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.

The Use of Antidepressant Medication before and after the Diagnosis of Amyotrophic Lateral Sclerosis: A Population-Based Cohort Study

“…The whole spectrum of disease phenotype, severity and sociodemographic patient characteristics are included. The ascertainment of cases is complete and incidence rates are within the range of registries [26]. …”

“…The ascertainment of cases is described in detail elsewhere [26]. Multiple sources, including hospital services databases, electronic medical records and archives of all Neurology departments, were used to detect patients diagnosed during inpatient or outpatient medical encounters.…”

Crowdsourced analysis of clinical trial data to predict amyotrophic lateral sclerosis progression

“…Symptoms include muscle weakness, paralysis and eventually death, usually within 3 to 5 years from disease onset. Approximately 1 out of 400 people will be diagnosed with, and die of ALS 1,2 , and modern medicine is faced with a major challenge in finding an effective treatment 2,3 . Riluzole (Rilutek) is the only approved medication for ALS, and has a limited effect on survival 4 .…”

Amyotrophic Lateral Sclerosis Descriptive Studies: Not Only Rates and Frequencies

“…In this issue of Neuroepidemiology, Drigo et al [1] assess the incidence and prevalence of amyotrophic lateral sclerosis (ALS) in Friuli Venezia Giulia, a north-eastern region in Italy, with a population of about 1.2 million, bordering Slovenia to the east and Austria to the north. This is an aging population with about 22% of subjects over 65 years old.…”

“…Unfortunately, although not insurmountable, the obstacles for such studies both in terms of implementation and interpretation will be considerable. Many hints from the study of Drigo et al [1] show that ALS descriptive studies are not only about rates and frequencies.…”