Number | 22 | 1 | 17 | 4 |
Sex (F:M) | 14:8 | 1:0 | 10:7 | 3:1 |
Age at onset of disease | 35 (12–57) | 13 | 32 (12–57) | 40 (37–43) |
EDSS before admission or relapse (median (range)) | 8.5 (2.0–9.5) | 3 | 8.5 (6–9.5) | 3.5 (2.0–8.0)* |
Type of ventilatory support | | | | |
Mechanical ventilation only | 9 | 0 | 7 | 2 |
Ventilation and tracheostomy | 9 | 1 | 7 | 1 |
Tracheostomy only | 4 | 0 | 3 | 1 |
Time from onset to mechanical ventilation/tracheostomy (median years (range)) | 17.5 (0.5–32) | 9 | 19 (8–32) | 2.5 (0.5–5)* |
Duration of ventilation (median days (range)) | 8.5 (1–65) | 10 | 8 (1–27) | 50 (5–65) |
Age (years) at time of ventilatory support (median (range)) | 50 (22–81) | 22 | 51 (27–81) | 43 (38–56) |
Indication for ventilation/tracheotomy | | | | |
Respiratory failure only | 13 | | 10 | 3 |
Airway protection only (seizure or coma)† | 4 | 1 | 3 | |
Both† | 5 | | 4 | 1 |
Underlying cause | | | | |
Aspiration pneumonia | 12 | 0 | 10 | 2 |
Mucous plugging | 12 | 1 | 10 | 1 |
Mechanical respiratory failure (PImax ⩽25 cm H2O) | 8 | 0 | 4 | 4 |
Follow up | | | | |
Survival: time to death or last follow up (months) | 16 (0–91) | 17 | 22 (0–91) | 22 (0–60) |
Median time to death after start of ventilation (months) (n = 17) | 22 (0–91) | NA | 22 (0–91) | 5 (0–39) |
Died within 6 months of ventilatory support | 8 | 0 | 6 | 2 |
Survived >3 years after discharge | 10 | 1 | 7 | 2 |
EDSS at discharge (median (range)) | 9.0 (7.5–9) | 8.5 | 9 (7.5–10) | 9 (8.5–10) |
Dead at last follow up | 17 | 0 | 14‡ | 3‡ |