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.
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References
Beghi E, Chiò A, Couratier P et al (2011) The epidemiology and treatment of ALS: focus on the heterogeneity of the disease and critical appraisal of therapeutic trials. Amyotroph Lateral Scler 12:1–10. doi:10.3109/17482968.2010.502940
Chio A, Logroscino G, Hardiman O et al (2009) Prognostic factors in ALS: a critical review. Amyotroph Lateral Scler 10:310–323. doi:10.3109/17482960802566824
Creemers H, Grupstra H, Nollet F, van den Berg LHBA (2015) Prognostic factors for the course of functional status of patients with ALS: a systematic review. J Neurol 262:1407–1423. doi:10.1007/s00415-014-7564-8
Miller RG, Mitchell JD, Moore DH (2012) Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND)(Review). Cochrane Database Syst Rev 3:1–34. doi:10.1002/14651858.CD001447
Katzberg H, Benatar M (2011) Enteral tube feeding for amyotrophic lateral sclerosis/motor neuron disease (Review). Cochrane Database Syst Rev 1:CD004030. doi:10.1002/14651858.CD004030.pub3
Bourke SC, Tomlinson M, Williams TL, Bullock RE, Shaw PJGG (2006) Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol 5:140–147
Radunovic A, Annane D, Jewitt K MN (2009) Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst Rev 4:CD004427. doi:10.1002/14651858.CD004427
Fini N, Georgoulopoulou E, Vinceti M, Monelli M, Pinelli G, Vacondio P, Giovannini M, Dallari R, Marudi AMJ (2014) Noninvasive and invasive ventilation and enteral nutrition for ALS in Italy. Muscle Nerve 50:508–516. doi:10.1002/mus.24187
Ng L, Khan F MS (2009) Multidisciplinary care for adults with amyotrophic lateral sclerosis or motor neuron disease. Cochrane Database Syst Rev 4:CD007425. doi:10.1002/14651858.CD007425.pub2
Rooney J, Byrne S, Heverin M, Tobin K, Dick A, Donaghy CHO (2015) A multidisciplinary clinic approach improves survival in ALS: a comparative study of ALS in Ireland and Northern Ireland. J Neurol Neurosurg Psychiatry 86:406–501. doi:10.1136/jnnp-2014-309601
Brooks BR, Miller RG, Swash M, Munsat TL (2000) El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 1:293–299. doi:10.1080/146608200300079536
Chiò A, Calvo A, Moglia C, Mazzini L, MGP study group (2011) Phenotypic heterogeneity of amyotrophic lateral sclerosis: a population based study. J Neurol Neurosurg Psychiatry 82:740–746. doi:10.1136/jnnp.2010.235952
Mandrioli J, Biguzzi S, Guidi C, et al. (2014) Epidemiology of amyotrophic lateral sclerosis in Emilia Romagna Region (Italy): A population based study. 8421:262–268. doi:10.3109/21678421.2013.865752
Andersen PM, Abrahams S, Borasio GD et al (2012) EFNS guidelines on the Clinical Management of Amyotrophic Lateral Sclerosis (MALS)–revised report of an EFNS task force. Eur J Neurol 19:360–375. doi:10.1111/j.1468-1331.2011.03501.x
Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, Kalra S, Katz JS, Mitsumoto H, Rosenfeld J, Shoesmith C, Strong MJ WSQSS of the AA of N (2009) Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): report of the Quality Standards Subcommittee of the American. Neurology 73:1227–1233
Logroscino G, Traynor B, Hardiman O et al (2008) Descriptive epidemiology of amyotrophic lateral sclerosis: new evidence and unsolved issues. J Neurol Neurosurg Psychiatry 79:6–11. doi:10.1136/jnnp.2006.104828
Pugliatti M, Parish LD, Cossu P et al (2013) Amyotrophic lateral sclerosis in Sardinia, insular Italy, 1995–2009. J Neurol 260:572–579. doi:10.1007/s00415-012-6681-5
Chiò A, Mora G, Calvo A et al (2009) Epidemiology of ALS in Italy: a 10-year prospective population-based study. Neurology 72:725–731. doi:10.1212/01.wnl.0000343008.26874.d1
Bandettini di Poggio M, Sormani MP, Truffelli R et al (2012) Clinical epidemiology of ALS in Liguria, Italy. Amyotroph Lateral Scler. doi:10.3109/17482968.2012.729062
Georgoulopoulou E, Vinceti M, Bonvicini F et al (2011) Changing incidence and subtypes of ALS in Modena, Italy: a 10-years prospective study. Amyotroph Lateral Scler 12:451–457. doi:10.3109/17482968.2011.593037
