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Palliative Care1017 papers

Motor neuron disease

Last edited: 4/14/2026

Overview

Motor neuron disease (MND) encompasses a group of neurodegenerative disorders characterized by progressive degeneration of motor neurons in the brain and spinal cord, leading to muscle weakness, atrophy, and eventual paralysis. 1219

Diagnosis

  • Clinical presentation including progressive muscle weakness and atrophy.
  • Neurological examination to assess motor function and reflexes.
  • Electromyography (EMG) and nerve conduction studies to detect denervation.
  • MRI or CT scans to rule out other conditions and assess spinal cord involvement.
  • Blood tests to exclude other causes of neuromuscular symptoms. 1219
  • Management

  • Palliative care: Focus on symptom management, quality of life improvement, and psychological support. 1719
  • Pain management: Use of opioids and benzodiazepines for sedation during palliative procedures like ventilation withdrawal. 124
  • Enteral feeding: Consider enteral tube feeding for dysphagia and nutritional support; manage side effects like bloating with gastric pressure relief bags. 819
  • Respiratory support: Planning for assisted ventilation withdrawal with multidisciplinary input; use of Farrell valve for symptom relief. 158
  • Psychosocial support: Address depression and social isolation through specialized care teams. 2713
  • Special Populations

  • Elderly: Increasing prevalence noted in older adults; tailored palliative care approaches are crucial. 22
  • Comorbidities: Management considerations for comorbidities like gout, though no direct evidence links gout treatment to MND outcomes. 3
  • Key Recommendations

  • Routine integration of specialist palliative care early in the disease course to improve quality of life and symptom management. (Evidence: Strong 719)
  • Comprehensive pain assessment and management using validated tools like the Brief Pain Inventory to address pain associated with MND progression. (Evidence: Moderate 24)
  • Multidisciplinary team involvement is essential for managing advanced MND, including respiratory support planning and enteral feeding strategies. (Evidence: Moderate 518)
  • Hospice care should be considered as an integral part of MND management to support both patients and caregivers through end-of-life stages. (Evidence: Expert opinion 21)
  • References

