Amyotrophic lateral sclerosis: a comparison of two staging systems in a population-based study

D. Ferraro; D. Consonni; N. Fini; A. Fasano; Cinzia Del Giovane; Emilia Romagna Registry for ALS (ERRALS) Group; J. Mandrioli; P. Nichelli; S. Biguzzi; Y. Handouk; E. Venturini; M. G. Passarin; C. Guidi; W. Neri; E. Sette; V. Tugnoli; M. R. Tola; E. Terlizzi; D. Guidetti; M. Currò Dossi; M. Pasquinelli; Jessica Andruccioli; A. Ravasio; M. Casmiro; F. Rasi; F. Salvi; I. Bartolomei; R. Michelucci; P. Avoni; S. de Pasqua; R. Liguori; R. Rizzi; Elena Canali; N. Marcello; A. Grassi; L. Delay; V. Pietrini; M. Aiello; D. Medici; E. Chierici; E. Montanari; M. Santangelo; S. Amidei; I. Casetta; E. Groppo; E. Granieri; P. De Massis; V. Mussuto; A. Borghi; A. Gabellini; T. Sacquegna; R. Rinaldi; F. Cirignotta; S. Ferro; R. D’Alessandro

doi:10.1111/ene.13053

Amyotrophic lateral sclerosis: a comparison of two staging systems in a population-based study

D. Ferraro, D. Consonni, N. Fini, A. Fasano, Cinzia Del Giovane, Emilia Romagna Registry for ALS (ERRALS) Group, J. Mandrioli, P. Nichelli, S. Biguzzi, Y. Handouk, E. Venturini, M. G. Passarin, C. Guidi, W. Neri, E. Sette, V. Tugnoli, M. R. Tola, E. Terlizzi, D. Guidetti, M. Currò DossiM. Pasquinelli, Jessica Andruccioli, A. Ravasio, M. Casmiro, F. Rasi, F. Salvi, I. Bartolomei, R. Michelucci, P. Avoni, S. de Pasqua, R. Liguori, R. Rizzi, Elena Canali, N. Marcello, A. Grassi, L. Delay, V. Pietrini, M. Aiello, D. Medici, E. Chierici, E. Montanari, M. Santangelo, S. Amidei, I. Casetta, E. Groppo, E. Granieri, P. De Massis, V. Mussuto, A. Borghi, A. Gabellini, T. Sacquegna, R. Rinaldi, F. Cirignotta, S. Ferro, R. D’Alessandro

Research output: Contribution to journal › Article › peer-review

Abstract

Background and purpose: To compare two recently developed staging systems for amyotrophic lateral sclerosis (ALS) [King's College and Milano-Torino staging (MITOS) systems] in an incident, population-based cohort of patients with ALS. Methods: Since 2009, a prospective registry has been recording all incident cases of ALS in the Emilia Romagna region in Italy. For each patient, detailed clinical information, including the ALS functional rating scale score, is collected at each follow-up. Results: Our study on 545 incident cases confirmed that King's College stages occurred at predictable times and were quite evenly spaced out throughout the disease course (occurring at approximately 40%, 60% and 80% of the disease course), whereas MITOS stages were mostly skewed towards later phases of the disease. In the King's College system there was a decrease in survival and an increase in deaths with escalating stages, whereas in the MITOS system survival curves pertaining to intermediate stages overlapped and the number of deaths was fairly homogenous throughout most stages. Conclusions: The King's College staging system had a higher homogeneity (i.e. smaller differences in survival among patients in the same stage) and a higher discriminatory ability (i.e. greater differences in survival among patients in different stages), being more suitable for individualized prognosis and for measuring efficacy of therapeutic interventions. © 2016 EAN

Original language	English
Pages (from-to)	1426-1432
Number of pages	7
Journal	European Journal of Neurology
Volume	23
Issue number	9
DOIs	https://doi.org/10.1111/ene.13053
Publication status	Published - 2016

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10.1111/ene.13053

https://www.scopus.com/inward/record.uri?eid=2-s2.0-84982166153&doi=10.1111%2fene.13053&partnerID=40&md5=1466494e1e64e8eaa3b163b18ebd8707

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Ferraro, D., Consonni, D., Fini, N., Fasano, A., Del Giovane, C., Group, E. R. R. F. ALS., Mandrioli, J., Nichelli, P., Biguzzi, S., Handouk, Y., Venturini, E., Passarin, M. G., Guidi, C., Neri, W., Sette, E., Tugnoli, V., Tola, M. R., Terlizzi, E., Guidetti, D., ... D’Alessandro, R. (2016). Amyotrophic lateral sclerosis: a comparison of two staging systems in a population-based study. European Journal of Neurology, 23(9), 1426-1432. https://doi.org/10.1111/ene.13053

