title: [Clinical characteristics of elderly Japanese patients with amyotrophic lateral sclerosis; with special reference to the development of respiratory failure].
abstract: OBJECTIVE
  To clarify the characteristics of elderly-onset amyotrophic lateral sclerosis (ALS).
  
  
  METHODS
  We analyzed the pattern of progression of clinical symptoms and respiratory dysfunction in 26 sporadic ALS patients (19 men, 7 women; mean age 73.2 +/- 6.0 years) with onset at age 65 years or older (E-ALS). We compared the results with those of 28 ALS patients (20 men, 8 women; 53.7 +/- 7.6 years) with younger onset ALS (Y-ALS).
  
  
  RESULTS
  Among E-ALS patients, the bulbar palsy type (BP) was the most common (11 patients, 42%) followed by the respiratory failure type (RF) (7 patients, 27%). In contrast, upper extremity type (UE) was the most common (14 patients, 50%) in Y-ALS patients. Mean vital capacity percentage (%VC) at the initial examination was 71.1 +/- 20.4% (vital capacity (VC): 2.12 +/- 0.85 L) in all E-ALS patients, 64.5 +/- 14.5% in BP, 58.1 +/- 5.1% in RF, 94.4 +/- 11.1% in lower extremity type (LE), and 73.9 +/- 30.2% in UE. In all Y-ALS patients, %VC was 90.1 +/- 14.0% (2.94 +/- 0.57 L). The initial % VC value in E-ALS was significantly lower than that in Y-ALS (p < 0.01). VC was lower in RF and BP among E-ALS patients. It was also lower in BP E-ALS patients than in BP Y-ALS patients. Mean period from initial symptom until first examination was significantly shorter in RF in both groups, followed by BP. Twenty-two patients with E-ALS and 26 with Y-ALS died from respiratory failure. Four patients with E-ALS and 2 with Y-ALS required a mechanical ventilator. The mean period until death or ventilation support was 20.9 +/- 10.4 months in E-ALS, and 38.8 +/- 21.1 months in Y-ALS. Significantly shorter survival was observed in E-ALS than Y-ALS (p<0.01). In E-ALS patients, the mean period until death or ventilation support was 21.4 +/- 9.1 months with BP, 10.3 +/- 7.6 months with RF, 29.8 +/- 4.0 months with LE, and 29.3 +/- 5.4 months with UE. This period was significantly shorter in RF patients in both groups, followed by BP patients (p< 0.05, 0.01). Time until death or ventilation support was significantly shorter in BP and UE patients with E-ALS than in those with Y-ALS (p< 0.05).
  
  
  CONCLUSIONS
  In regard to the progression of respiratory function deterioration, early % VC was lower in E-ALS than in Y-ALS patients, and the period until VC fell below 1 L was shorter. The period until death was particularly short in elderly BP and RF patients, suggesting the possibility that the duration until death from respiratory failure is shorter in E-ALS, because of a decrease in respiratory reverse capacity that accompanies age.
authors.affiliation: Division of General Medicine, Department of Internal Medicine, Aichi Medical University School of Medicine.
authors.forename: Shin-ichi
authors.initials: S
authors.lastname: Terao
authors.forename: Naofumi
authors.initials: N
authors.lastname: Miura
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Osano
authors.forename: Koshin
authors.initials: K
authors.lastname: Adachi
authors.forename: Gen
authors.initials: G
authors.lastname: Sobue
mesh_terms: D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D000375:Aging / Q000502:physiology; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications* / Q000401:mortality; D002423:Cause of Death; D018450:Disease Progression; D005260:Female; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D012121:Respiration, Artificial; D012131:Respiratory Insufficiency / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D013997:Time Factors; D014797:Vital Capacity