Table 1.
Cohort Characteristics: Demographics, Clinical Features, and Spectral Quality*
| Visit 1 | Visit 2 | Visit 3 | ||||
|---|---|---|---|---|---|---|
| ALS | Controls | ALS | Controls | ALS | Controls | |
| Sample size n | 19 | 18 | 14 | 15 | 10 | 13 |
| Time of exam after Visit 1, months | ‒ | ‒ | 6.6 ± 1.1 | 6.3 ± 0.8 | 12.8 ± 1.4 | 12.3 ± 1.0 |
| Sex ratio, male:female | 10:9 | 10:8 | 8:6 | 7:8 | 6:4 | 6:7 |
| Age, years | 57 ± 9 (31–74) |
57 ± 9 (30–69) |
58 ± 10 (32–71) |
59 ± 7 (48–69) |
58 ± 11 (32–70) |
58 ± 10 (31–69) |
| Brain hemisphere scanned, left:right | 8:11 | 8:10 | 5:9 | 7:8 | 3:7 | 6:7 |
| Riluzole use, yes:no | 8:11 | ‒ | 7:7 | ‒ | 4:6 | ‒ |
| Disease duration, months | 40.3 ± 43.1 (3.5–148.1) |
‒ | 47.1 ± 49.4 (9.9–154.2) |
‒ | 43.2 ± 41.8 (15.9–156.5) |
‒ |
| Progression rate, points per month | 0.37 ± 0.32 (0.03–1.43) |
‒ | 0.32 ± 0.20 (0.03–0.65) |
‒ | 0.41 ± 0.27 (0.03–1.01) |
‒ |
| King’s Disease Stage (no. of patients) | Stage 1 (6) Stage 2 (6) Stage 3 (6) Stage 4 (1) |
‒ | Stage 1 (4) Stage 2 (6) Stage 3 (2) Stage 4 (2) |
‒ | Stage 1 (1) Stage 2 (2) Stage 3 (2) Stage 4 (5) |
‒ |
|
ALSFRS-R Total Score (0 most severe ‒ 48 normal) |
39.8 ± 5.6 (26–45) |
‒ | 39.7 ± 4.1 (33–47) |
‒ | 36.4 ± 5.9 (26–45) |
‒ |
|
ALSFRS-R Upper Limb Subscore
(0 most severe ‒ 12 normal) |
8.7 ± 3.2 (0–12) |
‒ | 9.3 ± 1.6 (7–12) |
‒ | 8.4 ± 1.8 (5–12) |
‒ |
|
ALSFRS-R Bulbar Subscore
(0 most severe ‒ 12 normal) |
10.3 ± 1.9 (6–12) |
‒ | 9.7 ± 2.8 (4–12) |
‒ | 8.8 ± 3.8 (0–12) |
‒ |
|
Contralateral Upper Limb UMN Score** (0 normal ‒ 5 most severe) |
1.9 ± 1.7 (0–5) |
‒ | ‒ | ‒ | 1.9 ± 2.0 (0–5) |
‒ |
|
ECAS Total Score (0 most severe ‒ 136 normal) |
115.3 ± 6.5 (99–127) |
119.3 ± 8.8 (103–129) |
116.7 ± 8.2 (106–135) |
123.0 ± 7.1 (107–132) |
117.5 ± 6.6 (106–129) |
122.4 ± 7.2 (108–134) |
|
ECAS ALS-Specific Subscore (0 most severe ‒ 100 normal) |
86.3 ± 4.9 (74–95) |
‒ | 87.2 ± 6.3 (77–100) |
‒ | 86.6 ± 5.1 (80–97) |
‒ |
| Motor Cortex Spectra SNR | 205 ± 37 | 201 ± 34 | 215 ± 48 | 198 ± 45 | 208 ± 26 | 202 ± 44 |
| Motor Cortex Spectra Linewidth, Hz | 11 ± 2 | 11 ± 2 | 10 ± 2 | 11 ± 2 | 10 ± 2 | 11 ± 2 |
| Pons Spectra SNR | 45 ± 10 | 53 ± 17 | 51 ± 10 | 56 ± 16 | 52 ± 10 | 62 ± 12 |
| Pons Spectra Linewidth, Hz | 16 ± 2 | 15 ± 2 | 14 ± 2 | 15 ± 2 | 15 ± 2 | 15 ± 2 |
Data are given as counts or mean ± SD and range. Comparisons were made between patients and controls using Fisher’s exact tests (sex ratio and brain hemisphere scanned) and unpaired, two-tailed Student’s t-tests. Total ECAS scores trended lower in patients compared with controls at each visit. All other parameters were not different between groups.
Abbreviations: ALSFRS-R = ALS Functional Rating Scale-Revised; UMN = upper motor neuron; ECAS = Edinburgh Cognitive and Behavioral ALS Screen.
Pons spectra were acquired from fewer participants (see Results: 1H-MRS Data Completeness and Quality).
Neuromuscular examinations were conducted at Visits 1 and 3. Contralateral Upper Limb UMN burden was assessed in all patients at Visit 1 and in 7 patients at Visit 3.