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Neurological diseases cause physical, psychosocial, and spiritual or existential suffering from the time of their diagnosis. Palliative care focuses on improving quality of life for people with serious illness and their families by addressing this multidimensional suffering. Evidence from clinical trials supports the ability of palliative care to improve patient and caregiver outcomes by the use of outpatient or home-based palliative care interventions for people with motor neuron disease, multiple sclerosis, or Parkinson's disease; inpatient palliative care consultations for people with advanced dementia; telephone-based case management for people with dementia in the community; and nurse-led discussions with decision aids for people with advanced dementia in long-term care. Unfortunately, most people with neurological diseases do not get the support that they need for their palliative care under current standards of healthcare. Improving this situation requires the deployment of routine screening to identify individual palliative care needs, the integration of palliative care approaches into routine neurological care, and collaboration between neurologists and palliative care specialists. Research, education, and advocacy are also needed to raise standards of care., Competing Interests: Declaration of interests CJC has received research grants from the National Institutes of Health and is secretary for the International Neuropalliative Care Society. RG has received honoraria from Abbott Laboratories and travel support from Sun Pharma and Intas Pharma, and is a board member for the International Neuropalliative Care Society. LCH receives funding from the National Institute on Aging, Centers for Medicare and Medicaid Services Civil Monetary Penalty Fund, National Institute for Nursing Research, and the Patient Centered Outcomes Research Institute. BMK has received research grants from the National Institutes of Health and the Patient Centered Outcomes Research Institute; has received honoraria from the Parkinson Foundation and International Movement and Parkinson Disease Society, Davis Phinney Foundation, and American Academy of Neurology; has received royalties from Elsevier; and is President of the International Neuropalliative Care Society. JM has received research grants from the Patient Centered Outcomes Research Institute, Canadian Open Parkinson Network, Brain Canada, the Canadian Consortium on Neurodegeneration in Aging, the National Institutes of Health, and University Hospital Foundation; has received royalties from Elsevier; and is American Academy of Neurology Vice President, US delegate for Oxford University Press, and on the board of directors of the Parkinson Foundation, and on the board of directors of the International Neuropalliative Care Society (20192–021). DJO has received royalties from Oxford University Press and Springer, and is a board member for the International Neuropalliative Care Society. SZP has received support from the Gordon and Betty Moore Foundation, American Academy of Hospice and Palliative Medicine, Palliative Care Quality Collaborative, Cambia Foundation, Hellman Foundation, Alafi Family Foundation, Stupski Foundation, Archstone Foundation, and Unihealth Foundation; has received received royalties from McGraw Hill Publishers; and is President of Palliative Care Quality Collaborative, on the board of directors of By the Bay Health, and advisory board Chair for Sojourns Scholar Leadership Program. PH is a board member for the International Neuropalliative Care Society. MS is on the membership committee for the International Neuropalliative Care Society. All other authors declare no competing interests. LCH (R01-AG065394) and BMK (K02 AG062745) received support from the National Institute of Aging for this work., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Competing Interests: Competing interests: See bmj.com/about-bmj/freelance-contributors.

Patients, and their families, with Parkinson's disease (PD) and related disorders face many issues, including physical, psychological, social and spiritual. Palliative care is an essential part of care from the time of diagnosis, and should be provided by all services involved with the patient and family. Specialist palliative care is able to support the overall care particularly for complex issues-whether symptoms or psychosocial and spiritual problems, ethical and decisions making issues, and at the end of life. This should be in collaboration with other teams, working together to improve the quality of life (QOL) of patients and families, supporting the professional teams and enabling patients to be as fully involved in the decisions about their care and at the end of life.

Palliative care has been increasingly shown to improve patient outcomes, in particular symptom management, quality of life and patient and family satisfaction. This has been shown within the care of people with cancer, neurological disease and heart failure but the methods by which palliative care is introduced earlier in the diagnosis is still complex. There are different models of increasing integration- care solely by the specialist, referral to other professionals or members of the team as necessary or an integrated model where palliative care interventions occur early in the disease progression. The evidence for the effectiveness of early involvement is being developed. The challenge for the future is increasing the awareness of professionals, patients and families that palliative care may be helpful and may improve the quality of life, and maybe the survival, of patients. The complex team interactions need to be acknowledged and all professionals involved to be focussed on improving the outcome for the patient and family.

