Palliative care in amyotrophic lateral sclerosis: a review of current international guidelines and initiatives
Open accessAbstract
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.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
Patients with MND value others who are personable, reassuring, and remain calm.
“A further characteristic valued by patients was the “nature” of other's highlighting the value of individuals who were personable, reassuring and who remained calm (4, 6, 10).”
From Patients experiences of maintaining mental well-being and hope within motor neuron disease: a thematic synthesis (2015) · cites this paper as [6]
Patients with MND consistently highlight the need for choice and autonomy during interactions.
“Related to this, patients consistently highlighted the need for choice and autonomy given during an interaction (5, 6, 7, 9, 12, 14, 15, 20, 24, 25, 26, 28).”
From Patients experiences of maintaining mental well-being and hope within motor neuron disease: a thematic synthesis (2015) · cites this paper as [6]
Reassurance and trust from health care professionals are particularly important for end-of-life care for patients with MND.
“This was particularly important for end of life care (6, 11, 14, 28).”
From Patients experiences of maintaining mental well-being and hope within motor neuron disease: a thematic synthesis (2015) · cites this paper as [6]
Patients with MND cite the importance of reassurance and trust from health care professionals.
“Importantly, patients cited the importance of reassurance and trust from health care professionals (6, 9, 10, 14, 23, 28).”
From Patients experiences of maintaining mental well-being and hope within motor neuron disease: a thematic synthesis (2015) · cites this paper as [6]
Patients with MND use the strategy of focusing on what could be done or achieved rather than what they couldn't do or what they had lost.
“(c) Focusing on what could be done or achieved (2, 5, 6, 7, 11, 12, 13, 14, 27), rather than what they couldn't or what they had lost.”
From Patients experiences of maintaining mental well-being and hope within motor neuron disease: a thematic synthesis (2015) · cites this paper as [6]
Late referral to palliative services may negatively impact on the quality of life of patients with motor neuron disease.
“late referral to palliative services may negatively impact on the patients quality of life (Bede et al., 2011).”
From Patients experiences of maintaining mental well-being and hope within motor neuron disease: a thematic synthesis (2015) · cites this paper as Bede et al., 2011
Cites
Cohen, S. R., Mount, B. M., Strobel, M. G., & Bui, F. (1995) · Palliative Medicine · doi:10.1177/026921639500900306 · Cited by 737 · see citing works
Cognitive impairment in amyotrophic lateral sclerosis
Phukan, J., Pender, N. P., & Hardiman, O. M. (2007) · The Lancet Neurology · doi:10.1016/s1474-4422(07)70265-x · Cited by 610 · see citing works
Self-citationStrong, M. J., Grace, G. M., Freedman, M., Lomen-Hoerth, C., Woolley, S. C., Goldstein, L. H., Murphy, J. M., Shoesmith, C. L., Rosenfeld, J., Leigh, P. N., et al. (2009) · Amyotrophic Lateral Sclerosis · doi:10.1080/17482960802654364 · Cited by 543 · see citing works
Traynor, B. J. (2003) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.74.9.1258 · Cited by 493 · see citing works
Multidisciplinary ALS care improves quality of life in patients with ALS
den Berg, J. P. V., Kalmijn, S., Lindeman, E., Veldink, J. H., de Visser, M., van der Graaff, M. M., Wokke, J. H. J., & van den Berg, L. H. (2005) · Neurology · doi:10.1212/01.wnl.0000180717.29273.12 · Cited by 344 · see citing works
