Synopsis
Recognise progressive upper and lower motor neurone syndromes, expedite multidisciplinary diagnosis, and anticipate communication, swallowing and respiratory needs while preserving autonomy.
- Motor neurone disease causes progressive motor weakness with upper motor neurone signs, lower motor neurone signs or both, usually without sensory loss or sphincter disturbance.
- Lower motor neurone findings are wasting, fasciculation and weakness with reduced reflexes; upper motor neurone findings include spasticity, brisk reflexes, clonus and extensor plantar responses.
- Presentation may be limb onset, bulbar onset with dysarthria or dysphagia, or less commonly respiratory onset; persistent isolated symptoms still need mimic exclusion.
Key red flags
Focal wasting and fasciculation alongside brisk reflexes, spasticity or extensor plantars in progressive weakness strongly suggests combined motor neurone degeneration.
Investigation priorities
Demonstrate progressive upper and lower motor neurone involvement and identify sensory, ocular or sphincter clues to mimics.
Management branches
Focal weakness, wasting, fasciculation, spasticity or bulbar dysfunction progresses without a sensory explanation.
- Map upper and lower motor signs across regions, document progression and ask about speech, swallowing, breathing, cognition, sensation and sphincters.
- Arrange urgent neurology referral and targeted EMG, imaging and mimic bloods rather than requiring textbook-complete ALS before specialist review.
Key medicines
Riluzole50 mg orally twice daily, taken consistently in relation to food according to the current product information and specialist prescription.
Glycopyrronium bromide for sialorrhoeaUse a low oral or enteral specialist dose and titrate against drooling, secretion thickness and anticholinergic adverse effects.