Purpose of Review: Palliative care is an approach that improves the quality of life of patients and their families facing the problem associated with life-threatening illness. Neuromuscular disorders (NMDs) are characterized by progressive muscle weakness, leading to pronounced and incapacitating physical disabilities. Most NMDs are not amenable to curative treatment and would thus qualify for palliative care. Amyotrophic lateral sclerosis is a relentlessly progressive disease, which leads to death about 2 years after onset due to respiratory muscle weakness. Increasingly, neurologists caring for these patients learn to apply the principles of palliative care. However, this does not yet apply to other well known and frequently occurring NMDs., Recent Findings: There is sparse literature on palliative care in NMDs such as Duchenne muscular dystrophy, spinal muscular atrophy, muscular dystrophies, some congenital myopathies, Pompe's disease and myotonic dystrophy type 1. These NMDs are often associated with imminent respiratory insufficiency and/or heart failure leading to a reduced life expectancy. Reasons for underutilization may include misconceptions about palliative care amongst patients, family carers and healthcare professionals or lack of awareness of the usefulness of this approach in these severely affected patients and the possibilities of integration of palliative principles into care for children and adults with NMDs., Summary: There is an urgent need for increased attention to the development of palliative care in chronic progressive neuromuscular diseases associated with increasing functional incapacities and premature death. This will require education and training of the healthcare professionals, involvement of patient associations and funding to perform research.

Background: Dementia is the most common neurological disorder worldwide and is a life-limiting condition, but very often is not recognised as such. People with dementia, and their carers, have been shown to have palliative care needs equal in extent to those of cancer patients. However, many people with advanced dementia are not routinely being assessed to determine their palliative care needs, and it is not clear why this is so., Main Body: An interdisciplinary workshop on "Palliative Care in Neurodegeneration, with a focus on Dementia", was held in Cork, Ireland, in May 2016. The key aim of this workshop was to discuss the evidence base for palliative care for people with dementia, to identify 'gaps' for clinical research, and to make recommendations for interdisciplinary research practice. To lead the discussion throughout the day a multidisciplinary panel of expert speakers were brought together, including both researchers and clinicians from across Ireland and the UK. Targeted invitations were sent to attendees ensuring all key stakeholders were present to contribute to discussions. In total, 49 experts representing 17 different academic and practice settings, attended. Key topics for discussion were pre-selected based on previously identified research priorities (e.g. James Lind Alliance) and stakeholder input. Key discussion topics included: i. Advance Care Planning for people with Dementia; ii. Personhood in End-of-life Dementia care; iii. Topics in the care of advanced dementia at home. These topics were used as a starting point, and the ethos of the workshop was that the attendees could stimulate discussion and debate in any relevant area, not just the key topics, summarised under iv. Other priorities., Conclusions: The care experienced by people with dementia and their families has the potential to be improved; palliative care frameworks may have much to offer in this endeavour. However, a solid evidence base is required to translate palliative care into practice in the context of dementia. This paper presents suggested research priorities as a starting point to build this evidence base. An interdisciplinary approach to research and priority setting is essential to develop actionable knowledge in this area.

Collaboration between the neurologist and palliative care team in the care of patients with severe demyelinating disease can result in improved patient care, and discussion of the complex ethical issues that arise when a patient expresses a wish to die may be rewarding for both patients and caregivers.

Background: People with intellectual disabilities often present with unique challenges that make it more difficult to meet their palliative care needs., Aim: To define consensus norms for palliative care of people with intellectual disabilities in Europe., Design: Delphi study in four rounds: (1) a taskforce of 12 experts from seven European countries drafted the norms, based on available empirical knowledge and regional/national guidelines; (2) using an online survey, 34 experts from 18 European countries evaluated the draft norms, provided feedback and distributed the survey within their professional networks. Criteria for consensus were clearly defined; (3) modifications and recommendations were made by the taskforce; and (4) the European Association for Palliative Care reviewed and approved the final version., Setting and Participants: Taskforce members: identified through international networking strategies. Expert panel: a purposive sample identified through taskforce members' networks., Results: A total of 80 experts from 15 European countries evaluated 52 items within the following 13 norms: equity of access, communication, recognising the need for palliative care, assessment of total needs, symptom management, end-of-life decision making, involving those who matter, collaboration, support for family/carers, preparing for death, bereavement support, education/training and developing/managing services. None of the items scored less than 86% agreement, making a further round unnecessary. In light of respondents' comments, several items were modified and one item was deleted., Conclusion: This White Paper presents the first guidance for clinical practice, policy and research related to palliative care for people with intellectual disabilities based on evidence and European consensus, setting a benchmark for changes in policy and practice., (© The Author(s) 2015.)

