Table 2.
Comprehensive flow of ALS progression before and after PEG tube insertion.
| Diagnosis(t2) | T-rec for gastrostomy(t3) | PEG insertion(t4) | One-year post-PEG(t5) | |
|---|---|---|---|---|
| Duration from onset (months, median (IQR)) | 8 (5–13) | 22 (15–30) | 30 (20–44) | 41 (29–55) |
| Bulbar onset | 9 (6–15) | 20 (12–30) | 23 (19–41) | 34 (26–53) |
| Limb onset | 8 (5–12) | 23 (15–31.5) | 32 (23–47) | 43 (33.5–57) |
| ALSFRS-R score (points, median (IQR)) | 41 (37–44) | 30.5 (25–35) | 17 (10–24) | 6.5 (0–14) |
| Bulbar onset | 41 (37–44) | 35 (30–38) | 24 (16–30) | 8 (0–18) |
| Limb onset | 41 (37–44) | 28 (23–34) | 14 (8–19) | 5 (0–13) |
| ALSFRS-R bulbar score (points, median (IQR))a | 10 (9–12) | 8 (6–9) | 4 (3–6) | |
| Bulbar onset | 9 (7–10) | 7 (5–7) | 4 (2–5) | |
| Limb onset | 11 (10–12) | 8 (7–9) | 5 (3–6) | |
| ALSFRS-R swallowing score (points, median (IQR))b | 3 (3–4) | 2 (2–3) | 1 (0–1) | |
| Bulbar onset | 3 (3–3) | 2 (2–2) | 1 (0–2) | |
| Limb onset | 4 (3–4) | 2 (2–3) | 1 (0–1) | |
| Rate of ALSFRS-R decline (points, median (IQR))c | 0.8 (0.5–1.4)d | 0.7 (0.3–1.2)e | 1.7 (0.9–4.0)f | 0.6 (0.1–1.9)g |
| Bulbar onset | 0.7 (0.4–1.3) | 0.5 (0.1–1.0) | 2.1 (0.9–4.7) | 1.0 (0.3–2.5) |
| Limb onset | 0.8 (0.5–1.4) | 0.8 (0.5–1.2) | 1.6 (0.9–3.4) | 0.5 (0.1–1.3) |
| BMI (kg/m2, mean ± SD) | 22.7 ± 3.2 | 21.3 ± 3.1 | 19.5 ± 3.4 | |
| Bulbar onset | 22.2 ± 3.2 | 21.1 ± 3.3 | 19.7 ± 3.5 | |
| Limb onset | 23.0 ± 3.2 | 21.5 ± 3.0 | 19.4 ± 3.3 | |
| Weight loss (%, mean ± SD) | 6.1 ± 5.3 | 13.7 ± 11.8 | ||
| Bulbar onset | 4.9 ± 5.5 | 11.4 ± 10.2 | ||
| Limb onset | 6.7 ± 5.0 | 15.0 ± 12.5 |
T-rec for gastrostomy, Recommended time for PEG insertion; PEG, percutaneous endoscopic gastrostomy; ALSFRS-R, amyotrophic lateral sclerosis functional rating scale-revised; IQR, interquartile range; BMI, body mass index; SD, standard deviation.
aSum of the bulbar subset of ALSFRS-R questions 1–3 (speech, salivation, swallowing) ranges from 0 to 12 points; bScore for ALSFRS-R question 3 (swallowing) ranges from 0 to 4 points; cThe disease progression was measured by the rate of decline in ALSFRS-R score using the following formula: the rate of decline = (difference of ALSFRS-R score between the two-time points) / (duration between the two time points in months). dThe median (IQR) rate of decline from symptom onset(t1) to diagnosis(t2); eThe median (IQR) rate of decline from diagnosis(t2) to T-rec for gastrostomy(t3); fThe median (IQR) rate of decline from T-rec for gastrostomy(t3) to PEG insertion(t4); gThe median (IQR) rate of decline from PEG insertion(t4) to one year past PEG insertion (t5).