Riluzole and Prognostic Factors in Amyotrophic Lateral Sclerosis Long-term and Short-term Survival: A Population-Based Study of 1149 Cases in Taiwan

Abstract

BackgroundAmyotrophic 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.MethodsThe 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.ResultsOf 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.ConclusionsThe results support the use of riluzole to improve ALS survival. Patients who received riluzole and underwent tracheotomy had the best survival.

Point-Of-Care Stem Cell Therapy (POCST): Multisite transplantation of autologous bone marrow-derived mononuclear cells in 85 patients with Amyotrophic Lateral Sclerosis improves survival

“…We decided to take point 0 as time of diagnosis even this could mislead the comparison to other studies, setting our patients appearing to have worse status in less time. Nevertheless, there is no study with better outcome compared to our data at 1, 5 or 10 years of follow-up (Table 1) [14,15,22,73,80].…”

“…The factors that are thought to contribute to a better survival are: limb-onset, youth, better motor function, better respiratory status, stable weight, longer interval between appearing of symptoms and diagnosis, inter alia [21]. The therapeutic interventions including Riluzole combined with other measurements including tracheotomy infer modestly on the survival [22]. The symptomatic interventions (percutaneous gastrostomy, mechanical ventilation, tracheostomy and comprehensive care) are the ones specially claimed to be responsible for prolonged survival rates [21,23,24], even they are not related to the disease process itself.…”

Epidemiological and clinical factors impact on the benefit of riluzole in the survival rates of patients with ALS

“…Clinical studies showed that survival time is shorter in more impaired patients 4,15 . Many studies showed that patients with ALS treated with riluzole (50 mg/twice a day) survive longer 6,15,23 . Lee et al 6 showed that the mortality rate among patients receiving riluzole was reduced by almost 66%.…”

“…Many studies showed that patients with ALS treated with riluzole (50 mg/twice a day) survive longer 6,15,23 . Lee et al 6 showed that the mortality rate among patients receiving riluzole was reduced by almost 66%. However, patients who started treatment with severe and generalized clinical impairment had less benefit from riluzole.…”

“…The patient may experience fatigue, gait instability, changes in speed or rhythm during speech, and a weak hand grip. Survival time after diagnosis is variable in the literature ( from eight to 66 months) 5,6,7,9,10,11,12,13,14,15,16,17,18,19,20,21,22 . In general, shorter intervals from symptom onset to diagnosis predict lower survival rates.…”