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Educational draft · awaiting clinical reviewUse Rapid for revision, not patient-care decisions. Check current national and local guidance and the BNF or BNFC before acting.

Motor neurone disease

Neurology · UK scope · Sources checked 27 Aug 2026 · Clinical review pending

RapidFull
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Escalate

Breathlessness at rest or supine, morning headache with drowsiness, weak cough, inability to handle secretions, choking, aspiration, rapidly worsening bulbar weakness or emotional crisis requires urgent respiratory, neurology and supportive assessment. Normal resting oxygen saturation does not exclude neuromuscular hypoventilation.

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.
  • Refer suspected MND without delay to a neurologist and then a coordinated multidisciplinary team; repeated musculoskeletal treatment can waste crucial planning time.
  • Assess cognition and behaviour because frontotemporal executive or language change affects communication, capacity, adherence and family support.
  • Offer riluzole for amyotrophic lateral sclerosis according to NICE, discussing modest survival benefit and liver and haematological monitoring rather than promising symptom improvement.
  • Monitor forced vital capacity or vital capacity plus SNIP or maximal inspiratory pressure and symptoms every 2–3 months or sooner when decline accelerates.
  • Non-invasive ventilation can improve quality of life and survival; discuss it before crisis, adapt interfaces and communication and clarify that oxygen alone can worsen unrecognised hypoventilation.
  • Discuss gastrostomy early while swallowing, respiratory reserve and decision-making allow choice; waiting for severe weight loss or aspiration increases procedural risk.

    • Key red flags

    Mixed motor signs

    Focal wasting and fasciculation alongside brisk reflexes, spasticity or extensor plantars in progressive weakness strongly suggests combined motor neurone degeneration.

    Bulbar onset

    Progressive slurred or nasal speech, weak tongue with fasciculation, choking and emotional lability can precede obvious limb weakness.

    Respiratory onset

    Orthopnoea, morning headache, unrefreshing sleep, weak cough and daytime somnolence with preserved lungs can reflect diaphragmatic and respiratory muscle weakness.

    Mimic feature

    Prominent numbness, a sensory level, sphincter dysfunction, fluctuating ptosis or pain-dominant weakness should prompt investigation for neuropathy, cord disease or junction disorder.

    Investigation priorities

    01
    Neurological examination across regionsFirst step

    Demonstrate progressive upper and lower motor neurone involvement and identify sensory, ocular or sphincter clues to mimics.

    Management branches

    SuspectRefer progressive motor dysfunction early

    Focal weakness, wasting, fasciculation, spasticity or bulbar dysfunction progresses without a sensory explanation.

    1. Map upper and lower motor signs across regions, document progression and ask about speech, swallowing, breathing, cognition, sensation and sphincters.
    2. Arrange urgent neurology referral and targeted EMG, imaging and mimic bloods rather than requiring textbook-complete ALS before specialist review.
    3. Assess nutrition, cough, supine breathing and communication immediately because supportive needs can predate diagnostic certainty.
    4. On diagnosis link the person and family to the MND multidisciplinary team and provide a named route for rapid change.

    Key medicines

    Riluzole50 mg orally twice daily, taken consistently in relation to food according to the current product information and specialist prescription.Monitor liver enzymes and blood count under the specialist schedule; nausea, fatigue and rare neutropenia or hepatic injury require review and interacting hepatotoxic drugs matter.
    Glycopyrronium bromide for sialorrhoeaUse a low oral or enteral specialist dose and titrate against drooling, secretion thickness and anticholinergic adverse effects.Can thicken secretions, dry the mouth, worsen constipation or retention and make airway clearance harder; pair with cough and secretion assessment.
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    Sources and review status4 sources · checked 27 Aug 2026 · clinical review pending+
    Sources

    Sources and review status

    National guidance is shown before implementation-dependent detail. Typical adult dose examples remain subject to patient factors, contraindications and the live BNF or specialist protocol. Source check completed 27 Aug 2026; clinical approval remains outstanding.

    • NICE NG42 motor neurone diseaseDiagnosis, multidisciplinary review, respiratory, nutrition, communication and end-of-life care.
    • NICE TA20 riluzole for motor neurone diseaseNICE indication for disease-modifying treatment in ALS.
    • BNF riluzole monographDose, administration, adverse effects and laboratory safety.
    • NICE NG127 suspected neurological conditionsRecognition and referral for progressive weakness and bulbar symptoms.

    Editorial contact: Doctor’s Passport editorial team. Source-check date is separate from clinical approval. Report a content problem · Content changes

    Authoring stateRapid draftClinical stateAwaiting reviewJurisdictionUnited Kingdom