PROGNOSTIC FACTORS IN THE SURVIVAL OF PAT IENTS SUFFERING FROM NEUROMUSCULAR DISEASE AFTER AN EPISODE OF ACUTE RESPIRATORY-FAILURE

We have studied the survival of 49 patients suffering from neuromuscul ar disease, who were hospitalised in the Respiratory Intensive Care Un it between 1981 and 1990 (29 males and 20 females with a mean age of 4 9.3 +/- 17 years with a range of 15 to 79). The neuromuscular diseases consisted of 8 with multiple sclerosis, 9 with amyotrophic lateral sc lerosis, 8 with Steinert's disease, 11 myopathies, and 10 suffering fr om miscellaneous neurological diseases. Initially 27 of the 49 patient s had been intubated and ventilated. During the hospital stay long-ter m ventilation was undertaken in 2 7 patients (21 by tracheotomy and 6 by nasal mask). The principal prognostic factor was the aetiology. Thr ee groups of varying degrees of severity could be individualized: prog ressive neuromuscular disease (amyotrophic lateral sclerosis and multi ple sclerosis), primary muscle disorders (myopathies and Steinert's di sease), and neuromuscular disease with little or no evolution (surviva l at two years was 15%, 45% and 71% respectively for three groups. p = 0.001 by logrank testing). The other factors which influence survival are age (p <0.01), the presence of false route (p <0.01), and the rea son for hospitalisation (acute as opposed to chronic progressive deter ioration, p <0.05). In a multivariate analysis the most significant fa ctors associated with the diagnosis were age, the reason for hospitali sation, and the existence of false routes. The initial treatment (intu bation) and the prescription of long-term ventilation did not bring wi th it any significant further information as to prognosis, compared to the model which included these four factors.