Objective: Examining the relationship between psychological status and
survival in amyotrophic lateral sclerosis. Our hypothesis is that psy
chological distress is associated with greater mortality and shorter s
urvival time than psychological well-being. Design: Cross-sectional, l
ongitudinal. The baseline evaluations used were disease severity and 1
0 psychometric tests. A psychological status score was derived from th
ese tests. Survival status was monitored for 3.5 years. Interviewers w
ere blinded to other interviews and data analysis. Setting: Patient's
residence. Patients: The criteria for eligibility were diagnosis of am
yotrophic lateral sclerosis by a neurologist, dementia or alcoholism a
bsent, communication in English, and any severity or length of disease
. It was a volunteer sample consisting of 144 patients from amyotrophi
c lateral sclerosis clinics or community-based amyotrophic lateral scl
erosis support groups. In this sample 66% were men, 94% were white, me
an age at diagnosis was 55 years, 79% were married, 60% had some colle
ge education, and 61%, died during the study. Interventions: None. Mai
n Outcome Measures: End points: mortality during study, survival time
from intake to last follow-up. Results: Comparison between high and lo
w psychological score groups: 32% of high and 82% of low died; surviva
l cur-ves were significantly different. Controlling for confounding fa
ctors (length of illness, disease severity, age), patients with psycho
logical distress had a greater risk of mortality (relative risk, 6.76;
95% confidence limits, 1.69 to 27.12) and greater likelihood of dying
in any given time period (relative risk, 2.24; 95% confidence limits,
1.08 to 4.64) than those with psychological well-being. Conclusion: A
djusting for confounding factors, psychological status is strongly rel
ated to outcome in amyotrophic lateral sclerosis. Further studies on p
sychological status should be done to confirm its prognostic value.