2007 Volume 55 Issue 4 Pages 181-190
The clinical status and daily-life activity of 136 patients with amyotrophic lateral sclerosis (ALS) living in Nagano prefecture were evaluated. The mean age of the patients was 67.4 ± 10.4 years, and the mean age at onset was 61.0 ± 12.3 years. Thirty-nine patients (29.1%) developed the disease after 70 years of age. The mean duration of disease was 6.5 years (maximum 41 years), and 26 patients showed a duration of disease of over 10 years. Sixteen of these patients did not need artificial nutritional or respiratory support. Sixty-seven patients (49.3%) needed total support for dressing and 54 (39.7%) for walking/moving. Forty-nine (36.3%) were unable to speak, and 49 (36.0%) needed tube feeding. Sixty-six (48.5%) had respiratory difficulties, and 34 needed mechanical ventilation. Only 27 patients (19.9%) were almost independent. Out of 133 patients, 99 (74.4%) lived in their own home, with the remainder being hospitalized or institutionalized. Of the 34 patients requiring mechanical ventilation, 19 (55.9%) lived in their own home, 12 were hospitalized, and 3 were institutionalized.
A tendency of late-onset for ALS patients, which has been recently reported worldwide, was confirmed in Nagano. There were a significant number of ALS patients who had not developed bulbar palsy or respiratory failure regardless of duration of disease. As expected, artificial respiratory support was a major factor in the confinement of ALS patients to hospitals or nursing institutions.
