19–43), NL: 39 (16–48)				0
Composite cognitive score, mean (SD) per visit	−3.85 (3.89)	−3.79 (4.42)	−4.10 (4.52)	−4.23 (4.57)	0	2 (1%)	12 (5%)	35 (15%)
Total GM volume Mean (SD) per visit	0.052 (3.9)	−0.13 (3.9)	−0.17 (3.9)	−0.30 (4.1)	19 (8%)	27 (12%)	54 (24%)	77 (34%)
Caudate Volume Mean (SD) per visit	−0.0012 (0.09)	−0.0070 (0.09)	−0.0114 (0.09)	−0.0231 (0.09)	14 (6%)	22 (10%)	47 (21%)	52 (23%)
UHDRS TMS at baseline median (min–max)	6 (0–52)				0
Visit from baseline (years) mean (SD)	0	0.96 (0.08)	1.99 (0.08)	3.03 (0.10)	0	2 (0.9%)	10 (4%)	28 (12%)
MSH3	Number of participants with 0 3a alleles: 126 1 3a alleles: 71 2 3a alleles: 18		3a allele non-carriers: 126 (55%) 3a allele carriers: 89 (39%)		14 (6%)
FAN1	Number of participants with 0 G alleles: 89 1 G alleles: 107 2 G alleles: 17		G carriers (rs2140734) Non-carriers: 89 (39%) Carriers: 124 (54%)		16 (7%)
BDNF	Number of participants with 0 Met66 alleles: 136 1 Met66 alleles: 73 2 Met66 alleles: 4		Met66 Non-carriers: 136 (59%) Met66 Carriers: 77 (34%)		16 (7%)
COMT	Number of participants with 0 Met158 alleles: 57 1 Met158 alleles: 113 2 Met158 alleles: 43		Val158 carriers: 170 (74%) Met158 homozygotes: 43 (19%)		16 (7%)
MAPT	Number of participants with 0 H2 alleles: 120 1 H2 alleles: 78 2 H2 alleles: 15		H1 homozygotes: 120 (52%) H2 carriers: 93 (41%)		16 (7%)