Ferraro, D, Consonni, D, Fini, N, Fasano, A, Del Giovane, C, Group, ERRFALS, Mandrioli, J, Nichelli, P, Biguzzi, S, Handouk, Y, Venturini, E, Passarin, MG, Guidi, C, Neri, W, Sette, E, Tugnoli, V, Tola, MR, Terlizzi, E, Guidetti, D, Currò Dossi, M, Pasquinelli, M, Andruccioli, J, Ravasio, A, Casmiro, M, Rasi, F, Salvi, F, Bartolomei, I, Michelucci, R , Avoni, P, de Pasqua, S, Liguori, R, Rizzi, R, Canali, E, Marcello, N, Grassi, A, Delay, L, Pietrini, V, Aiello, M, Medici, D, Chierici, E, Montanari, E, Santangelo, M, Amidei, S, Casetta, I, Groppo, E, Granieri, E, De Massis, P, Mussuto, V, Borghi, A, Gabellini, A, Sacquegna, T, Rinaldi, R, Cirignotta, F, Ferro, S & D’Alessandro, R 2016, 'Amyotrophic lateral sclerosis: a comparison of two staging systems in a population-based study', European Journal of Neurology, vol. 23, no. 9, pp. 1426-1432. https://doi.org/10.1111/ene.13053

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title = "Amyotrophic lateral sclerosis: a comparison of two staging systems in a population-based study",

abstract = "Background and purpose: To compare two recently developed staging systems for amyotrophic lateral sclerosis (ALS) [King's College and Milano-Torino staging (MITOS) systems] in an incident, population-based cohort of patients with ALS. Methods: Since 2009, a prospective registry has been recording all incident cases of ALS in the Emilia Romagna region in Italy. For each patient, detailed clinical information, including the ALS functional rating scale score, is collected at each follow-up. Results: Our study on 545 incident cases confirmed that King's College stages occurred at predictable times and were quite evenly spaced out throughout the disease course (occurring at approximately 40%, 60% and 80% of the disease course), whereas MITOS stages were mostly skewed towards later phases of the disease. In the King's College system there was a decrease in survival and an increase in deaths with escalating stages, whereas in the MITOS system survival curves pertaining to intermediate stages overlapped and the number of deaths was fairly homogenous throughout most stages. Conclusions: The King's College staging system had a higher homogeneity (i.e. smaller differences in survival among patients in the same stage) and a higher discriminatory ability (i.e. greater differences in survival among patients in different stages), being more suitable for individualized prognosis and for measuring efficacy of therapeutic interventions. {\textcopyright} 2016 EAN",

keywords = "amyotrophic lateral sclerosis, population-based study, prognosis, staging",

author = "D. Ferraro and D. Consonni and N. Fini and A. Fasano and {Del Giovane}, Cinzia and Group, {Emilia Romagna Registry for ALS (ERRALS)} and J. Mandrioli and P. Nichelli and S. Biguzzi and Y. Handouk and E. Venturini and Passarin, {M. G.} and C. Guidi and W. Neri and E. Sette and V. Tugnoli and Tola, {M. R.} and E. Terlizzi and D. Guidetti and {Curr{\`o} Dossi}, M. and M. Pasquinelli and Jessica Andruccioli and A. Ravasio and M. Casmiro and F. Rasi and F. Salvi and I. Bartolomei and R. Michelucci and P. Avoni and {de Pasqua}, S. and R. Liguori and R. Rizzi and Elena Canali and N. Marcello and A. Grassi and L. Delay and V. Pietrini and M. Aiello and D. Medici and E. Chierici and E. Montanari and M. Santangelo and S. Amidei and I. Casetta and E. Groppo and E. Granieri and {De Massis}, P. and V. Mussuto and A. Borghi and A. Gabellini and T. Sacquegna and R. Rinaldi and F. Cirignotta and S. Ferro and R. D{\textquoteright}Alessandro",

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doi = "10.1111/ene.13053",

language = "English",

volume = "23",

pages = "1426--1432",

journal = "European Journal of Neurology",

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TY - JOUR

T1 - Amyotrophic lateral sclerosis: a comparison of two staging systems in a population-based study

AU - Ferraro, D.

