Prognostical factors and corelation with the survival of patients with amyotrophic lateral sclerosis

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder. Median survival from symptom onset is 3 years, but some patients live longer. Identification of prognostic factors will facilitate optimal use of medical and social resources. OBJECTIVE: In order to define the prognostic factor for survival, at the first visit, we investigated our ALS database of patients residents in Belgrade, the last 17 years. MATERIAL AND METHODS: In the period 1992.-2009. 325 patients with the diagnosis of definite or probable ALS were discovered in the Belgrade district. The survival were assessed with the Kaplan-Meier method. The prognostic value of each factor was estimated using Cox proportional hazard analyses. RESULTS: As of December 2009. 267 ALS patients (82.2%) ALS have died. The median survival time was 3 years, (95%CI: 2.86-3.14). Mean survival time from symptoms onset was 4.35 ± 0.20 years. The mean age at onset was 57.74 ± 11.46 years and the mean age at diagnosis was 59.21 ± 11.26 years. Eighty four patients (25.8%) had a bulbar onset and 241 (74.1%) had spinal onset of the disease. Mean survival time from onset for patients with spinal onset was 4.5 ± 0.2 years and 3.7 ± 0.4 years for patients with bulbar onset (p=0.016). Mean survival time for men was 4.40 ± 0.3 years and 4.2 ± 0.3 years for women (p>0.05). In the Cox multivariable model, the factors independently related to a longer survival were younger age, longer diagnostic delay, higher ALSFRS score at first examination and early riluzole treatment. CONCLUSIONS: The identification of younger age, longer diagnostic delay as predictors of prolonged survival in ALS clinic population supports the findings of several, earlier studies. Several additional variables assessed at the first examination predict longer survival: higher ALSFRS score and early riluzole treatment. Gender had no effect on survival. Advanced age and bulbar signs at onset indicated poorer prognosis in our population. UVOD: Amiotrofična lateralna skleroza (ALS) je progresivno neurodegenerativno obolenje sa prosečnim srednjim vremenom preživljavanja od 3 godine. Kod pojedinih bolesnika je, međutim, preživljavanje duže. S obzirom na brz i fatalan tok bolesti, značajno je identifikovati prognostičke faktore preživljavanja u cilju optimalne upotrebe medicinskih i društvenih resursa. CILj RADA: ustanoviti moguće faktore dužeg preživljavanja bolesnika sa ALS u trenutku postavljanja dijagnoze, tokom 17 –ogodišnjeg p