Riluzole and prognostic factors in amyotrophic lateral sclerosis long-term and short-term survival

A Population-based study of 1149 cases in Taiwan

Charles Tzu Chi Lee, Yi Wen Chiu, Kai Chen Wang, Chi Shin Hwang, Kuan Hsiang Lin, I. Ta Lee, Ching Piao Tsai

Research output: Contribution to journalArticle

42 Citations (Scopus)

Abstract

Background: Amyotrophic lateral sclerosis (ALS) is a rare disease in Taiwan; thus, estimation of ALS mortality is difficult. We evaluated factors associated with ALS survival in Taiwan. Methods: The study enrolled 1149 Taiwanese with a primary diagnosis of ALS during 1999-2008. Follow-up information was available for all patients; mean (SD) duration of follow-up was 2.91 (2.62) years. Medical interventions, including noninvasive positive pressure ventilation (NIPPV), tracheotomy, gastrostomy, and riluzole, were included in time-dependent survival analysis. Results: Of the 1149 ALS patients, 438 (38.12%) died during follow-up. Mortality in the first year was 16%, which was 13 times (95% CI 11.1-15.2) the age- and sex-standardized rate of the general population in Taiwan. The average annual crude mortality rate was 13.1% (person-years). Factors significantly associated with increased mortality were male sex, advanced age, rural residence, lower economic status, no tracheotomy, and no riluzole treatment. Significant predictors of long-term versus average survival were younger age at diagnosis, being a dependent or receiving social welfare, and NIPPV support. Significant predictors of short-term versus average survival were older age, being employed, no tracheotomy, and no riluzole use. Conclusions: The results support the use of riluzole to improve ALS survival. Patients who received riluzole and underwent tracheotomy had the best survival.

Original languageEnglish
Pages (from-to)35-40
Number of pages6
JournalJournal of Epidemiology
Volume23
Issue number1
DOIs
Publication statusPublished - Mar 12 2013
Externally publishedYes

Fingerprint

Riluzole
Amyotrophic Lateral Sclerosis
Taiwan
Tracheotomy
Survival
Population
Positive-Pressure Respiration
Mortality
Gastrostomy
Social Welfare
Survival Analysis
Rare Diseases
Economics

Keywords

  • Amyotrophic lateral sclerosis
  • Riluzole
  • Survival
  • Tracheotomy

ASJC Scopus subject areas

  • Epidemiology

Cite this

Riluzole and prognostic factors in amyotrophic lateral sclerosis long-term and short-term survival : A Population-based study of 1149 cases in Taiwan. / Lee, Charles Tzu Chi; Chiu, Yi Wen; Wang, Kai Chen; Hwang, Chi Shin; Lin, Kuan Hsiang; Lee, I. Ta; Tsai, Ching Piao.

In: Journal of Epidemiology, Vol. 23, No. 1, 12.03.2013, p. 35-40.

Research output: Contribution to journalArticle

Lee, Charles Tzu Chi ; Chiu, Yi Wen ; Wang, Kai Chen ; Hwang, Chi Shin ; Lin, Kuan Hsiang ; Lee, I. Ta ; Tsai, Ching Piao. / Riluzole and prognostic factors in amyotrophic lateral sclerosis long-term and short-term survival : A Population-based study of 1149 cases in Taiwan. In: Journal of Epidemiology. 2013 ; Vol. 23, No. 1. pp. 35-40.
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abstract = "Background: Amyotrophic lateral sclerosis (ALS) is a rare disease in Taiwan; thus, estimation of ALS mortality is difficult. We evaluated factors associated with ALS survival in Taiwan. Methods: The study enrolled 1149 Taiwanese with a primary diagnosis of ALS during 1999-2008. Follow-up information was available for all patients; mean (SD) duration of follow-up was 2.91 (2.62) years. Medical interventions, including noninvasive positive pressure ventilation (NIPPV), tracheotomy, gastrostomy, and riluzole, were included in time-dependent survival analysis. Results: Of the 1149 ALS patients, 438 (38.12{\%}) died during follow-up. Mortality in the first year was 16{\%}, which was 13 times (95{\%} CI 11.1-15.2) the age- and sex-standardized rate of the general population in Taiwan. The average annual crude mortality rate was 13.1{\%} (person-years). Factors significantly associated with increased mortality were male sex, advanced age, rural residence, lower economic status, no tracheotomy, and no riluzole treatment. Significant predictors of long-term versus average survival were younger age at diagnosis, being a dependent or receiving social welfare, and NIPPV support. Significant predictors of short-term versus average survival were older age, being employed, no tracheotomy, and no riluzole use. Conclusions: The results support the use of riluzole to improve ALS survival. Patients who received riluzole and underwent tracheotomy had the best survival.",
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