title: Clinical epidemiology of amyotrophic lateral sclerosis in Liguria, Italy: An update of LIGALS register.
abstract: Our objectives were: (1) to assess amyotrophic lateral sclerosis (ALS) incidence and its trend over time in Liguria, an Italian north-western region, performing an analysis of data prospectively collected from 1 January 2009 to 31 December 2014; (2) to determine the mean and median survival in the 2009-2014 Ligurian ALS incident cases; and (3) to evaluate the presence of disease prognostic factors. The Liguria Register for ALS (LIGALS) is an ongoing, multicentre prospective register enrolling all ALS incident cases in Liguria. Cases were identified using several concurrent sources. ALS diagnosis was based on El Escorial revised criteria (EEC-R). Two hundred and ninety-eight patients were enrolled in this study. The mean annual crude incidence rate in the 2009-2014 period was 3.11/100,000 population (95% CI 2.77-3.49); the point prevalence at 31 December 2014 was 7.85/100,000 (95% CI 6.54-9.36) population. Survival analysis demonstrated a median survival from symptom onset of 37.0 months (95% CI 32.0-42.0). In conclusion, ALS crude incidence in Liguria is higher compared to other Italian regions. Clinical and epidemiological data are comparable with those of the Italian ALS population. Survival analysis showed that higher age at onset, bulbar onset, definite EEC-R diagnostic category and a shorter diagnostic delay are related with worse outcomes.
authors.affiliation: a Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI) , University of Genova, IRCCS AOU San Martino-IST , Genova.
authors.forename: Carlo
authors.initials: C
authors.lastname: Scialò
authors.affiliation: a Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI) , University of Genova, IRCCS AOU San Martino-IST , Genova.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Novi
authors.affiliation: a Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI) , University of Genova, IRCCS AOU San Martino-IST , Genova.
authors.forename: Monica
authors.initials: M
authors.lastname: Bandettini di Poggio
authors.affiliation: b ALS Centre 'Rita Levi Montalcini' Department of Neuroscience, University of Torino, Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI) , University of Genova, IRCCS AOU San Martino-IST , Genova.
authors.forename: Antonio
authors.initials: A
authors.lastname: Canosa
authors.affiliation: c Biostatistics Unit, Department of Health Sciences , University of Genova, IRCCS AOU San Martino-IST , Genova , and.
authors.forename: Maria Pia
authors.initials: MP
authors.lastname: Sormani
authors.affiliation: a Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI) , University of Genova, IRCCS AOU San Martino-IST , Genova.
authors.forename: Paola
authors.initials: P
authors.lastname: Mandich
authors.affiliation: a Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI) , University of Genova, IRCCS AOU San Martino-IST , Genova.
authors.forename: Paola
authors.initials: P
authors.lastname: Origone
authors.affiliation: d Fondazioni Serena ONLUS, 'La Colletta' Hospital , Service of Rehabilitation of Neuromuscular Diseases , Arenzano, Genova , Italy.
authors.forename: Romina
authors.initials: R
authors.lastname: Truffelli
authors.affiliation: a Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI) , University of Genova, IRCCS AOU San Martino-IST , Genova.
authors.forename: Giovanni Luigi
authors.initials: GL
authors.lastname: Mancardi
authors.affiliation: a Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI) , University of Genova, IRCCS AOU San Martino-IST , Genova.
authors.forename: Claudia
authors.initials: C
authors.lastname: Caponnetto
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis / Q000453:epidemiology* / Q000235:genetics / Q000401:mortality; D000704:Analysis of Variance; D000073885:C9orf72 Protein; D004268:DNA-Binding Proteins / Q000235:genetics; D005260:Female; D006801:Humans; D015994:Incidence; D007558:Italy / Q000453:epidemiology; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D009154:Mutation / Q000235:genetics; D015995:Prevalence; D011506:Proteins / Q000235:genetics; D012042:Registries; D012189:Retrospective Studies; D011795:Surveys and Questionnaires; D016019:Survival Analysis
chemical_list: D000073885:C9orf72 Protein; C568651:C9orf72 protein, human; D004268:DNA-Binding Proteins; D011506:Proteins; C000598624:TARDBP protein, human
keywords: Amyotrophic lateral sclerosis; epidemiology; incidence; motor neuron disease; survival