Table 1.
Study participant characteristics
| All Participants | Longitudinal Subset | ||||||||
|---|---|---|---|---|---|---|---|---|---|
| Control (N=34) |
At-risk (N=79) |
Converter (N=14) |
Affected (N=22) |
Control (N=13) |
At-risk (N=55) |
Converter (N=10) |
Affected (N=16) |
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| # of collections | Median (Range) |
(n/a) | 2 (2-6) |
3 (2-7) |
3.5 (2-7) |
3 (2-7) |
|||
| Follow-up duration (years) | Median (Range) |
1.5 (0.6-7.8) |
3.7 (1.0-8.7) |
2.7 (0.3-4.0) |
1.0 (0.3-2.6) |
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| Baseline age (years) | Mean ± SD (Range) |
46.4 ± 11.4 (24.2-69.2) |
44.8 ± 12.4 (18.9-77.0) |
51.1 ± 12.2 (31.9-74.8) |
59.2 ± 7.7 (45.6-75.8) |
48.1 ± 11.9 (34.5-69.2) |
47.0 ± 11.4 (18.9-67.6) |
51.8 ± 11.7 (32.0-74.8) |
59.8 ± 6.9 (45.6-67.7) |
| Male | N (%) | 15 (44%) | 28 (35%) | 7 (50%) | 12 (55%) | 4 (31%) | 19 (35%) | 3 (30%) | 9 (56%) |
| Genotype |
SOD1 A4V SOD1 nonA4V C9ORF72 HRE Other Unknown |
(n/a) | 27 22 25 5 0 |
9 2 2 1 0 |
1 1 5 0 15 |
(n/a) | 21 17 13 4 0 |
6 2 1 1 0 |
1 0 4 0 11 |
| Site of onset | Bulbar Limbs Other Unknown |
(n/a) | 2 10 2 0 |
2 18 0 2 |
(n/a) | 1 7 2 0 |
1 13 0 2 |
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| Baseline years since onset | Median (Range) |
−1.6 (−6.0, −0.1) |
2.2 (0.7, 7.6) |
−1.8 (−6.0, −0.1) |
2.0 (0.7, 7.6) |
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| Baseline years since diagnosis | Median (Range) |
−1.7 (−6.2, −0.3) |
1.0 (0.1, 3.4) |
−1.9 (−6.2, −0.3) |
0.9 (0.1, 3.4) |
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| Baseline ALSFRS-R | Mean ± SD (Range) |
(n/a) | 34.2 ± 7.8 (9-44) a,b |
(n/a) | 35.4 ± 8.5 (9-44) a,c |
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| Baseline ∆FRS | Mean ± SD (Range) |
(n/a) | 0.53 ± 0.38 (0.14-1.45) a |
(n/a) | 0.49 ± 0.34 (0.14-1.31) a |
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Baseline = first visit at which serum sample was available (with or without contemporaneous CSF collection)
Follow-up duration = time between the participant’s first and last serum sample included in this study
(n/a) = not applicable.
a
Baseline ALSFRS-R not available for N=2
b
Excluding N=1 with baseline ALSFRS-R=9, 35.5 ± 5.2 (27-44).
c
Excluding N=1 with baseline ALSFRS-R=9, 37.4 ± 4.0 (31-44).