A Predictive Model for Prognosis in Motor Neuron Disease

Objectives: There have been attempts to establish biomarkers for motor neuron disease, without success. The study aim to seek possible markers to be used in the clinical routine evaluation, to optimize timing for palliative interventions. Methods: A cohort study evaluated clinical, respiratory and neurophysiological variables every 3-4 months across 20 months in 101 patients with motor neuron disease using riluzole. Primary endpoint was death or tracheostomy. The most significant parameters in cox regression analysis created a predictive model. Results: There were 58 men and 43 women with a mean age of 57.2 ± 11.7 years. 77 patients (76.2%) had spinal onset and 24 (23.8%) had bulbar onset. The mean survival time was 43.5 ± 5.7 months (CI 95% 32.3-54.8). The variables related to worse prognosis were: age > 65 years (HR=2.50, CI 95% (1.23–5.08); involvement of a second site in less than six months (HR=2.02, CI 95% (1.04 – 3.94); supine Forced Vital Capacity <63% (HR=2.78, CI 95% 1.03–7.48), neck weakness (HR=2.28, CI 95% (1.03–5.05) and presence of pyramidal syndrome (HR= 2.36, CI 95% (1.05–5.33). Conclusion: It was created a five-factors set that predicts evolution to death or tracheostomy within one year.