title: Factors predicting survival in ALS: a multicenter Italian study.
abstract: The aim of this multicenter, retrospective study is to investigate the role of clinical characteristics and therapeutic intervention on ALS prognosis. The study included patients diagnosed from January 1, 2009 to December 31, 2013 in 13 Italian referral centers for ALS located in 10 Italian regions. Caring neurologists collected a detailed phenotypic profile and follow-up data until death into an electronic database. One center collected also data from a population-based registry for ALS. 2648 incident cases were collected. The median survival time from onset to death/tracheostomy was 44 months (SE 1.18, CI 42-46). According to univariate analysis, factors related to survival from onset to death/tracheostomy were: age at onset, diagnostic delay, site of onset, phenotype, degree of certainty at diagnosis according to revised El Escorial criteria (R-EEC), presence/absence of dementia, BMI at diagnosis, patients' provenance. In the multivariate analysis, age at onset, diagnostic delay, phenotypes but not site of onset, presence/absence of dementia, BMI, riluzole use, R-EEC criteria were independent prognostic factors of survival in ALS. We compared patients from an ALS Registry with patients from tertiary centers; the latter ones were younger, less frequently bulbar, but more frequently familial and definite at diagnosis. Our large, multicenter study demonstrated the role of some clinical and demographic factors on ALS survival, and showed some interesting differences between referral centers' patients and the general ALS population. These results can be helpful for clinical practice, in clinical trial design and to validate new tools to predict disease progression.
authors: "Rita Levi Montalcini" Department of Neuroscience, ALS Center, University of Torino, Turin, Italy.
authors: Andrea
authors: A
authors: Calvo
authors: "Rita Levi Montalcini" Department of Neuroscience, ALS Center, University of Torino, Turin, Italy.
authors: Cristina
authors: C
authors: Moglia
authors: NEuroMuscular Omnicentre (NEMO), Serena Onlus Foundation, Milan, Italy.|NEMO Sud Clinical Center for Neuromuscular Diseases, Aurora Onlus Foundation, Messina, Italy.
authors: Christian
authors: C
authors: Lunetta
authors: Department of Neurorehabilitation ALS Center Scientific Institute of Milan, Salvatore Maugeri Foundation IRCCS, Milan, Italy.
authors: Kalliopi
authors: K
authors: Marinou
authors: Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Milan, Italy.|Department of Pathophysiology and Transplantation 'Dino Ferrari' Center, University of Milan, Milan, Italy.
authors: Nicola
authors: N
authors: Ticozzi
authors: Neurorehabilitation Unit/ALS Center, Salvatore Maugeri Foundation, IRCCS, Mistretta, Messina, Italy.
authors: Gianluca Drago
authors: GD
authors: Ferrante
authors: Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics Maternal and Child Health (DINOGMI), University of Genova, IRCCS AOU San Martino-IST, Genoa, Italy.
authors: Carlo
authors: C
authors: Scialo
authors: Department of Neurosciences Neuromuscular Center, University of Padova, Padua, Italy.
authors: Gianni
authors: G
authors: Sorarù
authors: Department of Medical, Surgical, Neurological, Metabolic and Aging Sciences, MRI Research Center SUN-FISM, Second University of Naples, Naples, Italy.
authors: Francesca
authors: F
authors: Trojsi
authors: NEuroMuscular Omnicentre (NEMO), Serena Onlus Foundation: Pol. A. Gemelli Foundation, Rome, Italy.
authors: Amelia
authors: A
authors: Conte
authors: Department of Neurology Division of Neuroscience Institute of Experimental Neurology, San Raffaele Scientific Institute, Milan, Italy.
authors: Yuri M
authors: YM
authors: Falzone
authors: Department of Clinical Research in Neurology, University of Bari "A. Moro", at Pia Fondazione "Card. G. Panico" Tricase, Lecce, Italy.