Background: Non-invasive ventilation (NIV) is beneficial for respiratory failure in motor neurone disease (MND) but some patients may wish to stop the intervention. Guidance from the National Institute for Health and Care Excellence recommends that research is needed on NIV withdrawal. There is little in the literature focusing on the issues doctors face when withdrawing NIV in this group., Aim: To identify issues and challenges that palliative medicine doctors encounter in relation to the withdrawal of NIV in MND patients., Method: An electronic questionnaire was sent to members of the Association of Palliative Medicine of Great Britain and Ireland. Participants rated how practically, emotionally and ethically challenging they found the process of NIV withdrawal., Results: 76 doctors responding had been directly involved in withdrawal of NIV at the request of a patient with MND. A high percentage rated the practical, ethical and emotional challenges as 7 or more on a 0-10 scale. Thematic analysis of the free text revealed some common difficulties. Lack of guidance on practical aspects of withdrawal, poor advance care planning and the need to support all involved to prevent conflict were recurrent themes. Statements relating to the emotional burden were diverse but suggest many palliative care doctors feel significant personal impact., Conclusions: The withdrawal of NIV in patients with MND appears to pose considerable challenges to palliative medicine doctors; emotionally, practically and to a lesser extent ethically. Development of guidelines and a clear ethical statement of conduct may help but emotional issues appear more complex.

Death anxiety may interfere with health care workers' (HCWs) relationships with patients and patients' families and increase HCWs' levels of burnout. This study shows the impact of a six-day course for HCWs that provided training in communication, in offering emotional and spiritual support to patients, and in personal introspection on death anxiety. The HCWs were given questionnaires to evaluate their level of burnout, personal well-being, and death anxiety as well as the quality of their relationships with patients before the course and four months after it. There were 150 study participants, all HCWs involved in caring for dying patients (85 in palliative care units and 65 in other settings). There was a control group of 26 HCWs who cared for the dying in settings other than palliative care units. The results show that the course appeared to lead to a significant reduction in levels of burnout and death anxiety; they also indicated an increase in personal well-being and professional fulfillment, and participants perceived an improvement in the quality of their relationships with patients and patients' families.

Interventions, such as the use of percutaneous endoscopic gastrostomy (PEG) and non-invasive ventilation (NIV), are used in the management of people with motor neurone disease with the aim of improving quality of life and relieving symptoms. However, the number of people receiving these interventions varies across the UK. This study has looked at the involvement and knowledge of consultants, within specialist palliative care services, with these procedures, to ascertain if there were differences in attitudes to their use. Twenty-two consultants took part in a telephone audit. There appeared to be great variation in their involvement in and knowledge of the use of these interventions. The majority of services were involved in the care of people with MND, but often only in the terminal stages. There appears to be a need for the wider application of guidelines on the use of PEG and NIV, as well as the development of a collaborative approach with other services, including neurology and rehabilitation services.

Background: Amyotrophic lateral sclerosis (ALS) is a relentlessly progressive neurodegenerative condition. Optimal management requires a palliative approach from diagnosis with emphasis on patient autonomy, dignity and quality of life., Objective: To conduct a systematic analysis of the type, level and timing of specialist palliative care intervention in ALS., Results: Despite an international consensus that ALS management should adopt a multidisciplinary approach, integration of palliative care into ALS management varies considerably across health care systems. Late referral to palliative services in ALS is not uncommon and may impact negatively on the quality of life of ALS patients and their caregivers. However, common themes and principles of engagement can be identified across different jurisdictions, and measurement systems have been established that can assess the impact of palliative care intervention., Conclusions: There is considerable evidence that palliative care intervention improves quality of life in patients and carers. International consensus guidelines would assist in the development of a framework for active palliative care engagement in ALS and other neurodegenerative diseases.

