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Predictors of survival in amyotrophic lateral sclerosis
Introduction: Amyotrophic lateral sclerosis (ALS) is a progressive, degenerative disorder of upper and lower motor neurons with a progressive bulbar or limbic muscular atrophy. The average survival in ALS patients lies between 3 to 5 years, but the survival in an individual ALS-patient is known to have a wide variety and is considered to be difficult to predict.
Methods: We identified 479 patients in our ALS Database who fulfilled the diagnostic criteria for probable or definite ALS according to the El Escorial criteria of the World Federation of Neurology and who have regularly been followed at our ALS-clinic in a time period from 1996 to 2006. Demographic, clinical and electro-physiological data were collected at each clinical visit. The ALSFRS-R (ALS Functional Rating Scale Revised) score is performed at each follow-up visit at approximately 3-month intervals for evaluations of disease progression. Major interventions such as non-invasive ventilation (NIV) or percutaneous endoscopic gastrostomy (PEG) are recorded during follow-up. The descriptive analyses and correlation (Pearson test two sided) were performed with SPSS-Software.
Results: The study cohort consisted of 479 patients, 269 men and 210 women. The mean age at the time of disease onset was 58 years (SD 12), ranging from 25 to 89 years. 165 of the analyzed patients had died at time of analyse. Survival from symptom-onset ranged from 4 months up to 11.9 years. 5% of patients died within 1 year after symptom-onset (age: 43–77 years), 81% (age: 34–85 years) of the patients lived one to five years after first symptoms, 12% (Age: 38–75 years) survived five to 10 years, and 2% lived longer than 10 years after the first symptom.
Discussion: Older age at onset, bulbar onset, lower initial FVC, shorter time between first symptom and first visit, lower ALSFRS-R score at first contact, higher rate of ALSFRS-R score between first symptom and first contact or during whole disease correlate with a higher risk of death. Predictors of survival are important for designing new trials and to randomize patients correctly.