본문 바로가기
KERD
논문KCI 등재

Long-Term Outcome of Amyotrophic Lateral Sclerosis in Korean Subjects

2

조회

0

다운로드

2

인용

0

참고문헌

키워드

주제분류재활의학

초록

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.

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.

참고문헌 (19)

  1. Chancellor AM, "The prognosis of adult-onset motor neuron disease: a prospective study based on the Scottish Motor Neuron Disease Register", J Neurol, 240, pp.339~346 (1993)
  2. Norris F, "Onset, natural history and outcome in idiopathic adult motor neuron disease", J Neurol Sci, 118, pp.48~55 (1993)
  3. Ringel SP, "The natural history of amyotrophic lateral sclerosis", Neurology, 43, pp.1316~1322 (1993)
  4. Chancellor AM, "Adult onset motor neuron disease: worldwide mortality, incidence and distribution since 1950", J Neurol Neurosurg Psychiatry, 55, pp.1106~1115 (1992)
  5. Strand EA, "Management of oral-pharyngeal dysphagia symptoms in amyotrophic lateral sclerosis", Dysphagia, 11, pp.129~139 (1996)
  6. Qureshi M, "The natural history of ALS is changing: improved survival", Amyotroph Lateral Scler, 10, pp.324~331 (2009)
  7. Pupillo E, "Long-term survival in amyotrophic lateral sclerosis: a population-based study", Ann Neurol, 75, pp.287~297 (2014)
  8. Dreyer P, "Survival in ALS with home mechanical ventilation non-invasively and invasively: a 15-year cohort study in west Denmark", Amyotroph Lateral Scler Frontotemporal Degener, 15, pp.62~67 (2014)
  9. Mahajan KR, "Diaphragm pacing and noninvasive respiratory management of amyotrophic lateral sclerosis", motor neuron disease, 2012, pp.46, 851~855
  10. Onders RP, "Final analysis of the pilot trial of diaphragm pacing in amyotrophic lateral sclerosis with long-term followup:diaphragm pacing positively affects diaphragm respiration", Am J Surg, 207, pp.393~397 (2014)
  11. Lewis CM, "Therapeutic applications of mesenchymal stem cells for amyotrophic lateral sclerosis", Stem Cell Res Ther, 5, pp.32~ (2014)
  12. Pronto-Laborinho AC, "Roles of vascular endothelial growth factor in amyotrophic lateral sclerosis", Biomed Res Int, 2014, pp.947513~ (2014)
  13. Brooks BR, "El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis", Amyotroph Lateral Scler Other Motor Neuron Disord, 1, pp.293~299 (2000)
  14. Lee CT, "Riluzole and prognostic factors in amyotrophic lateral sclerosis long-term and short-term survival:a population-based study of 1149 cases in Taiwan", J Epidemiol, 23, pp.35~40 (2013)
  15. Jennum P, "Mortality, health, social and economic consequences of amyotrophic lateral sclerosis: a controlled national study", J Neurol, 260, pp.785~793 (2013)
  16. Zoccolella S, "Predictors of long survival in amyotrophic lateral sclerosis: a population-based study", J Neurol Sci, 268, pp.28~32 (2008)
  17. Spataro R, "Percutaneous endoscopic gastrostomy in amyotrophic lateral sclerosis: effect on survival", J Neurol Sci, 304, pp.44~48 (2011)
  18. Fasano A, "Percutaneous endoscopic gastrostomy, body weight loss and survival in amyotrophic lateral sclerosis:a population-based registry study", Amyotroph Lateral Scler Frontotemporal Degener, 18, pp.233~242 (2017)
  19. del Aguila MA, "Prognosis in amyotrophic lateral sclerosis: a population-based study", Neurology, 60, pp.813~819 (2003)

관련 논문AI추천

한국의 근위축측삭경화증: 임상 특징 및 예후 예측 인자김승현, 김현영, 박아람, 백원기대한신경과학회 · 대한신경과학회지2011 · 제29권 제1호Current Status of the Diagnosis and Management of Amyotrophic Lateral Sclerosis in Korea: A Multi-Center Cross-Sectional Study김병조, 김승현, 배종석, 백원기, 손은희, 조중양, 홍윤호대한신경과학회 · Journal of Clinical Neurology2012 · 제8권 제4호Clinical Course of Amyotrophic Lateral Sclerosis according to Initial Symptoms: An Analysis of 500 Cases강성웅, 이장우, 최원아연세대학교의과대학 · Yonsei Medical Journal2021 · 제62권 제4호Healthcare Utilization and Supportive Care Timing in South Korean People Living With Amyotrophic Lateral Sclerosis: A Single-Center Retrospective Study성정준, Belong Cho, Inyoung Hwang, Jeongmi Shin, Kyae Hyung Kim, Min Seol Jang, Min Sun Kim, Seok-Jin Choi, Shin Hye Yoo, Sun Young Lee대한신경과학회 · Journal of Clinical Neurology2024 · 제20권 제2호Homozygous SMN2 Deletion is a Major Risk Factor among Twenty-Five Korean Sporadic Amyotrophic Lateral Sclerosis Patients서경임, 이경아, 이준범, 최영철, 홍지만연세대학교의과대학 · Yonsei Medical Journal2012 · 제53권 제1호한국 근위축성측삭경화증 등록체계의 구축과 향후 전망김병조, 김종국, 배종석, 백원기, 서범천, 성정준, 손은희, 안석원, 조중양, 홍윤호대한임상신경생리학회 · Annals of Clinical Neurophysiology2011 · 제13권 제2호

이 간행물의 다른 논문 (23건)