Table 2:
Demographic and clinical data on ALS study patients
| Sex | Age | Onset | 1Duration (m) | 2Pre-study ΔFRS | Study duration (m) |
3ALSFRS-R |
4ΔFRS | 5Muscle |
6MRC grade |
|||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Entry | End | Entry | End | |||||||||
| ALS1 | M | 59 | Bulbar | 7 | 2.9 | 6* | 28 | 13 | 2.5 | DL | 4 | 4 |
| ALS2 | M | 64 | Bulbar | 8 | 1.5 | 6* | 36 | 21 | 2.5 | DL | 5 | 4 |
| ALS3 | M | 41 | Spinal | 15 | 1.6 | 12 | 24 | 12 | 1.0 | TA | 4 | 2 |
| ALS4† | M | 44 | Spinal | 17 | 1.1 | 12 | 30 | 19 | 0.9 | DL | 5 | 2 |
| ALS5 | M | 54 | Spinal | 47 | 0.3 | 12 | 34 | 27 | 0.6 | BI | 4 | 1 |
| ALS6 | M | 66 | Spinal | 17 | 0.2 | 12 | 44 | 42 | 0.2 | DL | 4 | 4 |
| ALS7† | F | 53 | Spinal | 26 | 0.2 | 12 | 43 | 40 | 0.3 | DL | 5 | 4 |
| ALS8† | M | 62 | Spinal | 16 | 1.2 | 12 | 29 | 27 | 0.2 | DL | 5 | 5 |
1
From symptom onset to study entry.
2
Estimated monthly decline of the revised ALS Functional Rating Scale (ALSFRS-R) score prior to study entry (19).
3
ALSFRS-R score at entry and end of study.
4
Estimated monthly decline of FRS during the study period.
5
Muscle biopsied for CYP27B1 mRNA measurement. BI, biceps; DL, deltoid; TA, tibialis anterior.
6
Medical Research Council (MRC) power grade of biopsied muscle at entry and end of study.
*
Expired before the 12-month visit.
†
Received edaravone treatment during the study period.