Motor Neurone Disease
Motor neurone disease (MND) is a progressive neurodegenerative syndrome affecting upper and lower motor neurones, usually with preserved sensation and eye movements but possible cognitive, behavioural, bulbar and respiratory involvement; suspected MND needs prompt specialist assessment and coordinated multidisciplinary care.
Definition
Motor neurone disease is a group of progressive neurodegenerative disorders affecting upper and lower motor neurones, including amyotrophic lateral sclerosis, progressive bulbar palsy, progressive muscular atrophy and primary lateral sclerosis. It causes progressive motor disability with variable bulbar, respiratory, cognitive and behavioural involvement and is diagnosed clinically with supportive investigations that exclude mimics.
First principles
Recognise the motor-system pattern, but do not make it absolute
Upper motor neurone involvement causes spasticity, hyperreflexia and extensor plantar responses; lower motor neurone involvement causes weakness, wasting, fasciculations and reduced reflexes. MND may show both patterns in one person, but the distribution and timing vary between phenotypes. Sensory loss, a clear sensory level, early sphincter dysfunction, ophthalmoplegia or a structural explanation should prompt a search for an alternative or additional diagnosis rather than being dismissed as atypical MND.
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