Our objective was to study the use of opioid and other medication at the end of life for patients with ALS/MND under specialist palliative care. A retrospective study looked at the medication received by 62 patients with MND/ALS in the last 72 h of life in six hospices in the UK and Ireland. Medication is widely used in the last 24 h of life, and use of the parenteral route increases as death approaches. We found that the doses of opioids and other medication do not increase appreciably during this period. The mean dose of opioid in the last 24 h of life was 80 mg oral morphine equivalent/24 h. These results are further evidence that opioids can be used both effectively and safely to manage symptoms at the end of life for people with MND/ALS.

The interdisciplinary team approach is essential in the assessment and management of the palliative care of patients and their families. An innovative approach has been developed to allow the interdisciplinary team to see patients and their families in an outpatient clinic setting. This has allowed an improved assessment and has demonstrated to patients that a wider approach is undertaken. These interdisciplinary outpatient clinics have been audited and appear to be effective, and acceptable to patients and families.

Good communication among the members of the multidisciplinary team is essential in the provision of specialist palliative care. It allows the patient's and family's care to be coordinated and all team members to be involved in planning care. In multidisciplinary team meetings at the Wisdom Hospice in the UK, a pro forma has been used with an overhead projector to show details of the patient, their family, history and problems. An audit of team members' views found the pro forma to be effective and useful in encouraging interdisciplinary workings.

To ascertain the involvement of palliative care with neurology services in the care of people with amyotrophic lateral sclerosis (ALS) in the United Kingdom, Italy and Switzerland, in particular the collaboration with and referral from neurology, the involvement in multidisciplinary team care and in the respiratory support of ALS patients. In 2019, two online surveys were undertaken of palliative care specialists, using specialist groups of the European Academy of Neurology, European Association of Palliative Care and the Association of Palliative Medicine for Great Britain and Ireland. The respondents were specialist palliative care professionals, predominantly senior doctors, involved in the care of people with ALS. As the numbers of respondents from many countries were in single figures the analysis was restricted to the United Kingdom, Italy and Switzerland. The time of involvement varied, with early involvement commonest in the UK. Barriers to referral included neurologists not referring and financial issues, particularly in Switzerland. The reluctance of patients and families to see palliative care services was reported as less than 20% in all countries. Respondents were often involved in the care of people receiving noninvasive ventilation (NIV), in all countries. and with tracheostomy ventilation (TV), particularly in Italy. Palliative care services are often involved in the care of people with ALS, but the extent and timing of involvement varies. The use of clinical guidelines and education on palliative care for neurology services may encourage collaboration, for the benefit of people with ALS and their families. [ABSTRACT FROM AUTHOR]

Introduction: The development of palliative care across the World is varied and often limited. The purpose of this comparative study was to explore healthcare professionals’ (doctors, nurses, and healthcare workers) understanding of palliative care in several different countries. Patients and methods: Eight focus groups were held in eight different countries using open-ended semi-structured questions that allowed participants to report on their understanding and experience of palliative care, the referral to services and the difficulties in providing and accessing palliative care. Transcriptions were subjected to thematic analysis. Results: Sixty people in total participated in focus groups held across eight countries. The main themes derived from the data included: definitions of palliative care; place of death and last care; and barriers to providing palliative care. Knowledge and understanding of palliative care vary across countries and between professionals and family carers. Cultural attitudes around death and dying in some countries appear to impact the introduction, availability, and use of palliative care services. The place of death of people with palliative care needs may also be influenced by resources and cultural norms. Limitations: The participants were self–selected and may not be representative of the wider views within each country. Conclusions: Increased educational interventions about palliative care for both professionals and the public would help improve palliative care provision, taking into account the social and cultural norms within communities. [ABSTRACT FROM AUTHOR]

Palliative care, neurology, amyotrophic lateral sclerosis (ALS), Parkinson's disease, stroke, glioma, communication, assisted dying Palliative care was shown to have a major role in the support of patients and families, with specialist palliative care being integrated early into care planning. Keywords: palliative care; neurology; amyotrophic lateral sclerosis (ALS); Parkinson's disease; stroke; glioma; communication; assisted dying EN palliative care neurology amyotrophic lateral sclerosis (ALS) Parkinson's disease stroke glioma communication assisted dying 1 3 3 02/18/22 20220216 NES 220216 Palliative care for people with neurological disease has become more accepted over the years, and there is increasing literature on this area of care. [Extracted from the article]

