Update information

This guideline amalgamates new guidance on the assessment and management of motor neurone disease (MND) with NICE guideline CG105 (published July 2010), and replaces it.

New recommendations have been added in a number of areas, including recognition and referral, information and support at diagnosis, cognitive assessments, prognostic factors, psychosocial and social support, organisation of care, planning for end of life care, and managing symptoms.

Recommendations are marked as [new 2016] if the evidence has been reviewed and the recommendation has been added or updated.

Where recommendations end [2010], the evidence has not been reviewed since the original guideline.

Where recommendations end [2010, amended 2015], the evidence has not been reviewed but changes have been made to the recommendation wording that change the meaning (for example, because of equalities duties or a change in the availability of medicines, or incorporated guidance has been updated). Explanations of the reasons for the changes are given in 'Amended recommendation wording (change to meaning)' for information.

Amended recommendation wording (change to meaning)

Recommendations are labelled [2010, amended 2016] if the evidence has not been reviewed but changes have been made to the recommendation wording that change the meaning.

Recommendation in 2010 guideline

Recommendation in current guideline

Reason for change

Discussions should be appropriate to the stage of the patient's illness, carried out in a sensitive manner and include information on:

  • the possible symptoms and signs of respiratory impairment (see table 1 in recommendation 1.1.7)

  • the natural progression of MND and what to expect in the future

  • the purpose, nature and timing of respiratory function tests, and explanations of the test results

  • available interventions for managing respiratory impairment, including the benefits and limitations of each intervention

  • accessing and using respiratory equipment, including that for non‑invasive ventilation

  • how non-invasive ventilation (as a treatment option) can improve symptoms associated with respiratory impairment and can be life prolonging, but does not stop progression of the underlying disease

  • how non-invasive ventilation can be withdrawn

  • palliative strategies as an alternative to non‑invasive ventilation.(1.1.3)

1.14.2 Discussions about non‑invasive ventilation should be appropriate to the stage of the person's illness, carried out in a sensitive manner and include information on:

  • the possible symptoms and signs of respiratory impairment (see box 1)

  • the purpose, nature and timing of respiratory function tests, and explanations of the test results

  • how non-invasive ventilation (as a treatment option) can improve symptoms associated with respiratory impairment and can be life prolonging, but does not stop progression of the underlying disease. [2010, amended 2016]

Amended to include specific reference to non‑invasive ventilation, and to ensure consistency with new recommendations on information about non‑invasive ventilation.

1.1.5 Provide the patient and their family and carers with support and assistance to manage non‑invasive ventilation. This should include:

  • training on using non‑invasive ventilation and ventilator interfaces, for example:

    • emergency procedures

    • night-time assistance if the patient is unable to use the equipment independently (for example, emergency removal or replacement of interfaces)

    • how to use the equipment with a wheelchair or other mobility aids if required

    • what to do if the equipment fails

  • assistance with secretion management

  • information on general palliative strategies

  • an offer of ongoing emotional and psychological support[1] for the patient and their family and carers.

1.14.23 Provide the person and their family and/or carers (as appropriate) with support and assistance to manage non‑invasive ventilation. This should include:

  • training on using non‑invasive ventilation and ventilator interfaces, for example:

    • emergency procedures

    • night-time assistance if the person is unable to use the equipment independently (for example, emergency removal or replacement of interfaces)

    • how to use the equipment with a wheelchair or other mobility aids if required

    • what to do if the equipment fails

  • assistance with secretion management

  • information on general palliative strategies

  • an offer of ongoing emotional and psychological support for the person and their family and carers. [2010, amended 2016]

Footnote removed as psychological and social support are included in the new guideline.

1.1.7 Monitor the symptoms and signs listed in table 1 routinely to detect potential respiratory impairment.

1.14.7 Monitor the symptoms and signs listed in box 1 to detect potential respiratory impairment. [2010, amended 2016]

'Routinely' removed as new recommendations for the multidisciplinary team advise on frequency of assessment.

1.1.10 A healthcare professional with appropriate competencies should perform the respiratory function tests every 3 months, although tests may be performed more or less often depending on:

  • whether there are any symptoms and signs of respiratory impairment (see recommendation 1.1.7)

  • the rate of progression of MND

  • the patient's preference and circumstances.

1.14.10 A healthcare professional with appropriate competencies should perform the respiratory function tests every 2–3 months, although tests may be performed more or less often depending on:

  • whether there are any symptoms and signs of respiratory impairment (see box 1)

  • the rate of progression of MND

  • the person's preference and circumstances. [2010, amended 2016]

Time period removed as already included in recommendations for the multidisciplinary team.

1.1.15 If any of the results listed in table 2 is obtained, discuss with the patient and (if the patient agrees) their family and carers:

  • the impact of respiratory impairment

  • treatment options

  • possible referral to a specialist respiratory service for further assessment.

1.14.15 If any of the results listed in box 2 is obtained, discuss with the person and (if appropriate) their family and carers:

  • their respiratory impairment

  • their treatment options

  • possible referral to a respiratory ventilation service for further assessment based on discussion with the person, and their wishes. [2010, amended 2016]

Wording changed for consistency and to emphasise patient choice for referral.

