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Neurology

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.

In a nutshell

Motor neurone disease is a progressive motor-neurone syndrome causing upper motor neurone signs, lower motor neurone signs, bulbar dysfunction and respiratory impairment in varying combinations. Sensation and eye movements are often preserved, but cognition and behaviour can be affected. There is no cure: prompt specialist referral, coordinated multidisciplinary care, riluzole for suitable ALS, respiratory and nutrition planning, symptom control, communication support and advance care planning are central.

Classic presentation

A person develops steadily progressive focal weakness with wasting or fasciculations, later showing brisk reflexes or spasticity, with no clear sensory level. Ask about dysarthria, dysphagia, choking, orthopnoea, morning headache, daytime sleepiness, weight loss, cognition and behaviour.

Key points

  • Suspected MND needs prompt neurological referral; state the possible diagnosis clearly and do not wait for a complete textbook pattern.
  • The syndrome may contain upper motor neurone signs, lower motor neurone signs or both; the distribution and rate of progression vary.
  • There is no single diagnostic test: neurophysiology supports the diagnosis and MRI or blood tests exclude mimics.
  • Bulbar and respiratory symptoms are high priority; ask about choking, ineffective cough, orthopnoea, sleep-related symptoms and daytime somnolence.
  • Assess respiratory function serially and discuss non-invasive ventilation through the specialist respiratory pathway when impairment is present.
  • Discuss swallowing, nutrition and gastrostomy early, alongside speech and language therapy and communication aids.
  • Riluzole is an option for suitable ALS, started with specialist input and monitored under current BNF or shared-care guidance.
  • Cognitive or behavioural change can affect communication, capacity and safety; offer advance care planning sensitively and early enough to be useful.
  • A person with MND must notify the DVLA; give urgent safety-net advice for choking, secretion failure, breathlessness or sudden deterioration.

First-line investigation

Urgent specialist clinical assessment with neurophysiology and targeted tests to exclude mimics, plus baseline respiratory, swallowing, nutrition, communication and cognitive assessment.

Management

Refer and coordinate specialist care

  • Refer suspected MND without delay, clearly identify the concern, and establish a specialist multidisciplinary pathway with a named contact and emergency plan.1,5

Offer riluzole when ALS is suitable

  • Discuss specialist-led riluzole for the ALS form of MND; use current BNF and shared-care arrangements for formulation, contraindications, monitoring and adverse effects.3,1,7

Monitor breathing and plan ventilation

  • Perform serial respiratory assessment, ask about sleep-related hypoventilation and cough effectiveness, and discuss non-invasive ventilation through the respiratory ventilation service when indicated.6,1

Protect swallowing, nutrition and communication

  • Involve speech and language therapy and dietetics early, assess swallowing and weight, discuss gastrostomy before respiratory or nutritional reserve is critically reduced, and provide communication aids without delay.1,2

Preserve function and control symptoms

  • Tailor physiotherapy, occupational therapy, orthoses, mobility equipment, cough assistance, saliva management and symptom medicines to goals, swallowing, cognition and respiratory safety.1,7

Plan ahead and safety-net

  • Discuss advance care planning at diagnosis and key progression points, integrate palliative and carer support, give an emergency contact plan, and ensure the person reports MND to the DVLA.5,1,10

Exam traps

  • A normal MRI or an EMG showing denervation does not independently diagnose MND.
  • Sensory loss, a sensory level, early sphincter dysfunction, ophthalmoplegia or a structural lesion should prompt an alternative diagnosis search.
  • Do not wait for severe respiratory symptoms before monitoring respiratory function or discussing non-invasive ventilation.
  • Riluzole is not an automatic treatment for every MND phenotype; use specialist and current BNF or shared-care guidance.
  • Gastrostomy should be discussed early because late placement can carry greater respiratory and procedural risk, but the person's preference remains central.
  • MND is not purely a motor disease: cognitive and behavioural change can alter capacity, communication and care planning.
  • Sudden breathlessness, choking, inability to clear secretions or reduced consciousness is an emergency, not routine disease progression.

Illustrations

Focal shoulder-girdle wasting in monomelic amyotrophyAnterior and posterior clinical views showing marked unilateral atrophy of the left deltoid, supraspinatus and infraspinatus muscles in monomelic amyotrophy, a focal motor-neuron disorder. Use this as a differential illustration rather than a typical image of MND.Al-Ghawi E et al., Journal of Medical Case Reports 2016, CC-BY-4.0 · CC-BY-4.0

Key sources

  1. NICE NG42, Motor neurone disease: assessment and management (NICE guideline published 2016 and last updated 23 July 2019; current recommendations cover recognition and referral, coordinated multidisciplinary care, cognition, symptoms, nutrition, communication, respiratory monitoring, non-invasive ventilation and advance care planning)Updated 23 Jul 2019
  2. NHS, Motor neurone disease (Current NHS condition information last reviewed 19 February 2025: symptoms, diagnosis, types, treatment, multidisciplinary support and progressive functional impact)Updated 19 Feb 2025
  3. NICE TA20, Guidance on the use of riluzole for the treatment of motor neurone disease (NICE technology appraisal recommending riluzole as an option for the amyotrophic lateral sclerosis form of MND; prescribing and monitoring must follow current BNF and local shared-care arrangements)Updated 24 Jan 2001
  4. Motor Neurone Disease Association, Riluzole information for people with or affected by MND (UK specialist charity information dated January 2026: specialist initiation and shared-care context, ALS-focused benefit, formulations for swallowing difficulty, adverse effects and monitoring; used as supplementary patient-facing context rather than to override NICE or BNF)Updated 1 Jan 2026
  5. NICE QS126, Motor neurone disease quality standard (NICE quality standard linked to NG42: specialist information at diagnosis, coordinated care and opportunities to discuss preferences and concerns about end-of-life care at diagnosis and key progression points)Updated 24 Feb 2016
  6. NICE NG42, respiratory-function and non-invasive-ventilation recommendations (Current NICE NG42 respiratory pathway: baseline resting room-air oxygen saturation plus FVC or VC and/or SNIP or MIP, serial assessment tailored to symptoms and progression, blood gas and ventilation-service escalation when indicated)Updated 23 Jul 2019
  7. BNF, current prescribing information for riluzole and symptom-control medicines (Current UK prescribing source for riluzole dose, contraindications, interactions, formulations, monitoring and symptom medicines; direct access was restricted and the available browser session was unavailable, so unsupported doses were omitted)
  8. NICE CG32, Nutrition support for adults (NICE nutrition-support guidance used for the general enteral-feeding context; MND-specific timing, swallowing assessment and gastrostomy decisions remain anchored to NG42)Updated 22 Feb 2006
  9. NICE NG142, End of life care for adults: service delivery (NICE end-of-life service guidance used for accessible, documented and coordinated advance-care planning alongside the condition-specific NG42 recommendations)Updated 16 Oct 2019
  10. GOV.UK, Motor neurone disease and driving (Current DVLA patient guidance: a person with MND must tell the DVLA using the relevant reporting route for their licence category)
  11. GOV.UK, Neurological disorders: assessing fitness to drive (DVLA professional guidance updated 7 November 2025 for chronic neurological disorders, including MND, and the effect of neurological impairment on driving)Updated 7 Nov 2025

This page is exam revision material, not medical advice, and must not be used for patient care. Always check drug doses against the BNF and current guidance. Full disclaimer.