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Amyotrophic lateral sclerosis: early predictors of prolonged survival

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Abstract

Objective

In order to define the predictors of prolonged survival available at the time of first examination we performed a historical cohort study of amyotrophis sclerosis (ALS) patients referred to our ALS Clinic over the last 20 years.

Methods

In a group of 1034 patients with the diagnosis of definite or probable ALS the effects of individual prognostic factors on tracheostomy-free survival were assessed with the Kaplan-Meier life-table method. The prognostic value of each factor was estimated using univariate and multivariate Cox proportional hazard analyses.

Results

The median survival time was 3.45 years, (95%CI 3.27–3.74). Both the univariate and multivariate Cox models indicated that younger age, limb site of onset, longer diagnostic delay, lower Appel ALS score (AALSS) at first examination, lower AALSS-rate of change between first symptom and first exam (preslope), and higher baseline forced vital capacity (FVC) were associated with longer survival. In addition, four factors: age, diagnostic delay, baseline FVC and AALSS preslope have been identified as independent predictors of survival in our patient population.

Conclusions

The identification of younger age, limb site of onset and longer diagnostic delay as predictors of prolonged survival in ALS clinic population supports the findings of several, earlier studies that were based on smaller groups of patients. More significantly, several additional variables assessed at the first examination predict longer survival: lower baseline AALSS, lower AALSS- preslope and higher baseline FVC. All of these parameters are of value in patient management and in clinical trial development.

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Acknowledgements

We thank the patients and the members of the Muscular Dystrophy Association ALS Clinic team at Methodist Neurological Institute and Baylor College of Medicine in Houston, Texas for their contributions to gathering the database information. This work was supported by the MDA and the Houston Endowment. Adam Czaplinski was the recipient of the Sheila Essey ALS Fellowship Award and was also supported by Swiss National Science Foundation and Fonds zur Foerderung des Akademischen Nachwuchses University of Basel, Switzerland. We thank Dipl.-Math. Andreas Schoetzau for his thoughtful statistical advice and comments. We are grateful to Joan Appel for her assistance in coordinating the data-base investigation and manuscript review.

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Authors and Affiliations

  1. Dept. of Neurology, Baylor College of Medicine, Houston, TX, USA

    Adam Czaplinski MD

  2. Dept. of Neurology, Methodist Neurological Institute, 6560 Fannin, Suite 902, Houston, TX, 77030, USA

    Albert A. Yen MD & Stanley H. Appel

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  1. Adam Czaplinski MD
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  2. Albert A. Yen MD
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  3. Stanley H. Appel
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Correspondence to Stanley H. Appel.

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Received in revised form: 02 March 2006

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Czaplinski, A., Yen, A.A. & Appel, S.H. Amyotrophic lateral sclerosis: early predictors of prolonged survival. J Neurol 253, 1428–1436 (2006). https://doi.org/10.1007/s00415-006-0226-8

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