1.1.16 Base decisions on respiratory function tests for a patient with a diagnosis of dementia on considerations specific to their needs and circumstances, such as:

  • their ability to give consent[3]

  • their understanding of the tests

  • their tolerance of the tests and willingness to undertake them

  • the impact on their family and carers

  • whether they are capable of receiving non‑invasive ventilation.

1.14.16 Base decisions on respiratory function tests for a person with a diagnosis of frontotemporal dementia on considerations specific to their needs and circumstances, such as:

  • their ability to give consent2

  • their understanding of the tests

  • their tolerance of the tests and willingness to undertake them

  • the impact on their family and carers

  • whether they are capable of receiving non‑invasive ventilation. [2010, amended 2016]

2 See Mental Capacity Act 2005.

Amended for consistency without change in meaning.

'dementia' changed to 'frontotemporal dementia'.

Footnote wording has changed to refer to the Mental Capacity Act 2005

1.1.17 Offer a trial of non‑invasive ventilation if the patient's symptoms and signs and the results of the respiratory function tests indicate that the patient is likely to benefit from the treatment.

  • Discuss both the benefits and limitations of the intervention with the patient and their family and carers.

  • Only consider a trial of non‑invasive ventilation for a patient who has severe bulbar impairment or severe cognitive problems that may be related to respiratory impairment if they may benefit from an improvement in sleep‑related symptoms or correction of hypoventilation.

1.14.17 Offer a trial of non‑invasive ventilation if the person's symptoms and signs and the results of the respiratory function tests indicate that the person is likely to benefit from the treatment. [2010, amended 2016]

1.14.18 Consider a trial of non‑invasive ventilation for a person who has severe bulbar impairment or severe cognitive problems that may be related to respiratory impairment only if they may benefit from an improvement in sleep‑related symptoms or correction of hypoventilation. [2010, amended 2016]

Amended for consistency with new recommendations.

New recommendations developed for information following evidence review on stopping non‑invasive ventilation.

1.1.19 Before starting non‑invasive ventilation, the multidisciplinary team should prepare a comprehensive care plan, after discussion with the patient and their family and carers (who should be offered a copy of the plan). This should cover:

  • long-term support provided by the multidisciplinary team

  • the initial frequency of respiratory function tests and monitoring of respiratory impairment

  • the frequency of clinical reviews of symptomatic and physiological changes

  • the provision of carers

  • arrangements for device maintenance and 24‑hour emergency clinical and technical support

  • secretion management and respiratory physiotherapy assessment, including cough‑assist therapy (if required)

  • training in and support for the use of non‑invasive ventilation for the patient and their family and carers

  • regular opportunities to discuss the patient's wishes in relation to continuing or withdrawing non‑invasive ventilation, and other end‑of‑life considerations (see also recommendations 1.1.24 and 1.1.25).

1.14.20 Before starting non‑invasive ventilation, the multidisciplinary team in conjunction with the ventilation service should prepare a comprehensive care plan, after discussion with the person and their family and carers (who should be offered a copy of the plan). This should cover:

  • long-term support provided by the multidisciplinary team

  • the initial frequency of respiratory function tests and monitoring of respiratory impairment

  • the frequency of clinical reviews of symptomatic and physiological changes

  • the provision of carers

  • arrangements for device maintenance and 24‑hour emergency clinical and technical support

  • secretion management and respiratory physiotherapy assessment, including cough‑assist therapy (if required)

  • training in and support for the use of non‑invasive ventilation for the person and their family and carers

  • regular opportunities to discuss the person's wishes in relation to continuing or withdrawing non‑invasive ventilation. [2010, amended 2016]

Part of last bullet point deleted as replaced by recommendations on Planning for end of life.

1.1.23 Before a decision is made on the use of non-invasive ventilation for a patient with a diagnosis of dementia, the neurologist from the multidisciplinary team should carry out an assessment that includes:

  • the patient's capacity to make decisions and to give consent[3]

  • the severity of dementia and cognitive problems

  • whether the patient is likely to accept treatment

  • whether the patient is likely to achieve improvements in sleep‑related symptoms and/or behavioural improvements

  • a discussion with the patient's family and/or carers (with the patient's consent if they have the capacity to give it).

1.14.25 Before a decision is made on the use of non‑invasive ventilation for a person with a diagnosis of frontotemporal dementia, the multidisciplinary team in conjunction with the ventilation service should carry out an assessment that includes:

  • the person's capacity to make decisions and to give consent2

  • the severity of dementia and cognitive problems

  • whether the person is likely to accept treatment

  • whether the person is likely to achieve improvements in sleep‑related symptoms and/or behavioural improvements

  • a discussion with the person's family and/or carers (with the person's consent if they have the capacity to give it). [2010, amended 2016]

2See Mental Capacity Act 2005.

Amended to update wording and reflect changes to law.

'dementia' changed to 'frontotemporal dementia' and footnote changed to reflect Mental Capacity Act.

Footnote wording has changed to refer to the Mental Capacity Act 2005.

ISBN: 978-1-4731-1690-0

  • National Institute for Health and Care Excellence (NICE)