Epidemiology of amyotrophic lateral sclerosis in Emilia Romagna Region (Italy): A population based study

Abstract

Our objective was to describe incidence and clinical features of ALS from a prospective population-based study in Emilia Romagna Region (ERR). From 2009 onwards, a prospective registry recorded all incident cases of ALS among residents in the ERR (population, 4.4 million inhabitants), involving 17 neurological departments. For each patient, detailed demographic and clinical information was collected by caring physicians. Results showed that from 1 January 2009 to 31 December 2011, 347 patients received a new diagnosis of ALS with a crude incidence rate of 2.63/100,000/year. There was micro-geographic heterogeneity throughout ERR, with higher incidence rates in the low density population (3.27/100,000) (p < 0.01). ALS patients have been more frequently employed in agriculture than the general ERR population (8.64% vs. 4.6%, p < 0.01). Clinical features were similar to those described in previous population based studies. In conclusion, we report incidence rates similar to those reported by European registries, reflecting good accuracy of our prospective study. We confirmed previous studies reporting higher incidence rates in rural areas and among agricultural workers. Although genetics has been gaining increasing importance in ALS aetiology, some epidemiological data are still unexplained. Identifying geographical areas or populations with high incidence rates can be a starting point for identifying environmental risk factors. Further studies having this specific aim can shed light on these topics.

Variation in worldwide incidence of amyotrophic lateral sclerosis: a meta-analysis

“…For example, studies based on the original EEDC included all categories of ALS (Definite, Probable, Possible, Suspected), n = 12, 3 , 39 , 40 , 42–45 , 48 , 51 , 53 , 66 or excluded cases who, during follow-up, remained suspected ALS ( n = 4) 4,37,38,54 or suspected/possible ALS ( n = 2) 6 , 59 . Conversely, most papers that used the revised version of EEDC included all types of ALS (Definite, Probable, Probable laboratory supported, Possible) ( n = 13), 8–14 , 46 , 47 , 50 , 57 , 64 and only one excluded cases with possible ALS during follow-up ( n = 1) 121 . However, when looking at standardized rates (i.e when controlling for age and sex distribution of the underlying population), ALS heterogeneity was not explained by the diagnosis ( P = 0.51).…”

Pesticide exposure assessed through agricultural crop proximity and risk of amyotrophic lateral sclerosis

“…Strengths of this study include inclusion of two different agricultural areas of Italy, characterized by different lifestyle and environmental characteristics, though with ALS rates comparable with those of other areas of Italy as well as US regions for which population-based data are available [38, 71–76]. The Northern Italy area we investigated experiences air pollution due to motorized traffic and other sources [77], while the area of Southern Italy included is generally characterized by a lower environmental pollution and by consumption of the well-known and healthy ‘Mediterranean diet’ [78].…”

“…In fact, at the last Agricultural General Census carried out in 2010, percentage of agricultural land use was 42.2, 44.1 and 44.6 in Parma, Reggio Emilia and Modena, respectively, while the corresponding percentage of employees in agriculture was 9.0, 9.7 and 11.6 [37]. We used several available administrative data sources to identify patients diagnosed in these provinces, known to have rather similar ALS incidence rates [38, 39]: the ALS Emilia-Romagna Registry (established in 2009), the hospital discharge records for the entire study period released by the Emilia-Romagna and Sicily regions, the drug prescription directories and the death certificate files. Only ‘definite’ and ‘probable’ ALS cases, as defined by using El Escorial revised criteria [40], were included in the study.…”

Heterogeneity in ALSFRS-R decline and survival: a population-based study in Italy

“…Details about ERRALS have already been described [9]. Briefly, caring physicians collected a detailed phenotypic profile of each ALS patient, including the following data: age at onset and diagnosis, gender, type of onset, site and time of onset, affected regions, upper and lower motor neuron signs, El Escorial Revised classification, clinical phenotype (classic ALS, bulbar ALS, upper motor neuron predominant ALS, flail, respiratory ALS) [10], presence of dementia or extrapyramidal signs, family history, diagnostic tests (electromyography, magnetic resonance imaging, etc.…”

“…From 1.1.2009 onwards, a prospective registry (Emilia Romagna Registry for ALS-ERRALS) has been collecting all incident ALS cases among residents in ERR, diagnosed according to El Escorial Revised Diagnostic Criteria [9].…”