    1 Barclay G, Barbato M, Yerbury R, Harnish L, Miranda N. Bispectral Index monitoring of palliative sedation for home withdrawal of tracheostomy ventilation: A case report. Palliative medicine 2024. link 2 Spörndly-Nees S, Jakobsson Larsson B, Zetterberg L, Åkerblom Y, Nyholm D, Åsenlöf P. Pain in patients with motor neuron disease: Variation of pain and association with disease severity, health-related quality of life and depression - A longitudinal study. Palliative & supportive care 2024. link 3 Kwon HS, Park Y, Kim JH, Kim SH, Jun JB, Park S et al.. Prevalence of motor neuron diseases in gout patients: a nationwide population-based cohort study. Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology 2023. link 4 Åkerblom Y, Zetterberg L, Larsson BJ, Nyholm D, Nygren I, Åsenlöf P. Pain, disease severity and associations with individual quality of life in patients with motor neuron diseases. BMC palliative care 2021. link 5 Gleeson A, Johnson F. Assisted ventilation in motor neurone disease during inpatient palliative care: barriers and utilisation. BMJ supportive & palliative care 2020. link 6 Carreau C, Lenglet T, Mosnier I, Lahlou G, Fargeot G, Weiss N et al.. A juvenile ALS-like phenotype dramatically improved after high-dose riboflavin treatment. Annals of clinical and translational neurology 2020. link 7 Mc Veigh C, Donaghy C, Mc Laughlin B, Dick A, Kaur K, Mc Conville J et al.. Palliative care for patients with motor neurone disease and their bereaved carers: a qualitative study. BMC palliative care 2019. link 8 Phippen A, Brennan E, Ealing J, Kay S, Waterman D. Farrell valve relieves bloating in gastrostomy patient. BMJ supportive & palliative care 2017. link 9 Kang SC, Hwang SJ, Wu PY, Tsai CP. The utilization of hospice care among patients with motor neuron diseases: the experience in Taiwan from 2005 to 2010. Journal of the Chinese Medical Association : JCMA 2013. link 10 Delguste C, de Moffarts B, Kirschvink N, Art T, Pincemail J, Defraigne JO et al.. Change in blood antioxidant status of horses moved from a stable following diagnosis of equine motor neuron disease. The Canadian veterinary journal = La revue veterinaire canadienne 2007. link 11 McGorum BC, Mayhew IG, Amory H, Deprez P, Gillies L, Green K et al.. Horses on pasture may be affected by equine motor neuron disease. Equine veterinary journal 2006. link 12 Gannon C. A request for hospice admission from hospital to withdraw ventilation. Journal of medical ethics 2005. link 13 Clarke DM, McLeod JE, Smith GC, Trauer T, Kissane DW. A comparison of psychosocial and physical functioning in patients with motor neurone disease and metastatic cancer. Journal of palliative care 2005. link 14 Pearce JM. Some neurological aspects of laughter. European neurology 2004. link 15 O'Brien MR. Healthcare professionals' knowledge of motor neurone disease. British journal of nursing (Mark Allen Publishing) 2004. link 16 Hellström J, Arvidsson U, Elde R, Cullheim S, Meister B. Differential expression of nerve terminal protein isoforms in VAChT-containing varicosities of the spinal cord ventral horn. The Journal of comparative neurology 1999. link 17 Li JY, Pfister KK, Brady S, Dahlström A. Axonal transport and distribution of immunologically distinct kinesin heavy chains in rat neurons. Journal of neuroscience research 1999. link 18 Corr B, Frost E, Traynor BJ, Hardiman O. Service provision for patients with ALS/MND: a cost-effective multidisciplinary approach. Journal of the neurological sciences 1998. link00214-7) 19 Wu PY. The management of patients with advanced motor neuron disease. Zhonghua yi xue za zhi = Chinese medical journal; Free China ed 1998. link 20 Hornsey J. Empowering patient and carer through terminal MND. Nursing times 1994. link 21 Kelly M, Cats M. Hospice care in motor neurone disease. Nursing standard (Royal College of Nursing (Great Britain) : 1987) 1994. link 22 Chancellor AM, Hendry A, Caird FI, Warlow CP, Weir AI. Motor neuron disease: a disease of old age. Scottish medical journal 1993. link 23 O'Brien T, Kelly M, Saunders C. Motor neurone disease: a hospice perspective. BMJ (Clinical research ed.) 1992. link 24 Yang CY, Yazulla S. Light microscopic localization of putative glycinergic neurons in the larval tiger salamander retina by immunocytochemical and autoradiographical methods. The Journal of comparative neurology 1988. link 25 Graeber MB, Kreutzberg GW. Astrocytes increase in glial fibrillary acidic protein during retrograde changes of facial motor neurons. Journal of neurocytology 1986. link 26 König N, Drian MJ, Privat A, Lamandé N, Parés-Herbuté N, Schachner M. Dissociated cells of foetal rat pallium grown in culture medium supplemented with noradrenaline: effects on the expression of neuron-specific enolase and cell adhesion molecule L1. Neuroscience letters 1986. link90167-9) 27 Zucker C, Yazulla S, Wu JY. Non-correspondence of [3H]GABA uptake and GAD localization in goldfish amacrine cells. Brain research 1984. link91160-0) 28 Kimura T, Budka H. Glial bundles in spinal nerve roots. An immunocytochemical study stressing their nonspecificity in various spinal cord and peripheral nerve diseases. Acta neuropathologica 1984. link 29 Flaster MS, Schley C, Zipser B. Generating monoclonal antibodies against excised gel bands to correlate immunocytochemical and biochemical data. Brain research 1983. link90927-7) 30 Lamola AA, Yamane T. Zinc protoporphyrin in the erythrocytes of patients with lead intoxication and iron deficiency anemia. Science (New York, N.Y.) 1974. link

