title: The incidence of amyotrophic lateral sclerosis in Friuli Venezia Giulia, Italy, from 2002 to 2009: a retrospective population-based study.
abstract: BACKGROUND
  We conducted a retrospective population-based study to estimate the incidence of amyotrophic lateral sclerosis (ALS) in Friuli Venezia Giulia, Italy, from 2001 to 2009.
  
  
  METHODS
  Multiple sources were used for case ascertainment: Health databases, archives of the neurology departments and of the regional chapter of the Italian ALS Association. The diagnosis was validated through clinical documentation review. Crude and standardized incidence rates (IRs) per 100,000 person-years were calculated.
  
  
  RESULTS
  We identified 262 incident ALS cases, 50.4% men, 4.2% familial. Half of the patients had spinal onset (56.8% in men) and 25.2% bulbar (29% in women). Bulbar onset had a similar frequency in women (31.7%) and men (31.5%) aged 67 or above at diagnosis. The crude IR was 2.72 (95% confidence interval, 95% CI, 2.39-3.05) and the male:female ratio 1.08. The IR peaked in the 65-74 age group, with a second increase in men 85 years and older. The IR standardized to the 2001 Italian population was 2.38 (95% CI 2.13-2.63) and to the 2000 European population 2.58 (95% CI 2.34-2.81).
  
  
  CONCLUSIONS
  This retrospective study found IRs of ALS in the range of Italian and European prospective population-based registries, suggesting an almost complete case ascertainment.
authors.affiliation: Department of Medical and Biological Sciences, University of Udine, Udine, Italy.
authors.forename: Daniela
authors.initials: D
authors.lastname: Drigo
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Verriello
authors.forename: Elena
authors.initials: E
authors.lastname: Clagnan
authors.forename: Roberto
authors.initials: R
authors.lastname: Eleopra
authors.forename: Gilberto
authors.initials: G
authors.lastname: Pizzolato
authors.forename: Alessio
authors.initials: A
authors.lastname: Bratina
authors.forename: Delia
authors.initials: D
authors.lastname: D'Amico
authors.forename: Arianna
authors.initials: A
authors.lastname: Sartori
authors.forename: Giovanni
authors.initials: G
authors.lastname: Masè
authors.forename: Marco
authors.initials: M
authors.lastname: Simonetto
authors.forename: Lucio
authors.initials: L
authors.lastname: Lazzarino de Lorenzo
authors.forename: Laura
authors.initials: L
authors.lastname: Cecotti
authors.forename: Loris
authors.initials: L
authors.lastname: Zanier
authors.forename: Federica
authors.initials: F
authors.lastname: Pisa
authors.forename: Fabio
authors.initials: F
authors.lastname: Barbone
mesh_terms: D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000453:epidemiology*; D005260:Female; D006801:Humans; D015994:Incidence; D007558:Italy / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011446:Prospective Studies; D012042:Registries; D012189:Retrospective Studies; D017678:Sex Distribution