Table 1.
Demographic and clinical data
| Mean ± SD (range) | ALS (n = 11) | PLS (n = 9) | Asymptomatic genetic carriers (n = 12) | Controls old (n = 10) | Controls young (n = 10) |
|---|---|---|---|---|---|
| Age (years) | 63.5 ± 7.6 (48:74) | 59.6 ± 8.0 (44:70) | 51.7 ± 9.9 (36:66) | 61.7 ± 9.3 (45:75) | 51.0 ± 9.5 (37:64) |
| Gender | 9 M: 2 F | 2 M: 7 F | 2 M: 10 F | 4 M: 6 F | 3 M: 7 F |
| Handedness | 11 R | 9 R | 12 R | 9 R: 1 L | 8 R: 2 L |
| Site of onset or genetics | 1 bulbar | 1 bulbar | 10 SOD1 | N/A | N/A |
| 2 respiratory | 2 both legs | 2 C9orf72 | |||
| 1 RUL, 3 LUL | 1 RLL, 5 LLL | ||||
| 2 RLL, 2 LLL | |||||
| ALSFRS‐r | 34.8 ± 8.8 (21:48) | 35.1 ± 6.3 (24:43) | N/A | N/A | N/A |
| Disease Duration from Symptom Onset (months) | 23.7 ± 18.9 (5:72) | 121.0 ± 57.2 (47:283) | N/A | N/A | N/A |
| Progression Rate (48 – ALSFRS‐R/duration in months) | 0.79 ± 0.69 (0:2.4) | 0.12 ± 0.06 (0.05:0.46) | N/A | N/A | N/A |
| Cognition score (% correct) Intact/Borderline/Impaired | 84.8% ± 8.5 (69:95) | 84.6% ± 9.4 (73:95) | 91% ± 4.5 (80:97) | 96 .1% ± 3.1 (91:100) | 96.3% ± 2.4 (92:99) |
| (10 ECAS, 2 ACE‐r) 8/1/2 | (8 ECAS, 1 ACE‐r) 5/1/3 | (11 ACE‐r) | (9 ACE‐r) | (8 ACE‐r) |
Mean followed by SD (range). Genetic data only available for AGCs.