Table 1

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:female5:215:2
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 limb54
    Upper limb13
    Upper and lower limbs3
Initial diagnosis
Cause of deterioration
    Rapidly progressive dyspnoea16
    Under investigation when developed respiratory arrest5
    Nocturnal seizure1
    After routine general anaesthetic1
    Intubated overseas1