The epidemiology of amyotrophic lateral sclerosis (ALS/MND) in people aged 80 or over

Objective: to describe the clinical features, incidence, survival and process of care of people with Amyotrophic Lateral Sclerosis/ Motor Neurone Disease aged 80 years or more at diagnosis. Design: prospective, population-based descriptive epidemiological study. Setting: Scotland (population 5.1 million), The Scottish Motor Neurone Disease Register 1989-1998. Participants: 135 people aged 80 years or over at diagnosis. Methods: Descriptive Epidemiology of Amyotrophic Lateral Sclerosis/Motor Neurone Disease in the over 80s. Survival described using Kaplan-Meier curves. Results: 135 of 1226 cases (11%) were aged 80 years or more. Sixty-seven (50%) had bulbar onset, and 58 (43%) were men. The standardised incidence was 10.2/100,000 (95% CI 7.4-13.1) in men and 6.1/100,000 (95% CI - 4.3-7.6) in women. Median survival from first symptoms was 1.7 years (IQR 1.0-2.8), less than younger patients (P = 0.0003; log Rank test). We found evidence of differences in the process of care, as older people were less likely to be prescribed Riluzole (OR 0.12, 95% CI = 0.02-0.89) or be assessed by a neurologist (OR 0.76, 95% Cl = 0.67-0.86). Conclusion: this is the first comprehensive report of the epidemiology of Amyotrophic Lateral Sclerosis/Motor Neurone Disease in older people. Clinical presentation and survival differ from the population as a whole. There is evidence of a different process of care. While this may be to the detriment of their survival, this finding would need to be confirmed by further prospective studies.