TABLE 1.

Longitudinal clinical scores and digital measures in patients with Friedreich ataxia and healthy controls.

	Controls			FRDA
	Baseline	Follow-up	p-value	Baseline		Follow-up	p-value*
N	10	10		20	20
SARA	0	0	-	17.5 ± 6.1	19.0 ± 6.2		<0.01
mFARS	1.0 ± 1.4	0.0 ± 0.0	-	51.3 ± 14.0	52.1 ± 12.5		-
ADL	0	0	-	13.1 ± 5.1	14.1 ± 4.6		-
CCFS	962.8 ± 309.2	847.4 ± 49.0	-	1,233.6 ± 128.6	1,240.1 ± 112.2		-
8MWT #	4.2 ± 0.7	5.7 ± 0.7	-	7.5 ± 2.5	8.1 ± 2.2		-
Metabolic Equivalent of task rate
wais	1.19 ± 0.09	1.18 ± 0.09	-	1.02 ± 0.02	1.01 ± 0.01		-
wrist	1.65 ± 0.16	1.61 ± 0.14	-	1.37 ± 0.27	1.33 ± 0.24		-
N. of activity bouts
Waist	8.8 ± 4.4	6.5 ± 5.8	-	0.2 ± 0.9	0.0 ± 0.0		-
Wrist	48.7 ± 16.3	36.4 ± 13.1	-	26.7 ± 30.5	19.1 ± 23.5		-
Sedentary activity (%)
Waist	67.7 ± 6.7	69.0 ± 4.9	-	81.3 ± 9.4	84.1 ± 7.9		<0.01
wrist	31.7 ± 8.1	32.2 ± 8.2	-	42.9 ± 13.0	45.8 ± 13.1		-
Light activity (%)
Waist	21.8 ± 5.3	20.2 ± 5.0	-	16.0 ± 7.4	13.8 ± 6.6		<0.01
wrist	24.9 ± 3.1	25.8 ± 5.8	-	25.8 ± 5.0	26.7 ± 4.6		-
Moderate-Vigorous activity (%)
Waist	10.5 ± 3.2	10.8 ± 2.6	-	2.7 ± 2.4	2.1 ± 1.7		-
wrist	43.4 ± 8.9	42.0 ± 7.0	-	31.3 ± 13.3	28.2 ± 12.1		0.01
Vector Magnitude (CPM)
Waist	563.2 ± 159.0	535.2 ± 15.7	-	277.0 ± 155.3	228.8 ± 126.5		<0.01
wrist	2387.3 ± 474.7	2285.8 ± 333.3	-	1,656.5 ± 758.9	1,517.0 ± 679.1		-
Steps/minute
Waist	16.0 ± 3.6	15.7 ± 1.9	-	7.7 ± 4.1	6.5 ± 3.4		-
wrist	20.2 ± 3.5	18.4 ± 5.6	-	14.0 ± 5.1	12.6 ± 4.9		-

p-value after Bonferroni-corrected paired Wilcoxon test between baseline and follow-up; only p < 0.05 are reported. Data are mean ± SD; SARA: Scale for the Assessment and Rating of Ataxia. mFARS: modified Friedreich Ataxia Rating Scale; ADL: Activity of Daily Living part of FARS; CCFS: cerebellar composite functional severity score; CPM: counts per minute.

b8MWT: 8 m walk test (seconds), was performed at follow-up by 9 patients.