Chiò A, Calvo A, Ghiglione P et al (2010) Tracheostomy in amyotrophic lateral sclerosis: a 10-year population-based study in Italy. J Neurol Neurosurg Psychiatry 81:1141–1143. doi:10.1136/jnnp.2009.175984
Sabatelli M, Madia F, Conte A, Luigetti M, Zollino M, Mancuso I, Lo Monaco M, Lippi GTP (2008) Natural history of young-adult amyotrophic lateral sclerosis. Neurology 71:876–881. doi:10.1212/01.wnl.0000312378.94737.45
Chen L, Zhang B, Chen R, Tang L, Liu R, Yang Y, Yang Y, Liu X, Ye S, Zhan SFD (2015) Natural history and clinical features of sporadic amyotrophic lateral sclerosis in China. J Neurol Neurosurg Psychiatry 86:1075–1081. doi:10.1136/jnnp-2015-310471
Phukan J, Elamin M, Bede P, Jordan N, Gallagher L, Byrne S, Lynch C, Pender N, Hardiman O (2012) The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study. J Neurol Neurosurg Psychiatry 83:102–108. doi:10.1136/jnnp-2011-300188
Montuschi A, Iazzolino B, Calvo A, Moglia C, Lopiano L, Restagno G, Brunetti M, Ossola I, Lo Presti A, Cammarosano S, Canosa A, Chiò A (2015) Cognitive correlates in amyotrophic lateral sclerosis: a population-based study in Italy. J Neurol Neurosurg Psychiatry 86:168–173. doi:10.1136/jnnp-2013-307223
Sabatelli M, Zollino M, Luigetti M, Grande AD, Lattante S, Marangi G, Monaco ML, Madia F, Meleo E, Bisogni G, Conte A (2011) Uncovering amyotrophic lateral sclerosis phenotypes: clinical features and long-term follow-up of upper motor neuron-dominant ALS. Amyotroph Lateral Scler 12:278–282. doi:10.3109/17482968.2011.580849
Wei Q, Chen X, Zheng Z et al (2015) The predictors of survival in Chinese Amyotrophic Lateral Sclerosis patients. Amyotroph Lateral Scler Front Degener 16:237–244. doi:10.3109/21678421.2014.993650
Traxinger K, Kelly C, Johnson BA, Lyles RHGJ (2013) Prognosis and epidemiology of amyotrophic lateral sclerosis: analysis of a clinic population, 1997–2011. Neurol Clin Pr 3:313–320
Moura MC, Novaes MR, Eduardo EJ, Zago YS, LA Freitas Rdel NC (2015) Prognostic Factors in Amyotrophic Lateral Sclerosis: a Population-Based Study. PLoS One 10:e0141500. doi:10.1371/journal.pone.0141500
Paganoni S, Deng J, Jaffa M et al (2011) Body mass index, not dyslipidemia, is an independent predictor of survival in amyotrophic lateral sclerosis. Muscle Nerve 44:20–24. doi:10.1002/mus.22114
Chiò A, Canosa A, Gallo S et al (2011) ALS clinical trials: do enrolled patients accurately represent the ALS population? Neurology 77:1432–1437. doi:10.1212/WNL.0b013e318232ab9b
Acknowledgments
The authors thank all the collaborators of the multidisciplinary centers for motor neuron disease involved in the study: Antonio Fasano, Laura Ferri (Modena), Giovanni Novi (Genova), Rosa Capozzo (Tricase), Cinzia Femiano, Mattia Siciliano (Naples), Giulia Bisogni (Rome); Andrea Lizio, Eleonora Maestri, Claudia Tarlarini (Nemo Milano), Claudia Morelli, Federico Verde, Stefano Messina (Istituto Auxologico, Milano), Antonio Onniboni (Fondazione S. Maugeri, Mistretta), Riccardo Sideri (Fondazione S. Maugeri, Milano). Jessica Mandrioli has received research support from Regione EmiIia Romagna (Programma di ricerca Regione-Università 2010–2012, area 2, Ricerca per il Governo Clinico; Emilia Romagna Registry for Amyotrophic Lateral Sclerosis 2009–2015). Outside this work, Francesca Trojsi perceived Grants from Novartis and Maria Rosaria Monsurrò grants from Italfarmaco and Italian Association for Amyotrophic Lateral Sclerosis (AISLA). Amelia Conte and Mario Sabatelli thank I.CO.M.M. onlus, association for Amyotrophic Lateral Sclerosis research. Nicola Ticozzi received research support from the Italian Ministry of Health (Grant GR-2011-02347820—IRisALS) and from the Associazione “Io Corro con Giovanni”.
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Calvo, A., Moglia, C., Lunetta, C. et al. Factors predicting survival in ALS: a multicenter Italian study. J Neurol 264, 54–63 (2017). https://doi.org/10.1007/s00415-016-8313-y
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DOI: https://doi.org/10.1007/s00415-016-8313-y