Prolonged survival in motor neuron disease: a descriptive study of the King's database 1990–2002. Issue 7 (1st July 2003)

• Record Type: Journal Article
• Authors: Turner, M R; Parton, M J; Shaw, C E; Leigh, P N; Al-Chalabi, A
• Is Part Of: Journal of neurology, neurosurgery and psychiatry. Volume 74:Issue 7(2003)
• Page Start: 995
• Page End: 997
• Publication Date: 2003-07-01
• Subjects: amyotrophic lateral sclerosis -- database -- motor neurone disease; MND, motor neuron disease -- ALS, amyotrophic lateral sclerosis -- PLS, primary lateral sclerosis -- FVC, forced vital capacity; Neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; Psychiatry -- Periodicals; 616.8
• DOI: 10.1136/jnnp.74.7.995
• Languages: English
• ISSNs: 0022-3050

Abstract

Abstract : Motor neuron disease is a clinically heterogeneous disease with significant differences in survival. The authors have characterised a subset of long term survivors seen in a tertiary clinic over a 12 year period in terms of clinical variables and demographics, comparing them with short term survivors and the remaining population. Thirty of 769 patients survived more than 10 years, corresponding to 4% of the total population. Significantly younger onset of disease symptoms and a predominance of pure upper motor neuron signs at presentation characterised the long term survivors, but factors traditionally regarded as being associated with poor prognosis were also well represented. For a few people with motor neuron disease there remains the hope, whatever the initial presentation, that their subsequent survival will be longer than expected.