Table 1.
Demographics and clinical characteristics of ALS participants and controls
| Fibroblasts | WB | ||||||||
|---|---|---|---|---|---|---|---|---|---|
| NonC9-ALS | C9-ALS | Control | P-value | NonC9-ALS | C9-ALS | Control | P-value | ||
| N | 8 | 8 | 7 | 50 | 15 | 27 | |||
| Age | Years (mean ± SD) | 59.4 ± 6.8 | 58.2 ± 5.9 | 58.4 ± 7.2 | 0.71 | 62.9 ± 12.0 | 57.6 ± 5.7 | 61.6 ± 10.4 | 0.21 |
| Sex | Male | 5 | 4 | 4 | 0.88 | 25 | 6 | 15 | 0.63 |
| Female | 3 | 4 | 3 | 25 | 9 | 12 | |||
| Race | White | 8 | 8 | 7 | 49 | 14 | 23 | ||
| African American | . | . | . | . | . | 4 | |||
| Other/Not reported | . | . | . | 1 | 1 | . | |||
| Onset segment | Bulbar | 1 | 1 | . | 1.00 | 21 | 3 | . | 0.27 |
| Cervical | 2 | 2 | . | 13 | 5 | . | |||
| Lumbar | 5 | 5 | . | 16 | 7 | . | |||
| Initial El Escorial criteria | Definite | 11 | 2 | . | 0.42 | . | . | . | 0.59 |
| Probable | 15 | 8 | . | 6 | 5 | . | |||
| Probable, lab supported | 20 | 4 | . | 2 | 3 | . | |||
| Possible/suspected | 4 | 1 | . | . | . | . | |||
| Missing | . | . | . | . | . | . | |||
| Symptom duration | Days (mean ± SD) | 1268 ± 830 | 589 ± 53 | . | 0.04* | 1062 ± 930 | 579 ± 316 | . | 0.08 |
| ALS-FRS | Points (median + IQR) | 36.0 (34.8–40.0) | 36.0 (34.5–40.5) | . | 0.65 | 36.0 (33.0–41.0) | 35.0 (33.0–44.5) | . | 0.50 |
The significant differences among the groups (NonC9-ALS, C9-ALS and Control) were tested using one-way ANOVA for continuous variables and Chi-Square test for categorical variables with the significance cutoff of 0.05. * indicates P-value < 0.05. SD, Standard Deviation. ALS-FRS: Amyotrophic Lateral Sclerosis Functional Rating Scale. IQR, Inter-Quartile Range.