Riluzole and prognostic factors in amyotrophic lateral sclerosis long-term and short-term survival: a population-based study of 1149 cases in Taiwan.

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.

• Authors: Charles Tzu-Chi Lee, Yi-Wen Chiu, Kai-Chen Wang, Chi-Shin Hwang, Kuan-Hsiang Lin, I-Ta Lee, Ching-Piao Tsai
• Journal: Journal of epidemiology (J Epidemiol) Vol. 23 Issue 1 Pg. 35-40 ( 2013) ISSN: 1349-9092 [Electronic] Japan
• PMID: 23117224 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Neuroprotective Agents
• Riluzole

Topics

• Adult
• Age Distribution
• Aged
• Amyotrophic Lateral Sclerosis (mortality, therapy)
• Databases, Factual
• Female
• Follow-Up Studies
• Gastrostomy
• Humans
• Male
• Middle Aged
• Neuroprotective Agents (therapeutic use)
• Positive-Pressure Respiration
• Prognosis
• Retrospective Studies
• Riluzole (therapeutic use)
• Risk Factors
• Sex Distribution
• Socioeconomic Factors
• Survival Analysis
• Taiwan (epidemiology)
• Time Factors
• Tracheotomy