Andersen, P. M., Borasio, G. D., Dengler, R., Hardiman, O. M., Kollewe, K., Leigh, P. N., Pradat, P., Silani, V., & Tomik, B. (2005) · European Journal of Neurology · doi:10.1111/j.1468-1331.2005.01351.x · Cited by 290 · see citing works
Self-citationQuality of life in ALS depends on factors other than strength and physical function
Simmons, Z., Bremer, B. A., Robbins, R. A., Walsh, S. M., & Fischer, S. (2000) · Neurology · doi:10.1212/wnl.55.3.388 · Cited by 279 · see citing works
Self-citationThe effects of executive and behavioral dysfunction on the course of ALS
Olney, R. K., Murphy, J. M., Forshew, D. A., Garwood, E. R., Miller, B. L., Langmore, S. E., Kohn, M. A., & Lomen-Hoerth, C. (2005) · Neurology · doi:10.1212/01.wnl.0000188759.87240.8b · Cited by 258 · see citing works
Correlates of suffering in amyotrophic lateral sclerosis
Ganzini, L., Johnston, W. S., & Hoffman, W. F. (1999) · Neurology · doi:10.1212/wnl.52.7.1434 · Cited by 250 · see citing works
Regnard, C., Reynolds, J., Watson, B., et al. (2006) · Journal of Intellectual Disability Research · doi:10.1111/j.1365-2788.2006.00875.x · Cited by 169 · see citing works
Neudert, C., Wasner, M., & Borasio, G. D. (2004) · Journal of Palliative Medicine · doi:10.1089/jpm.2004.7.551 · Cited by 158 · see citing works
Self-citationNeurobehavioral symptoms in ALS are negatively related to caregivers’ burden and quality of life
Chiò, A., Vignola, A., Mastro, E., Giudici, A. D., Iazzolino, B., Calvo, A., Moglia, C., & Montuschi, A. (2010) · European Journal of Neurology · doi:10.1111/j.1468-1331.2010.03016.x · Cited by 157 · see citing works
The final month of life in patients with ALS
Ganzini, L. K., Johnston, W. S. W., & Silveira, M. J. (2002) · Neurology · doi:10.1212/wnl.59.3.428 · Cited by 149 · see citing works
The syndromes of frontotemporal dysfunction in amyotrophic lateral sclerosis
Strong, M. J. (2008) · Amyotrophic Lateral Sclerosis · doi:10.1080/17482960802372371 · Cited by 148 · see citing works
The Cornell-Brown Scale for Quality of Life in Dementia
Ready, R. E., Ott, B. R., Grace, J., & Fernandez, I. (2002) · Alzheimer Disease & Associated Disorders · doi:10.1097/00002093-200204000-00008 · Cited by 138 · see citing works
Simmons, Z., Felgoise, S. H., Bremer, B. A., Walsh, S., Hufford, D. J., Bromberg, M. B., David, W., Forshew, D. A., Heiman-Patterson, T. D., Lai, E. C., et al. (2006) · Neurology · doi:10.1212/01.wnl.0000242887.79115.19 · Cited by 128 · see citing works
Self-citationPalliative Care for Patients With Amyotrophic Lateral Sclerosis
Mitsumoto, H., & Rabkin, J. G. (2007) · JAMA · doi:10.1001/jama.298.2.207 · Cited by 126 · see citing works
Correlates of Quality of Life in people with motor neuron disease (MND)
Goldstein, L. H., Atkins, L., & Leigh, P. N. (2002) · Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders · doi:10.1080/146608202760834120 · Cited by 126 · see citing works
Borasio, G. D., Shaw, P. J., Hardiman, O. M., Ludolph, A., Luis, M. S., & Silani, V. (2001) · Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders · doi:10.1080/146608201753275517 · Cited by 118 · see citing works
Self-citationFegg, M. J., Kramer, M., L’hoste, S., & Borasio, G. D. (2008) · Journal of Pain and Symptom Management · doi:10.1016/j.jpainsymman.2007.05.007 · Cited by 100 · see citing works
Self-citationDementia Care Mapping (DCM): initial validation of DCM 8 in UK field trials
Brooker, D. J. R., & Surr, C. (2006) · International Journal of Geriatric Psychiatry · doi:10.1002/gps.1600 · Cited by 91 · see citing works
Some difficult decisions in ALS/MND
