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  • The GP’s role in motor neurone disease (MND) is pivotal, not just in coordinating services, but also in the prompt management of symptoms, which can generally improve quality of life

How does amyotrophic lateral sclerosis/motor neurone disease present?

  • Presentation is commonly in two patterns: either ‘spinal’ (75%) with weakness in one or more limbs, or ‘bulbar’ with speech and swallowing problems (25%)
  • The earliest symptoms may be non-specific such as muscle cramps or persistent muscle twitching (fasciculation). However it should be remembered that both symptoms are frequently a normal phenomenon in healthy people, and fasciculation tends to be less intrusive to MND patients. There may be insidious ‘clumsiness’ and/or falls, which go unreported
  • The recognition of progressive weakness without sensory involvement is the key to prompting the consideration of MND as a diagnosis

What differential diagnoses need to be considered?

  • Other diseases which lead to muscular wasting, especially of the upper limbs, should be considered. They are (in approximate order of frequency):
    • cervical spondylosis
    • inclusion body myositis
    • myasthenia gravis
    • multi-focal motor neuropathy with conduction block (MMN/CB)
    • cervical rib
    • peripheral nerve lesions
    • syringomyelia
    • intramedullary spinal tumour
    • polymyositis

What is the role of the multi-disciplinary team (MDT)?

  • The optimal care of MND patients and their families/carers is best delivered by a well co-ordinated multi-disciplinary team approach:
    • physiotherapists have expertise in relation to mobility and posture
    • early referral to speech and language therapy services at the first suggestion of bulbar dysfunction is important
    • prompt referral to a dietitian is advised if there is concern regarding inadequate caloric intake. Weight loss has a significant adverse effect on survival


1. Does the patient have one or more of these symptoms?

  • Bulbar features:
    • dysarthria
      • slurred or quiet speech often when tired
    • dysphagia
      • liquids and/or solids
      • excessive saliva
      • choking sensation especially when lying flat
    • tongue fasciculations
  • Limb features:
    • focal weakness
    • falls/trips—from foot drop
    • loss of dexterity
    • muscle wasting
    • muscle twitching/fasciculations
    • cramps
    • no sensory features
  • Respiratory features:
    • hard to explain respiratory symptoms
    • shortness of breath on exertion
    • excessive daytime sleepiness
    • fatigue
    • early morning headache
    • orthopnoea
  • Cognitive features (may not be a presenting feature):
    • behavioural change
    • emotional lability (not related to dementia)
    • fronto-temporal dementia

2. Is there progression?

  • Supporting factors:
    • asymmetrical features
    • age—MND can present at any age
    • positive family history of MND or other neurodegenerative disease
  • Factors NOT supportive of MND diagnosis
    • bladder/bowel involvement
    • prominent sensory symptoms
    • double vision/ptosis
    • improving symptoms

If yes to 1 and 2, query MND and refer to Neurology

  • If you think it might be MND please state explicitly in the referral letter
  • Common causes of delay are initial referral to ENT or orthopaedic services
  • The RCGP accredited version of the Red Flag diagnosis tool along with an accompanying presentation are available to download from www.mndassociation.org/redflag

Useful sources of information


full guidelines available from…


Motor Neurone Disease Association and Royal College of General Practitioners. Red flag diagnosis tool  2014
First included: February 2015.