There is increasing collaboration between palliative care and neurology, intending to improve the quality of care and quality of life of people with neurological disease and their families. [ABSTRACT FROM AUTHOR]

Initially, the focus was on amyotrophic lateral sclerosis (ALS) which "represents a paradigm for palliative care in neurological diseases" ([4]). More recently a palliative care approach also found its way to diseases such as high-grade glioma of which the course is also relentlessly progressive like in ALS, Parkinson's disease (PD), and multiple sclerosis (MS). They found that neurological symptoms were common, both in patients with neurological diseases and other patients receiving palliative care. Although in the past palliative care for neurological patients has tended to focus on ALS, and to a lesser extent PD and MS, there is increasing awareness of the role for other disease groups. [Extracted from the article]

Background: The use of specific triggers has been suggested to help identify patients with progressive neurological disease who would benefit from palliative care. Aim: This study aimed to improve the evidence base for the use of triggers for patients with progressive neurological disease. Design: An evaluation of palliative care services was undertaken using a retrospective case note review of the timing and presence of triggers in the last 2 years of life. Setting/participants: A total of 12 specialist palliative care units across the United Kingdom provided data from 300 patients: mean patient age 70 years, 50% male, diagnoses included motor neurone disease 58%, Parkinson's disease 17% and Parkinson's Plus syndromes 12%. Results: There was a high burden of triggers - 17 in the last 2 years of life and 10 in the last 6 months of life. The most frequent triggers were deteriorating physical function, complex symptoms and dysphagia. Four factors were found to explain 64% of the total variance: Factor 1 - Deterioration in physical function, dysphagia, significant complex symptoms and pain; Factor 2 - Weight loss and respiratory symptoms; Factor 3 - Recurrent infections and cognitive decline; Factor 4 - Aspiration pneumonia. Cox regression analyses found different triggers were associated with survival from diagnosis versus survival from referral to palliative care. Different triggers were also associated with survival for different neurological conditions. Conclusion: This study demonstrates that there is a high burden of triggers in the last months and years of life and that these could potentially be reduced to fewer components. Prospective studies assessing which triggers are useful for different conditions are now required. [ABSTRACT FROM AUTHOR]

Palliative care is developing for people with neurological disease. This may be integrated early in the disease progression, and may have a variable input over time. The careful assessment and management of all issues — physical, psychological, social and spiritual — provides the patient and family the opportunity to maximise quality of life. This requires a multidisciplinary approach and close collaboration with other services. [ABSTRACT FROM AUTHOR]

The care of elderly people with progressive neurodegenerative disease is often complex and palliative care may be helpful from soon after diagnosis to enable the quality of life to be maintained as much as possible. Palliative care may be involved in an episodic way, as new problems and issues are faced, throughout the disease progression. There are many issues which require careful assessment, including symptoms, psychological, and social aspects of care. This may include the assessment of cognitive change and ensuring that symptoms and issues are addressed correctly, even if the person cannot communicate or is cognitively compromised. This care will also include carers, spouses and families, professional carers, and carers within residential and nursing homes. Patients and carers may need ongoing support, to cope with the multiple and continual losses faced within neurological disease progression, with the complex interaction with other comorbidities and the effects of ageing. As the progression continues the recognition of end of life, through the assessment of triggers, is important so that all can be prepared for dying and death. [ABSTRACT FROM AUTHOR]

A pathway has been developed using a multidisciplinary group from within specialist palliative care to ensure a comprehensive approach to the insertion of gastromy tubes for patients with motor neurone disease (MND) with swallowing difficulties. The pathway has ensured that there is a coordinated approach and the professionals involved are clear as to their responsibilities in the discussion and planning of the insertion, ensuring the best support for the patient and family. [ABSTRACT FROM AUTHOR]

Palliative care has a very important role in the care of patients with motor neurone disease and their families. There is increasing emphasis on the multidisciplinary assessment and support of patients within guidelines, supported by research. This includes the telling of the diagnosis, the assessment and management of symptoms, consideration of interventions, such as gastrostomy and ventilatory support, and care at the end of life. The aim of palliative care is to enable patients, and their families, to maintain as good a quality of life as possible and helping to ensure a peaceful death. [ABSTRACT FROM AUTHOR]