Prolonged survival in motor neuron disease: a descriptive study of the King’s database 1990–2002

• Published in: Journal of neurology, neurosurgery and psychiatry neurosurgery and psychiatry, 2003-07, Vol.74 (7), p.995-997
• Main Authors: Turner, M R, Parton, M J, Shaw, C E, Leigh, P N, Al-Chalabi, A
• Format: Article
• Language: English
• Subjects: Adult; Age; Age of Onset; Aged; ALS; Amyotrophic lateral sclerosis; Biological and medical sciences; Cohort Studies; Databases, Factual; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Female; forced vital capacity; Funding; FVC; Humans; Male; Medical prognosis; Medical sciences; Middle Aged; MND; motor neuron disease; Motor Neuron Disease - pathology; motor neurone disease; Neurology; Ostomy; PLS; Population; primary lateral sclerosis; Prognosis; Risk Factors; Short Report; Studies; Survival
• ISSN: 0022-3050; 1468-330X
• DOI: 10.1136/jnnp.74.7.995

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.