Objective: To describe the clinical features at first evaluation that best predict survival of the amyotrophic lateral sclerosis (ALS) population from the Salpêtrière Hospital between 1995 and 2009. Methods: Data are collected and entered into a clinical database from all patients seen at the Paris ALS Center. Variables analyzed were demographic and baseline information, strength testing (manual muscle testing; 1995-2009), the revised ALS Functional Rating Scale (ALSFRS-R; 2002-2009) and survival status. The χ2 test and ANOVA assessed differences in variables by region and across time period. Univariate and multivariate Cox proportional hazards models determined which variables best predicted survival. Flexible modeling of continuous predictors (splines) assessed trends in survival for different variables. Results: 3,885 patients with ALS were seen in 1995-2009, of whom 2,037 had ALSFRS-R scores. Age, weight, strength, and site of onset varied by region of residence. The proportion of patients living outside Paris, the time to first visit, patient age, and motor function differed across time periods. In Cox models, site of onset, time to first visit greater than 18 months, strength and the year of visit after 2006 predicted survival (all p values <0.0001). Compared to patients first seen between 1999 and 2002, the hazard ratio of death was 1.04 (95% CI = 0.95-1.14) for 2003-2006, and 0.76 (95% CI = 0.66-0.87) after 2006, while adjusting for other predictors of survival. The use of noninvasive ventilation increased during 2004-2008 from 16 to 51% of patients. Conclusions: Older age, bulbar onset, shorter delay to first visit and poor motor function at first visit predicted shorter survival rates in this large center-based sample from France, showing marked consistency across time and region of residence. Survival improved after 2006, concurrent with increasing rates of noninvasive ventilation use. Clinicopathologic correlation could better define subgroups, but identification of etiologies may be needed to elucidate individual forms of ALS with unique survival patterns.

1.
Logroscino G, Traynor BJ, Hardiman O, et al: Descriptive epidemiology of amyotrophic lateral sclerosis: new evidence and unsolved issues. J Neurol Neurosurg Psychiatry 2008;79:6-11.
2.
Louwerse ES, Visser CE, Bossuyt PM, Weverling GJ: Amyotrophic lateral sclerosis: mortality risk during the course of the disease and prognostic factors. The Netherlands ALS Consortium. J Neurol Sci 1997;152 (suppl 1):S10-S17.
3.
del Aguila MA, Longstreth WT Jr, McGuire V, Koepsell TD, van BG: Prognosis in amyotrophic lateral sclerosis: a population-based study. Neurology 2003;60:813-819.
4.
Testa D, Lovati R, Ferrarini M, Salmoiraghi F, Filippini G: Survival of 793 patients with amyotrophic lateral sclerosis diagnosed over a 28-year period. Amyotroph Lateral Scler Other Motor Neuron Disord 2004;5:208-212.
5.
Millul A, Beghi E, Logroscino G, Micheli A, Vitelli E, Zardi A: Survival of patients with amyotrophic lateral sclerosis in a population-based registry. Neuroepidemiology 2005;25:114-119.
6.
Czaplinski A, Yen AA, Appel SH: Amyotrophic lateral sclerosis: early predictors of prolonged survival. J Neurol 2006;253:1428-1436.
7.
Kollewe K, Mauss U, Krampfl K, Petri S, Dengler R, Mohammadi B: ALSFRS-R score and its ratio: a useful predictor for ALS-progression. J Neurol Sci 200815;275:69-73.
8.
Zoccolella S, Beghi E, Palagano G, et al: Predictors of long survival in amyotrophic lateral sclerosis: a population-based study. J Neurol Sci 2008;268:28-32.
9.
Kaufmann P, Levy G, Thompson JL, et al: The ALSFRSr predicts survival time in an ALS clinic population. Neurology 2005;64:38-43.
10.
Chio A, Mora G, Leone M, et al: Early symptom progression rate is related to ALS outcome: a prospective population-based study. Neurology 2002;59:99-103.
11.
Paillisse C, Lacomblez L, Dib M, Bensimon G, Garcia-Acosta S, Meininger V: Prognostic factors for survival in amyotrophic lateral sclerosis patients treated with riluzole. Amyotroph Lateral Scler Other Motor Neuron Disord 2005;6:37-44.
12.
Desport JC, Preux PM, Truong TC, Vallat JM, Sautereau D, Couratier P: Nutritional status is a prognostic factor for survival in ALS patients. Neurology 1999;53:1059-1063.
