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Age and Ageing 2004; 33: 131-134
© 2004, British Geriatrics Society


Research Paper

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

Raeburn B. Forbes1, Shuna Colville2 and Robert J. Swingler for the Scottish ALS/MND Register2

1 Department of Neurology, Royal Victoria Hospital, Belfast BT12 6BA, UK
2 Department of Neurology, Ninewells Hospital and Medical School, Dundee DD1 9SY, UK

Address correspondence to: R. B. Forbes. Fax: (+44) 28 902 35258. Email: Raeburnforbes{at}aol.com

Abstract

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% CI = 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.

Keywords: amyotrophic lateral sclerosis, epidemiology, survival, elderly, ALS/MND, motor neurone disease

Received February 18, 2003; Revision received August 27, 2003. accepted in revised form August 27, 2003.


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