Table 1.
Demographics of subject groups, baseline evaluation.
| Controls | sALS patients | C9orf72 subjects (all) | C9 + diagnosis subgroups |
||||
|---|---|---|---|---|---|---|---|
| Asymptomatic | ALS | ALS-FTD | bvFTD | ||||
| Number | 28 | 22 | 27 | 7 | 11 | 6 | 3 |
| Age (years) | 52.8 ± 9.1 | 55.9 ± 11.1 | 52.2 ± 11.7 | 41.2 ± 11a | 52.3 ± 8.8 | 60.8 ± 10.2 | 60.6 ± 5.1 |
| Male:Female | 18:10 | 11:11 | 15:12 | 1:6 | 5:6 | 6:0 | 3:0 |
| Symptom Duration* (months) |
– | 27.1 ± 17.4 | 33.7 ± 24.0 | – | 30.7 ± 23.9 | 33.7 ± 29.3 | 29.4 ± 26.5 |
| ALSFRS-R | 48 | 40.0 ± 6.9 | 41.6 ± 7.6 | 48 | 36.2 ± 8.4b | 41.8 ± 5.26b | 46.3 ± 2.9 |
| MMSE | 29.0 ± 1.2 | ||||||
| DRS-2 age/educ Scaled score |
– | 8.3 ± 5.1 | 7.6 ± 4.3 | 10.7 ± 2.9a | 8.6 ± 3.6 | 3.8 ± 3.8 | 2.3 ± 2.3 |
| Letter fluency (total for 3 letters) | – | 35.4 ± 11.8 | 28.6 ± 14.4 | 42.3 ± 12.4a | 30.0 ± 11.2 | 13.8 ± 3.0 | 16.0 ± 4.4 |
*Excluding asymptomatic carriers; MMSE mini-mental state exam; DRS-2 — Mattis Dementia Rating Scale, scaled for age and education, normal mean = 10; within C9 + subgroups.
a
Significantly different from C9 + ALS-FTD and bvFTD subgroups.
b
Significantly different from C9 + asymptomatic subjects.