13.
Stambler N, Charatan M, Cedarbaum JM: Prognostic indicators of survival in ALS. ALS CNTF Treatment Study Group. Neurology 1998;50:66-72.
14.
Lopez-Vega JM, Calleja J, Combarros O, Polo JM, Berciano J: Motor neuron disease in Cantabria. Acta Neurol Scand 1988;77:1-5.
15.
Chancellor AM, Slattery JM, Fraser H, Swingler RJ, Holloway SM, Warlow CP: The prognosis of adult-onset motor neuron disease: a prospective study based on the Scottish Motor Neuron Disease Register. J Neurol 1993;240:339-346.
16.
Brooks BR, Miller RG, Swash M, Munsat TL: El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 2000;1:293-299.
17.
Great Lakes ALS Study Group: A comparison of muscle strength testing techniques in amyotrophic lateral sclerosis. Neurology 2003;61:1503-1507.
18.
Cedarbaum JM, Stambler N, Malta E, et al: The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III). J Neurol Sci 1999;169:13-21.
19.
Thiebaut AC, Benichou J: Choice of time-scale in Cox's model analysis of epidemiologic cohort data: a simulation study. Stat Med 2004;23:3803-3820.
20.
Greenland S: Dose-response and trend analysis in epidemiology: alternatives to categorical analysis. Epidemiology 1995;6:356-365.
21.
Royston P, Sauerbrei W: Multivariable modeling with cubic regression splines: a principled approach. Stata J 2007;7:45-70.
22.
Gordon PH, Salachas F, Bruneteau G, et al: Improving survival in a large French ALS center cohort. J Neurol 2012, E-pub ahead of print.
23.
Preux PM, Couratier P, Boutros-Toni F, et al: Survival prediction in sporadic amyotrophic lateral sclerosis. Age and clinical form at onset are independent risk factors. Neuroepidemiology 1996;15:153-160.
24.
Zoccolella S, Beghi E, Palagano G, et al: Analysis of survival and prognostic factors in amyotrophic lateral sclerosis: a population based study. J Neurol Neurosurg Psychiatry 2008;79:33-37.
25.
Tysnes OB, Vollset SE, Aarli JA: Epidemiology of amyotrophic lateral sclerosis in Hordaland county, western Norway. Acta Neurol Scand 1991;83:280-285.
26.
Gordon PH, Delgadillo D, Piquard A, et al: The range and clinical impact of cognitive impairment in French patients with ALS: a cross-sectional study of neuropsychological test performance. Amyotroph Lateral Scler 2011;12:372-378.
27.
Haverkamp LJ, Appel V, Appel SH: Natural history of amyotrophic lateral sclerosis in a database population. Validation of a scoring system and a model for survival prediction. Brain 1995;118:707-719.
28.
Thijs V, Peeters E, Theys P, Matthijs G, Robberecht W: Demographic characteristics and prognosis in a Flemish amyotrophic lateral sclerosis population. Acta Neurol Belg 2000;100:84-90.
29.
Beghi E, Millul A, Logroscino G, Vitelli E, Micheli A: Outcome measures and prognostic indicators in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler 2008;9:163-167.
30.
Ganesalingam J, Stahl D, Wijesekera L, et al: Latent cluster analysis of ALS phenotypes identifies prognostically differing groups. PLoS One 2009;4:e7107.
31.
Czaplinski A, Yen AA, Appel SH: Forced vital capacity (FVC) as an indicator of survival and disease progression in an ALS clinic population. J Neurol Neurosurg Psychiatry 2006;77:390-392.
32.
McDonald ER, Wiedenfeld SA, Hillel A, Carpenter CL, Walter RA: Survival in amyotrophic lateral sclerosis. The role of psychological factors. Arch Neurol 1994;51:17-23.
33.
Olney RK, Murphy J, Forshew D, et al: The effects of executive and behavioral dysfunction on the course of ALS. Neurology 2005;65:1774-1777.
34.
Chio A, Logroscino G, Hardiman O, et al: Prognostic factors in ALS: a critical review. Amyotroph Lateral Scler 2009;10:310-323.
35.
Pinto AC, Evangelista T, Carvalho M, Alves MA, Sales Luis ML: Respiratory assistance with a non-invasive ventilator (Bipap) in MND/ALS patients: survival rates in a controlled trial. J Neurol Sci 1995;129(suppl):19-26.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.