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Summary for primary care

Motor Neurone Disease: Assessment and Management

This Guidelines summary includes recommendations from the NICE guideline on the assessment and management of motor neurone disease.

Recognition and Referral

  • Ensure that robust protocols and pathways are in place to:
    • inform healthcare professionals about motor neurone disease (MND) and how it may present
    • inform healthcare professionals in all settings about local referral arrangements
    • ensure continued and integrated care for people with MND across all care settings
  • Be aware that MND causes progressive muscular weakness that may first present as isolated and unexplained symptoms. These symptoms may include:
    • functional effects of muscle weakness, such as loss of dexterity, falls or trips
    • speech or swallowing problems, or tongue fasciculations (this is known as bulbar presentation)
    • muscle problems, such as weakness, wasting, twitching, cramps, and stiffness
    • breathing problems, such as shortness of breath on exertion or respiratory symptoms that are hard to explain
    • effects of reduced respiratory function, such as excessive daytime sleepiness, fatigue, early morning headache or shortness of breath when lying down
  • Be aware that MND may first present with cognitive features, which may include:
    • behavioural changes
    • emotional lability (not related to dementia)
    • frontotemporal dementia
  • If you suspect MND, refer the person without delay and specify the possible diagnosis in the referral letter. Contact the consultant neurologist directly if you think the person needs to be seen urgently
  • Provide information and support for people and their family members and/or carers (as appropriate) throughout the diagnostic process, particularly during periods of diagnostic uncertainty or delay

Information and Support at Diagnosis

  • Information about the diagnosis, prognosis and management of MND should be given by a consultant neurologist with up-to-date knowledge and experience of treating people with MND unless it is clinically necessary to give the diagnosis in an urgent situation. The neurologist should have knowledge and expertise in the following:
    • symptoms of MND
    • types and possible causes of MND
    • treatment options
    • how MND may progress (including cognitive and behavioural changes) and how progression may affect the treatments offered
    • crisis prevention (for example, if there is an acute hospital admission or a breakdown in care arrangements)
    • opportunities for people with MND to be involved in research
    • likely needs and concerns of people with MND and their family members and/or carers (as appropriate)
    • advance care planning
  • Ask people about how much information they wish to receive about MND, and about their preferences for involving their family members and/or carers (as appropriate)
  • Ensure people are provided with information about MND and support at diagnosis or when they ask for it. If the person agrees, share the information with their family members and/or carers (as appropriate). Information should be oral and written, and may include the following:
    • what MND is
    • types and possible causes
    • likely symptoms and how they can be managed
    • how MND may progress
    • treatment options
    • where the person's appointments will take place
    • which healthcare professionals and social care practitioners will undertake the person's care
    • expected waiting times for consultations, investigations and treatments
    • local services (including social care and specialist palliative care services) and how to get in touch with them
    • local support groups, online forums and national charities, and how to get in touch with them
    • legal rights, including social care support, employment rights, and benefits
    • requirements for disclosure, such as notifying the Driver and Vehicle Licensing Agency (DVLA)
    • opportunities for advance care planning
  • When MND is diagnosed, provide people with a single point of contact for the specialist MND multidisciplinary team (see the section Organisation of care). Provide information about what to do if there are any concerns between assessments or appointments, during 'out-of-hours' or in an emergency, or if there is a problem with equipment
  • Offer the person with MND a face-to-face, follow-up appointment with a healthcare professional from the multidisciplinary team, to take place within 4 weeks of diagnosis
  • When MND is suspected or confirmed, inform the person's GP without delay and provide information about the likely prognosis
  • Set aside enough time to discuss the person's concerns and questions, which may include the following:
    • what will happen to me?
    • are there any treatments available?
    • is there a cure?
    • how long will I live?
    • what will the impact on my day-to-day life be?
    • what will happen next with my healthcare?
    • will my children get MND?
    • how do I tell my family and friends?
    • how will I die?
  • If the person has any social care needs, refer them to social services for an assessment. Be aware that some people with MND may not have informal care available, and may live alone or care for someone else
  • Advise carers that they have a legal right to have a Carer's Assessment of their needs; support them with requesting this from their local authority