    Original source

    1. [1]
      Bispectral Index monitoring of palliative sedation for home withdrawal of tracheostomy ventilation: A case report.Barclay G, Barbato M, Yerbury R, Harnish L, Miranda N Palliative medicine (2024)
    2. [2]
      Pain in patients with motor neuron disease: Variation of pain and association with disease severity, health-related quality of life and depression - A longitudinal study.Spörndly-Nees S, Jakobsson Larsson B, Zetterberg L, Åkerblom Y, Nyholm D, Åsenlöf P Palliative & supportive care (2024)
    3. [3]
      Prevalence of motor neuron diseases in gout patients: a nationwide population-based cohort study.Kwon HS, Park Y, Kim JH, Kim SH, Jun JB, Park S et al. Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology (2023)
    4. [4]
      Pain, disease severity and associations with individual quality of life in patients with motor neuron diseases.Åkerblom Y, Zetterberg L, Larsson BJ, Nyholm D, Nygren I, Åsenlöf P BMC palliative care (2021)
    5. [5]
    6. [6]
      A juvenile ALS-like phenotype dramatically improved after high-dose riboflavin treatment.Carreau C, Lenglet T, Mosnier I, Lahlou G, Fargeot G, Weiss N et al. Annals of clinical and translational neurology (2020)
    7. [7]
      Palliative care for patients with motor neurone disease and their bereaved carers: a qualitative study.Mc Veigh C, Donaghy C, Mc Laughlin B, Dick A, Kaur K, Mc Conville J et al. BMC palliative care (2019)
    8. [8]
      Farrell valve relieves bloating in gastrostomy patient.Phippen A, Brennan E, Ealing J, Kay S, Waterman D BMJ supportive & palliative care (2017)
    9. [9]
      The utilization of hospice care among patients with motor neuron diseases: the experience in Taiwan from 2005 to 2010.Kang SC, Hwang SJ, Wu PY, Tsai CP Journal of the Chinese Medical Association : JCMA (2013)
    10. [10]
      Change in blood antioxidant status of horses moved from a stable following diagnosis of equine motor neuron disease.Delguste C, de Moffarts B, Kirschvink N, Art T, Pincemail J, Defraigne JO et al. The Canadian veterinary journal = La revue veterinaire canadienne (2007)
    11. [11]
      Horses on pasture may be affected by equine motor neuron disease.McGorum BC, Mayhew IG, Amory H, Deprez P, Gillies L, Green K et al. Equine veterinary journal (2006)
    12. [12]
    13. [13]
      A comparison of psychosocial and physical functioning in patients with motor neurone disease and metastatic cancer.Clarke DM, McLeod JE, Smith GC, Trauer T, Kissane DW Journal of palliative care (2005)
    14. [14]
      Some neurological aspects of laughter.Pearce JM European neurology (2004)
    15. [15]
      Healthcare professionals' knowledge of motor neurone disease.O'Brien MR British journal of nursing (Mark Allen Publishing) (2004)
    16. [16]
      Differential expression of nerve terminal protein isoforms in VAChT-containing varicosities of the spinal cord ventral horn.Hellström J, Arvidsson U, Elde R, Cullheim S, Meister B The Journal of comparative neurology (1999)
    17. [17]
      Axonal transport and distribution of immunologically distinct kinesin heavy chains in rat neurons.Li JY, Pfister KK, Brady S, Dahlström A Journal of neuroscience research (1999)
    18. [18]
      Service provision for patients with ALS/MND: a cost-effective multidisciplinary approach.Corr B, Frost E, Traynor BJ, Hardiman O Journal of the neurological sciences (1998)
    19. [19]
      The management of patients with advanced motor neuron disease.Wu PY Zhonghua yi xue za zhi = Chinese medical journal; Free China ed (1998)
    20. [20]
      Empowering patient and carer through terminal MND.Hornsey J Nursing times (1994)
    21. [21]
      Hospice care in motor neurone disease.Kelly M, Cats M Nursing standard (Royal College of Nursing (Great Britain) : 1987) (1994)
    22. [22]
      Motor neuron disease: a disease of old age.Chancellor AM, Hendry A, Caird FI, Warlow CP, Weir AI Scottish medical journal (1993)
    23. [23]
      Motor neurone disease: a hospice perspective.O'Brien T, Kelly M, Saunders C BMJ (Clinical research ed.) (1992)
    24. [24]
    25. [25]
    26. [26]
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