title: Late-onset patients with sporadic amyotrophic lateral sclerosis in Japan have a higher progression rate of ALSFRS-R at the time of diagnosis.
abstract: OBJECTIVE
  The population in Japan is aging at a faster rate than in other countries in the world. It is speculated that the number of patients with late-onset amyotrophic lateral sclerosis (ALS) will increase even more in the future. However, few studies have been undertaken on the characteristics of patients with late-onset ALS in Japan. This study sought to investigate the clinical features of patients with late-onset ALS compared with those with early-onset ALS using the progression rate (ΔFS).
  
  
  METHODS
  Forty-five patients with sporadic ALS were divided into 2 groups: 23 patients with early-onset of ALS (<65 years; early onset) and 22 patients with late-onset ALS (≥65 years; late onset). Every patient was followed up from the time of initial diagnosis to the primary endpoint (death or time culminating in death without tracheostomy or ventilation assistance including noninvasive positive pressure ventilation) or for at least 48 months after initial diagnosis.
  
  
  RESULTS
  ΔFS in the patient group with late onset was significantly higher than that of the group with early onset (p=0.010). Survival of patients with late onset was significantly decreased compared to that of patients with early onset (p=0.031).
  
  
  CONCLUSIONS
  Our finding suggested that patients with late-onset ALS showed more rapid disease progression than those with early-onset ALS using ΔFS.
authors.affiliation: Department of Neurology and Geriatrics, Graduate School of Medicine, Gifu University, Japan. yutanaka-gif@umin.net
authors.forename: Yuji
authors.initials: Y
authors.lastname: Tanaka
authors.forename: Nobuaki
authors.initials: N
authors.lastname: Yoshikura
authors.forename: Naoko
authors.initials: N
authors.lastname: Harada
authors.forename: Megumi
authors.initials: M
authors.lastname: Yamada
authors.forename: Akihiro
authors.initials: A
authors.lastname: Koumura
authors.forename: Takeo
authors.initials: T
authors.lastname: Sakurai
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Hayashi
authors.forename: Akio
authors.initials: A
authors.lastname: Kimura
authors.forename: Isao
authors.initials: I
authors.lastname: Hozumi
authors.forename: Takashi
authors.initials: T
authors.lastname: Inuzuka
mesh_terms: D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications / Q000453:epidemiology*; D018450:Disease Progression; D005500:Follow-Up Studies; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008875:Middle Aged; D011157:Population Dynamics; D016016:Proportional Hazards Models; D012131:Respiratory Insufficiency / Q000209:etiology / Q000628:therapy; D012720:Severity of Illness Index; D013030:Spain / Q000453:epidemiology