Cognitive Assessments

  • Be aware that people with MND and frontotemporal dementia may lack mental capacity. Care should be provided in line with the Mental Capacity Act 2005
  • At diagnosis, and if there is concern about cognition and behaviour, explore any cognitive or behavioural changes with the person and their family members and/or carers as appropriate. If needed, refer the person for a formal assessment in line with the NICE guideline on dementia
  • Tailor all discussions to the person's needs, taking into account their communication ability, cognitive status and mental capacity

Prognostic Factors

  • When planning care take into account the following prognostic factors, which are associated with shorter survival if they are present at diagnosis:
    • speech and swallowing problems (bulbar presentation)
    • weight loss
    • poor respiratory function
    • older age
    • Lower Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS or ALSFRS-R) score
    • shorter time from first developing symptoms to time of diagnosis

Organisation of Care

  • Provide coordinated care for people with MND, using a clinic-based, specialist MND multidisciplinary team approach. The clinic may be community or hospital based
  • The multidisciplinary team should:
    • include healthcare professionals and social care practitioners with expertise in MND, and staff who see people in their home
    • ensure effective communication and coordination between all healthcare professionals and social care practitioners involved in the person's care and their family members and/or carers (as appropriate)
    • carry out regular, coordinated assessments at the multidisciplinary team clinic (usually every 2–3 months) to assess people's symptoms and needs
    • provide coordinated care for people who cannot attend the clinic, according to the person's needs
  • The multidisciplinary team should assess, manage and review the following areas, including the person's response to treatment:
    • weight, diet, nutritional intake and fluid intake, feeding and swallowing
    • muscle problems, such as weakness, stiffness and cramps
    • physical function, including mobility and activities of daily living
    • saliva problems, such as drooling of saliva (sialorrhoea) and thick, tenacious saliva
    • speech and communication
    • cough effectiveness
    • respiratory function, respiratory symptoms and non-invasive ventilation
    • pain and other symptoms, such as constipation
    • cognition and behaviour
    • psychological support needs
    • social care needs
    • end of life care needs
    • information and support needs for the person and their family members and/or carers (as appropriate)
  • The core multidisciplinary team should consist of healthcare professionals and other professionals with expertise in MND, and should include the following:
    • neurologist
    • specialist nurse
    • dietitian
    • physiotherapist
    • occupational therapist
    • respiratory physiologist or a healthcare professional who can assess respiratory function
    • speech and language therapist
    • a healthcare professional with expertise in palliative care (MND palliative care expertise may be provided by the neurologist or nurse in the multidisciplinary team, or by a specialist palliative care professional)
  • The multidisciplinary team should have established relationships with, and prompt access to, the following:
    • clinical psychology and neuropsychology
    • social care
    • counselling
    • respiratory ventilation services
    • specialist palliative care
    • gastroenterology
    • orthotics
    • wheelchair services
    • assistive technology services
    • alternative and augmentative communication (AAC) services
    • community neurological care teams
  • Tailor the frequency of the multidisciplinary team assessments to the person's symptoms and needs, with more or less frequent assessments as needed
  • Ensure arrangements are in place to trigger an earlier multidisciplinary team assessment if there is a significant change in symptoms identified by the person, family members and/or carers (as appropriate), or healthcare professionals
  • Tailor the multidisciplinary team assessment to the person's needs, for example, adjust the format if the person has cognitive or behaviour changes or difficulties with communication
  • Inform all healthcare professionals and social care practitioners involved in the person's care about key decisions reached with the person and their family members and/or carers (as appropriate)
  • Ensure that all healthcare professionals and social care practitioners involved in the person's care are aware that MND symptoms may get worse quickly, and that people with MND will need repeated, ongoing assessments. Priority should be given to ensuring continuity of care and avoiding untimely case closure
  • Consider referral to a specialist palliative care team for people with current or anticipated significant or complex needs, for example, psychological or social distress, troublesome or rapidly progressing symptoms and complex future care planning needs
  • For guidance on the use of riluzole for people with MND, see the NICE technology appraisal guidance on the use of riluzole (Rilutek) for the treatment of motor neurone disease