Details of 24 patients with motor neurone disease (MND) presenting with acute respiratory failure
| Previously diagnosed (n=7) | Not previously diagnosed (n=17) | |
|---|---|---|
| ALS, amyotrophic lateral sclerosis form of motor neurone disease; PBP, progressive bulbar palsy; PMA, progressive muscular atrophy. *All patients with PBP and PMA forms of motor neurone disease subsequently progressed to fulfil the El Escorial criteria for the diagnosis of ALS. | ||
| Sex male:female | 5:2 | 15:2 |
| Mean (SD) age at first symptoms (years) | 57.8 (7.3) | 67.1 (6.1) |
| Mean (SD) age at intubation (years) | 60.3 (7.1) | 67.8 (6.2) |
| Time (range) between onset of symptoms and intubation (months) | 24 (6–84) | 6 (0–30) |
| Site of onset | ||
| Lower limb | 5 | 4 |
| Upper limb | 1 | 3 |
| Upper and lower limbs | 3 | |
| Bulbar | 1 | 4 |
| Diaphragm | 3 | |
| Initial diagnosis | ||
| ALS | 6 | 10 |
| PBP* | 1 | 4 |
| PMA* | 3 | |
| Cause of deterioration | ||
| Pneumonia | 5 | 4 |
| Rapidly progressive dyspnoea | 1 | 6 |
| Under investigation when developed respiratory arrest | 5 | |
| Nocturnal seizure | 1 | |
| After routine general anaesthetic | 1 | |
| Intubated overseas | 1 | |