Oliver, D., & Turner, M. R. (2010) · Amyotrophic Lateral Sclerosis · doi:10.3109/17482968.2010.487532 · Cited by 87 · see citing works
Self-citationPromoting excellence in end‐of‐life care in ALS
Mitsumoto, H., Bromberg, M. B., Johnston, W. S. W., Tandan, R., Byock, I. R., Lyon, M., Miller, R. G., Appel, S. H., Benditt, J., Bernat, J. L., et al. (2005) · Amyotrophic Lateral Sclerosis · doi:10.1080/14660820510028647 · Cited by 85 · see citing works
Self-citationvan den Berg, J., de Groot, I. J., Joha, B., van Haelst, J., van Gorcom, P., & Kalmijn, S. (2004) · Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders · doi:10.1080/14660820410021258 · Cited by 83 · see citing works
Rabkin, J. G., Albert, S. M., Tider, T., Bene, M. L. D., O'Sullivan, I., Rowland, L. P., & Mitsumoto, H. (2006) · Amyotrophic Lateral Sclerosis · doi:10.1080/14660820500515021 · Cited by 66 · see citing works
A rapid screening battery to identify frontal dysfunction in patients with ALS
Flaherty-Craig, C. V., Eslinger, P. J., Stephens, B., & Simmons, Z. (2006) · Neurology · doi:10.1212/01.wnl.0000247667.89251.43 · Cited by 64 · see citing works
Self-citationO’Leary, N., & Tiernan, E. (2008) · Palliative Medicine · doi:10.1177/0269216307084609 · Cited by 62 · see citing works
Meaning in life in patients with amyotrophic lateral sclerosis
Fegg, M. J., Kögler, M., Brandstätter, M., Jox, R. J., Anneser, J. M. H., Haarmann-Doetkotte, S., Wasner, M., & Borasio, G. D. (2010) · Amyotrophic Lateral Sclerosis · doi:10.3109/17482961003692604 · Cited by 59 · see citing works
Self-citationPalliative care and circumstances of dying in German ALS patients using non‐invasive ventilation
Kühnlein, P., Kübler, A., Raubold, S., Worrell, M., Kurt, A., Gdynia, H., Sperfeld, A., & Ludolph, A. C. (2008) · Amyotrophic Lateral Sclerosis · doi:10.1080/17482960701830495 · Cited by 55 · see citing works
Are We Referring Patients to Hospice Too Late? Patients' and Families' Opinions
Kapo, J. M., Harrold, J. K., Carroll, J. T., et al. (2005) · Journal of Palliative Medicine · doi:10.1089/jpm.2005.8.521 · Cited by 51 · see citing works
Palliative Care in Amyotrophic Lateral Sclerosis, Parkinson's Disease, and Multiple Sclerosis
Elman, L. B., Houghton, D. J., Wu, G. F., Hurtig, H. I., Markowitz, C., & McCluskey, L. F. (2007) · Journal of Palliative Medicine · doi:10.1089/jpm.2006.9978 · Cited by 47 · see citing works
The involvement of specialist palliative care in the care of people with motor neurone disease
Oliver, D., & Webb, S. (2000) · Palliative Medicine · doi:10.1191/026921600701536264 · Cited by 36 · see citing works
Self-citationMeasuring psychological well-being in cognitively impaired persons
Burgener, S. C., Twigg, P., & Popovich, A. (2005) · Dementia · doi:10.1177/1471301205058303 · Cited by 36 · see citing works
McCluskey, L. F., & Houseman, G. (2004) · Journal of Palliative Medicine · doi:10.1089/109662104322737241 · Cited by 34 · see citing works
Measuring life quality, physical function and psychological well-being in neurological illness
O'Doherty, L., Hickey, A., & Hardiman, O. (2010) · Amyotrophic Lateral Sclerosis · doi:10.3109/17482960903552488 · Cited by 32 · see citing works
Self-citationGrief support for informal caregivers of patients with ALS: A national survey
Hebert, R. S., Lacomis, D., Easter, C. R., Frick, V., & Shear, M. K. (2005) · Neurology · doi:10.1212/01.wnl.0000148586.79290.a6 · Cited by 25 · see citing works
Models of care for motor neuron disease: setting standards
Hardiman, O. M., Traynor, B. J., Corr, B., & Frost, E. (2002) · Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders · doi:10.1080/146608202760839002 · Cited by 23 · see citing works
