This study provides Class III evidence that (18)F-FDG-PET accurately distinguishes patients with ALS from normal controls (sensitivity 95.4%, specificity 82.5%).

; for the Piemonte and Valle d'Aosta Register for Amyotrophic Lateral Sclerosis (PARALS) IMPORTANCE There is an urgent need to identify reliable biomarkers of amyotrophic lateral sclerosis (ALS) progression for clinical practice and pharmacological trials. OBJECTIVES To correlate several hematological markers evaluated at diagnosis with ALS outcome in a population-based series of patients (discovery cohort) and replicate the findings in an independent validation cohort from an ALS tertiary center.

GARLIT Study Group collaborators are given in the Acknowledgments section.

IMPORTANCE This study reports the long-term epidemiologic trends of amyotrophic lateral sclerosis (ALS) based on a prospective register. OBJECTIVE To examine the 20-year epidemiologic trends of ALS in the Piemonte and Valle d'Aosta regions of Italy. DESIGN, SETTING, AND PARTICIPANTS The Piemonte and Valle d'Aosta Register for ALS (PARALS) is an epidemiologic prospective register that covers 2 Italian regions (population of 4 476 931 inhabitants according to the 2011 census) from January 1, 1995, through December 31, 2014. Case ascertainment is based on multiple sources (neurologic departments, hospital discharge archives, and mortality records). Incidence rates are age and sex standardized for the Italian population of the 2011 census. Age-period-cohort (APC) analysis was performed using a Poisson regression model. MAIN OUTCOMES AND MEASURES The primary study outcomes were long-term incidence and prevalence rates of ALS using a prospective design and their determinants. RESULTS During the study period, a total of 2702 patients (mean [SD] age at onset, 65.7 [11.1] years; 1246 [46.1%] female and 1456 [53.9%] male) received a diagnosis of ALS between 1995 and 2014, corresponding to a crude annual incidence rate of 3.03 per 100 000 population (95% CI, 2.85-3.23) and an adjusted incidence rate of 2.78 per 100 000 population (95% CI, 2.57-2.96). The age-adjusted incidence rate increased in the 2 decades of

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