title: Riluzole and prognostic factors in amyotrophic lateral sclerosis long-term and short-term survival: a population-based study of 1149 cases in Taiwan.
abstract: BACKGROUND
  Amyotrophic lateral sclerosis (ALS) is a rare disease in Taiwan; thus, estimation of ALS mortality is difficult. We evaluated factors associated with ALS survival in Taiwan.
  
  
  METHODS
  The study enrolled 1149 Taiwanese with a primary diagnosis of ALS during 1999-2008. Follow-up information was available for all patients; mean (SD) duration of follow-up was 2.91 (2.62) years. Medical interventions, including noninvasive positive pressure ventilation (NIPPV), tracheotomy, gastrostomy, and riluzole, were included in time-dependent survival analysis.
  
  
  RESULTS
  Of the 1149 ALS patients, 438 (38.12%) died during follow-up. Mortality in the first year was 16%, which was 13 times (95% CI 11.1-15.2) the age- and sex-standardized rate of the general population in Taiwan. The average annual crude mortality rate was 13.1% (person-years). Factors significantly associated with increased mortality were male sex, advanced age, rural residence, lower economic status, no tracheotomy, and no riluzole treatment. Significant predictors of long-term versus average survival were younger age at diagnosis, being a dependent or receiving social welfare, and NIPPV support. Significant predictors of short-term versus average survival were older age, being employed, no tracheotomy, and no riluzole use.
  
  
  CONCLUSIONS
  The results support the use of riluzole to improve ALS survival. Patients who received riluzole and underwent tracheotomy had the best survival.
authors.affiliation: Department of Public Health, Kaohsiung Medical University, Kaohsiung, Taiwan.
authors.forename: Charles Tzu-Chi
authors.initials: CT
authors.lastname: Lee
authors.forename: Yi-Wen
authors.initials: YW
authors.lastname: Chiu
authors.forename: Kai-Chen
authors.initials: KC
authors.lastname: Wang
authors.forename: Chi-Shin
authors.initials: CS
authors.lastname: Hwang
authors.forename: Kuan-Hsiang
authors.initials: KH
authors.lastname: Lin
authors.forename: I-Ta
authors.initials: IT
authors.lastname: Lee
authors.forename: Ching-Piao
authors.initials: CP
authors.lastname: Tsai
mesh_terms: D000328:Adult; D017677:Age Distribution; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000401:mortality / Q000628:therapy*; D016208:Databases, Factual; D005260:Female; D005500:Follow-Up Studies; D005774:Gastrostomy; D006801:Humans; D008297:Male; D008875:Middle Aged; D018696:Neuroprotective Agents / Q000627:therapeutic use*; D011175:Positive-Pressure Respiration; D011379:Prognosis; D012189:Retrospective Studies; D019782:Riluzole / Q000627:therapeutic use*; D012307:Risk Factors; D017678:Sex Distribution; D012959:Socioeconomic Factors; D016019:Survival Analysis; D013624:Taiwan / Q000453:epidemiology; D013997:Time Factors; D014140:Tracheotomy
chemical_list: D018696:Neuroprotective Agents; D019782:Riluzole