Self-citationControl of Symptoms: Cognitive Dysfunction
Goldstein, L. H. (2006) · Oxford University Press eBooks · doi:10.1093/acprof:oso/9780199212934.003.0008 · Cited by 10 · see citing works
Control of symptoms: cognitive dysfunction
Goldstein, L. H. (2014) · Oxford University Press eBooks · doi:10.1093/acprof:oso/9780199686025.003.0008 · Cited by 8 · see citing works
Flagged first, then by citations
Cited by
Sorted by citations
Amyotrophic lateral sclerosis and palliative care: where we are, and the road ahead
Blackhall, L. J. (2012) · Muscle & Nerve · doi:10.1002/mus.22305 · Cited by 88 · see citing works
Quality of life of patients with amyotrophic lateral sclerosis
Tramonti, F., Bongioanni, P., Di Bernardo, C., Davitti, S., & Rossi, B. (2012) · Psychology, Health & Medicine · doi:10.1080/13548506.2011.651149 · Cited by 47 · see citing works
Soundy, A., & Condon, N. (2015) · Frontiers in Psychology · doi:10.3389/fpsyg.2015.00606 · Cited by 35 · see citing works
D’Alvano, G., Buonanno, D., Passaniti, C., De Stefano, M., Lavorgna, L., Tedeschi, G., Siciliano, M., & Trojsi, F. (2021) · Brain Sciences · doi:10.3390/brainsci12010049 · Cited by 32 · see citing works
Antoniadi, A. M., Galvin, M., Heverin, M., Hardiman, O., & Mooney, C. (2021) · Proceedings of the 36th Annual ACM Symposium on Applied Computing · doi:10.1145/3412841.3441940 · Cited by 12 · see citing works
Self-citation
- Authors
- Bede, P., Oliver, D., Stodart, J., van den Berg, L., Simmons, Z., O Brannagain, D., Borasio, G. D., & Hardiman, O.
- Affiliations
- Trinity College Dublin (IE)
- University of Kent (GB)
- Wesley Hospital (AU)
- Pennsylvania State University (US)
- Ludwig-Maximilians-Universität München (DE)
- Corresponding author
- Bede, P.
- Venue
- Journal of Neurology, Neurosurgery & Psychiatry
- Publisher
- BMJ
- Published
- 2011-02-05
- Volume
- 82
- Issue
- 4
- Pages
- 413-418
- ISSN
- 0022-3050 (ISSN-L)
- OpenAlex
- W2311912039
- Cited by
- 123
- References
- 39
- Citations by year
- 2026: 3 · 2025: 4 · 2024: 3 · 2023: 10 · 2022: 7 · 2021: 10 · 2020: 7 · 2019: 18 · 2018: 6 · 2017: 8 · 2016: 10 · 2015: 12 · 2014: 6 · 2013: 5 · 2012: 9
- FWCI
- 7.85
- Type
- journal article
- Language
- en
- Open access
- green
- hal.science
- Licence
- other-oa
- Locations
- Journal of Neurology Neurosurgery & Psychiatry (publishedVersion)
- PubMed (publishedVersion)
- HAL (submittedVersion, other-oa)
- Open access LMU (Ludwid Maxmilian's Universitat Munchen) (submittedVersion)
- IRIS (submittedVersion)
- HighWire Press Open Archive (submittedVersion)
- SERVAL (Université de Lausanne) (submittedVersion)
- Trinity's Access to Research Output (TARA) (Trinity College Dublin) (submittedVersion)
- Lenus, The Irish Health Repository (Dr Steevens Hospital Library) (submittedVersion)
- Topics
- Health Sciences › Medicine › Public Health, Environmental and Occupational Health › Palliative Care and End-of-Life Issues
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Health Sciences › Medicine › Public Health, Environmental and Occupational Health › Palliative and Oncologic Care
- Keywords
- amyotrophic lateral sclerosis, palliative care, palliative care guidelines, multidisciplinary care, timing of palliative care referral, quality of life, caregiver burden, neurodegenerative diseases, patient autonomy
- MeSH terms
- Amyotrophic Lateral Sclerosis, Cognition Disorders, Consensus Statements as Topic, Humans, Palliative Care, Quality of Life, Caregivers, Practice Guidelines as Topic
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
