Ann Rehabil Med.  2017 Dec;41(6):1055-1064. 10.5535/arm.2017.41.6.1055.

Long-Term Outcome of Amyotrophic Lateral Sclerosis in Korean Subjects

• Suh MR
• Choi WA
• Choi YC
• Lee JW
• Hong JH
• Park J
• Kang SW

• KMID: 2400291
• DOI: http://doi.org/10.5535/arm.2017.41.6.1055

Abstract

OBJECTIVE
To report the latest long-term outcome of amyotrophic lateral sclerosis (ALS) and to analyze the predictors of prognosis.
METHODS
Subjects who were diagnosed with ALS between January 2005 and December 2009 at a single institute were followed up until death or up to December 2014. Data regarding age, sex, date of onset, date of diagnosis, presence of bulbar symptoms on onset, date of initiation of non-invasive ventilation (NIV), and the date of tracheostomy were collected. Survival was assessed using Kaplan-Meier curves and multivariate analyses of the risk of death were performed using the Cox proportional hazards model.
RESULTS
Among 212 suspicious subjects, definite ALS was diagnosed in 182 subjects. The survival rate at 3 and 5 years from onset was 61.5% and 40.1%, respectively, and the survival rate at 3 and 5 years post-diagnosis was 49.5% and 24.2%, respectively. Further, 134 patients (134/182, 73.6%) were initiated on NIV, and among them, 90 patients (90/182, 49.5%) underwent tracheostomy. Male gender and onset age of ≥65 years were independent predictors of adverse survival.
CONCLUSION
The analysis of long term survival in ALS showed excellent outcomes considering the overall poor prognosis of this disease.

Keyword

Amyotrophic lateral sclerosis; Survival; Prognosis

MeSH Terms

Age of Onset
Amyotrophic Lateral Sclerosis*
Diagnosis
Humans
Male
Multivariate Analysis
Noninvasive Ventilation
Prognosis
Proportional Hazards Models
Survival Rate
Tracheostomy

Figure

• 

  Fig. 1 Study flow. ALS, amyotrophic lateral sclerosis; NIV, noninvasive ventilation.

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  Fig. 2 Kaplan-Meier survival estimate of patients with amyotrophic lateral sclerosis (n=182).

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  Fig. 3 Survival estimates according to different variables. According to (A) gender, (B) onset age, (C) onset symptom, and (D) time from onset to diagnosis. Patients with amyotrophic lateral sclerosis showed a lower cumulative survival probability among those who were males, whose onset age was more than 65 years, who presented with bulbar symptoms at the onset period, and in whom the time from symptom onset to diagnosis was shorter than 12 months.

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