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.
- amyotrophic lateral sclerosis
- motor neurone disease
- MND, motor neuron disease
- ALS, amyotrophic lateral sclerosis
- PLS, primary lateral sclerosis
- FVC, forced vital capacity
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Funding: MRT is a Wellcome Trust Clinical Research Training Fellow. MJP is funded through a scholarship from The Guarantors of Brain. AAC is funded by a Medical Research Council Clinician Scientist Fellowship. We are most grateful to the Motor Neuron Disease Association (UK) who provide funding for the MND Care & Research Clinic.
Competing interests: none declared
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