AU - Consonni, D.

AU - Fini, N.

AU - Fasano, A.

AU - Del Giovane, Cinzia

AU - Group, Emilia Romagna Registry for ALS (ERRALS)

AU - Mandrioli, J.

AU - Nichelli, P.

AU - Biguzzi, S.

AU - Handouk, Y.

AU - Venturini, E.

AU - Passarin, M. G.

AU - Guidi, C.

AU - Neri, W.

AU - Sette, E.

AU - Tugnoli, V.

AU - Tola, M. R.

AU - Terlizzi, E.

AU - Guidetti, D.

AU - Currò Dossi, M.

AU - Pasquinelli, M.

AU - Andruccioli, Jessica

AU - Ravasio, A.

AU - Casmiro, M.

AU - Rasi, F.

AU - Salvi, F.

AU - Bartolomei, I.

AU - Michelucci, R.

AU - Avoni, P.

AU - de Pasqua, S.

AU - Liguori, R.

AU - Rizzi, R.

AU - Canali, Elena

AU - Marcello, N.

AU - Grassi, A.

AU - Delay, L.

AU - Pietrini, V.

AU - Aiello, M.

AU - Medici, D.

AU - Chierici, E.

AU - Montanari, E.

AU - Santangelo, M.

AU - Amidei, S.

AU - Casetta, I.

AU - Groppo, E.

AU - Granieri, E.

AU - De Massis, P.

AU - Mussuto, V.

AU - Borghi, A.

AU - Gabellini, A.

AU - Sacquegna, T.

AU - Rinaldi, R.

AU - Cirignotta, F.

AU - Ferro, S.

AU - D’Alessandro, R.

N1 - Export Date: 29 March 2017

PY - 2016

Y1 - 2016

N2 - Background and purpose: To compare two recently developed staging systems for amyotrophic lateral sclerosis (ALS) [King's College and Milano-Torino staging (MITOS) systems] in an incident, population-based cohort of patients with ALS. Methods: Since 2009, a prospective registry has been recording all incident cases of ALS in the Emilia Romagna region in Italy. For each patient, detailed clinical information, including the ALS functional rating scale score, is collected at each follow-up. Results: Our study on 545 incident cases confirmed that King's College stages occurred at predictable times and were quite evenly spaced out throughout the disease course (occurring at approximately 40%, 60% and 80% of the disease course), whereas MITOS stages were mostly skewed towards later phases of the disease. In the King's College system there was a decrease in survival and an increase in deaths with escalating stages, whereas in the MITOS system survival curves pertaining to intermediate stages overlapped and the number of deaths was fairly homogenous throughout most stages. Conclusions: The King's College staging system had a higher homogeneity (i.e. smaller differences in survival among patients in the same stage) and a higher discriminatory ability (i.e. greater differences in survival among patients in different stages), being more suitable for individualized prognosis and for measuring efficacy of therapeutic interventions. © 2016 EAN

AB - Background and purpose: To compare two recently developed staging systems for amyotrophic lateral sclerosis (ALS) [King's College and Milano-Torino staging (MITOS) systems] in an incident, population-based cohort of patients with ALS. Methods: Since 2009, a prospective registry has been recording all incident cases of ALS in the Emilia Romagna region in Italy. For each patient, detailed clinical information, including the ALS functional rating scale score, is collected at each follow-up. Results: Our study on 545 incident cases confirmed that King's College stages occurred at predictable times and were quite evenly spaced out throughout the disease course (occurring at approximately 40%, 60% and 80% of the disease course), whereas MITOS stages were mostly skewed towards later phases of the disease. In the King's College system there was a decrease in survival and an increase in deaths with escalating stages, whereas in the MITOS system survival curves pertaining to intermediate stages overlapped and the number of deaths was fairly homogenous throughout most stages. Conclusions: The King's College staging system had a higher homogeneity (i.e. smaller differences in survival among patients in the same stage) and a higher discriminatory ability (i.e. greater differences in survival among patients in different stages), being more suitable for individualized prognosis and for measuring efficacy of therapeutic interventions. © 2016 EAN

KW - amyotrophic lateral sclerosis

KW - population-based study

KW - prognosis

KW - staging

U2 - 10.1111/ene.13053

DO - 10.1111/ene.13053

M3 - Article

VL - 23

SP - 1426

EP - 1432

JO - European Journal of Neurology

JF - European Journal of Neurology

SN - 1351-5101

IS - 9

ER -