title: Epidemiology of amyotrophic lateral sclerosis and effect of riluzole on disease course.
abstract: OBJECTIVE
  To assess the epidemiology of ALS in Austria and to evaluate the long-term effect of riluzole treatment on survival.
  
  
  METHODS
  Hospital discharge and riluzole prescription databases were used to identify ALS cases from January 2008 to June 2012. Using the capture-recapture method we evaluated the incidence and prevalence of ALS and patients' survival in dependence of age, gender and riluzole treatment.
  
  
  RESULTS
  The corrected incidence and prevalence of ALS were 3.13/100,000 person-years (95% CI, 2.77 to 3.50) and 9.14/100,000 persons (95% CI, 8.53 to 9.79), respectively. Median survival from diagnosis was 676 days (95% CI, 591 to 761). A younger age at diagnosis was associated with a longer survival. Gender did not appear to affect survival time. Riluzole therapy was associated with a survival advantage only for the initial treatment period. The adjusted hazard ratio of mortality for using riluzole increased continually over time resulting in an apparent reversal of its beneficial effect after 6 months of therapy.
  
  
  CONCLUSIONS
  We report incidence and prevalence estimates that are on the upper end of the wide range discussed in literature. Riluzole seems to exert a beneficial effect only in the first 6 months of therapy.
authors.affiliation: Department of Neurology, Medical University of Vienna, Vienna, Austria.
authors.forename: Hakan
authors.initials: H
authors.lastname: Cetin
authors.forename: Jakob
authors.initials: J
authors.lastname: Rath
authors.forename: Judith
authors.initials: J
authors.lastname: Füzi
authors.forename: Berthold
authors.initials: B
authors.lastname: Reichardt
authors.forename: Gerhard
authors.initials: G
authors.lastname: Fülöp
authors.forename: Stefan
authors.initials: S
authors.lastname: Koppi
authors.forename: Marcus
authors.initials: M
authors.lastname: Erdler
authors.forename: Gerhard
authors.initials: G
authors.lastname: Ransmayr
authors.forename: Jörg
authors.initials: J
authors.lastname: Weber
authors.forename: Kurt
authors.initials: K
authors.lastname: Neumann
authors.forename: Michael
authors.initials: M
authors.lastname: Hagmann
authors.forename: Wolfgang N
authors.initials: WN
authors.lastname: Löscher
authors.forename: Eduard
authors.initials: E
authors.lastname: Auff
authors.forename: Fritz
authors.initials: F
authors.lastname: Zimprich
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000188:drug therapy / Q000453:epidemiology* / Q000401:mortality; D001317:Austria / Q000453:epidemiology; D016208:Databases, Factual; D018450:Disease Progression; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D018696:Neuroprotective Agents / Q000627:therapeutic use*; D015995:Prevalence; D011379:Prognosis; D019782:Riluzole / Q000627:therapeutic use*; D012737:Sex Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D018696:Neuroprotective Agents; D019782:Riluzole