authors: Rosanna
authors: R
authors: Tortelli
authors: Department of Clinical and Experimental Medicine, University of Messina and Nemo Sud Clinical Center for Neuromuscular Diseases, Aurora Foundation, Messina, Italy.
authors: Massimo
authors: M
authors: Russo
authors: "Rita Levi Montalcini" Department of Neuroscience, ALS Center, University of Torino, Turin, Italy.
authors: Adriano
authors: A
authors: Chiò
authors: NEuroMuscular Omnicentre (NEMO), Serena Onlus Foundation, Milan, Italy.|Department Biomedical Sciences for Health, University of Milan, Milan, Italy.
authors: Valeria Ada
authors: VA
authors: Sansone
authors: Department of Neurorehabilitation ALS Center Scientific Institute of Milan, Salvatore Maugeri Foundation IRCCS, Milan, Italy.
authors: Gabriele
authors: G
authors: Mora
authors: Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Milan, Italy.|Department of Pathophysiology and Transplantation 'Dino Ferrari' Center, University of Milan, Milan, Italy.
authors: Vincenzo
authors: V
authors: Silani
authors: Neurorehabilitation Unit/ALS Center, Salvatore Maugeri Foundation, IRCCS, Mistretta, Messina, Italy.
authors: Paolo
authors: P
authors: Volanti
authors: Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics Maternal and Child Health (DINOGMI), University of Genova, IRCCS AOU San Martino-IST, Genoa, Italy.
authors: Claudia
authors: C
authors: Caponnetto
authors: Department of Neurosciences Neuromuscular Center, University of Padova, Padua, Italy.
authors: Giorgia
authors: G
authors: Querin
authors: Department of Medical, Surgical, Neurological, Metabolic and Aging Sciences, MRI Research Center SUN-FISM, Second University of Naples, Naples, Italy.
authors: Maria Rosaria
authors: MR
authors: Monsurrò
authors: NEuroMuscular Omnicentre (NEMO), Serena Onlus Foundation: Pol. A. Gemelli Foundation, Rome, Italy.|Institute of Neurology, Catholic University of Sacred Heart, Rome, Italy.
authors: Mario
authors: M
authors: Sabatelli
authors: Department of Neurology Division of Neuroscience Institute of Experimental Neurology, San Raffaele Scientific Institute, Milan, Italy.
authors: Nilo
authors: N
authors: Riva
authors: Department of Clinical Research in Neurology, University of Bari "A. Moro", at Pia Fondazione "Card. G. Panico" Tricase, Lecce, Italy.
authors: Giancarlo
authors: G
authors: Logroscino
authors: NEMO Sud Clinical Center for Neuromuscular Diseases, Aurora Onlus Foundation, Messina, Italy.|Department of Clinical and Experimental Medicine, University of Messina and Nemo Sud Clinical Center for Neuromuscular Diseases, Aurora Foundation, Messina, Italy.
authors: Sonia
authors: S
authors: Messina
authors: Department of Neuroscience, S. Agostino-Estense Hospital and University of Modena and Reggio Emilia, Via Pietro Giardini n. 1355, 41100, Modena, Italy.
authors: Nicola
authors: N
authors: Fini
authors: Department of Neuroscience, S. Agostino-Estense Hospital and University of Modena and Reggio Emilia, Via Pietro Giardini n. 1355, 41100, Modena, Italy. j.mandrioli@ausl.mo.it.
authors: Jessica
authors: http://orcid.org/0000-0002-9244-9782
authors: J
authors: Mandrioli
mesh_terms: D017668:Age of Onset; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis* / Q000453:epidemiology* / Q000235:genetics / Q000628:therapy; D015992:Body Mass Index; D057210:Delayed Diagnosis; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007558:Italy / Q000453:epidemiology; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D011379:Prognosis; D012189:Retrospective Studies; D062606:Tertiary Care Centers
keywords: ALS; Population-based registries; Prognostic factors; Referral centers; Survival