Table 1.
Summary of patient characteristics.
| ALS | |||||||
|---|---|---|---|---|---|---|---|
| Spinal-Onset | Bulbar-Onset | PLS | BFS | HD | AD | p-Value | |
| Number | 102 | 28 | 8 | 7 | 20 | 29 | |
| Sex F:M a | 33:69 | 15:13 | 8:0 | 1:6 | 11:9 | 18:11 | 0.0001 |
| Age, Years b | 64.2 (31.8–87.5) | 66.7 (45.5–81.0) | 59.9 (46.9–72.5) | 41.5 (27.8–53.1) | 54.4 (24.1–69.1) | 77.23 (34.6–87.0) | <0.0001 |
| Duration, years | 1.8 (0.3–11.2) | 1.3 (0.3–5.1) | 5.3 (0.5–25.2) | 0.4 (0.0–10.7) | NA | NA | 0.0119 |
| BMI (kg/m2) b | 24.1 (16.5–39.9) | 23.4 (16.1–27.4) | 24.6 (18.7–36.9) | 23.5 (20.9–35.9) | 27.9 (20.2–35.4) | NA | 0.2724 |
| ALSFRS-R b | 34 (10–46) | 32 (8–45) | 34 (24–39) | 48 | NA | NA | <0.0001 |
| CK (U/L) b | 194 (35.0–1050.0) | 128 (35.0–593.0) | 68.5 (29.0–150.0) | 92.0 (74.0–199.0) | 142.5 (39.0–1082) | 99.0 (30.0–393.0) | <0.0001 |
| Females | 137 (36.0–1010.0) | 93 (35.0–150.0) | 68.5 (29.0–150.0) | 84.0 | 117 (39.0–1082.0) | 91.5 (38.0–228.0) | 0.0832 |
| Males | 234 (35.0–1050.0) | 154 (55.0–593.0) | NA | 93.5 (74.0–199.0) | 168 (64.0–231.0) | 135 (30.0–393.0) | 0.0030 |
| CK-MB (ng/mL) b | 6.40 (1.20–71.90) | 4.25 (0.80–17.1) | 1.45 (0.90–4.80) | 2.20 (0.80–4.40) | 2.60 (1.00–10.20) | 3.00 (0.90–7.30) | <0.0001 |
| Females | 5.10 (1.90–23.70) | 3.80 (0.80–17.10) | 1.45 (0.90–4.80) | 2.80 | 2.40 (1.00–10.20) | 2.60 (0.90–6.00) | 0.0007 |
| Males | 7.60 (1.20–71.90) | 5.00 (1.50–14.00) | NA | 2.10 (0.80–4.40) | 3.00 (1.40–5.20) | 3.20 (0.90–7.30) | <0.0001 |
| Creatinine (mg/dL) | 0.70 (0.20–1.87) | 0.73 (0.48–0.16) | 0.73 (0.60–1.20) | 0.83 (0.60–1.20) | 0.92 (0.60–1.08) | NA | 0.0298 |
| cTnT (ng/L) | 21.20 (3.0–300.0) | 12.95 (3.0–77.90) | 3.00 (3.00–9.30) | 3.10 (3.00–6.90) | 4.20 (3.00–13.90) | 10.70 (3.00–30.0) | <0.0001 |
| pNfH in CSF (pg/mL) b |
2219 (n = 50) (336–101725) |
1959 (n = 12) (592.3–4205) |
2541 (n = 2) (1400–3681) |
90 (n = 7) (62.5–387) |
246 (n = 3) (130.2–690.0) |
NA | 0.0002 |
a χ2 test of independence. b Kruskal–Wallis test. AD, Alzheimer’s disease; ALSFRS-R, revised ALS Functional Rating Scale; BFS, benign fasciculation syndrome; BMI, body mass index; CK, Creatinine kinase; CK-MB, Creatine Kinase MB Isoenzyme; CSF, cerebrospinal fluid; F, females; HD, Huntington’s disease; M, males; NA, data not available; pNfH, phosphorylated neurofilament heavy chain. All data are presented in medians and range in parenthesis.