Table 1.
Demographics of participants
| Healthy Controls |
C9+ Symptomatic* carriers |
C9+ Presymptomatic carriers |
|
|---|---|---|---|
| Age | 51.4 ± 9.3 | 57.1 ± 9.8 | 43.4 ± 9.7a |
| % Male | 53% | 63% | 20% |
| Symptom Duration (mos) | 36.6 ± 25.1 | ||
| ALSFRS-R | 48 | 35.9 ± 7.3 | 47.9 ± 0.4 |
| Letter Fluency (words/letter) Baseline | 14.3 ± 3.6 (n=22) | 7.4 ± 4.1* (n=26) | 12.7 ± 4.3 |
| 6-month follow-up | - | - | 14.6 ± 4.3 |
| 18-month follow-up (N=12) | - | - | 15.1 ± 3.4 |
| Cross-sectional N | 34 | 27 | 15 |
| Interval (N with 2 scans) | 4.85 ± 2.79 (N=21) | 5.87 ± 0.74 (N =17) | 5.71 ± 1.17 (N=14) |
| Interval (N with 3 scans) | 18.31 ± 2.11 (N=21) | 17.94 ± 0.92 (N= 8) | 18.25 ± 1.17 (N=13) |
a
C9+ presymptomatic < HC and C9+ symptomatic, ANOVA p<0.05 corrected
*
17 ALS/6 ALS-FTD/4 Dementia