Motor neurone disease (MND) is a progressive and ultimately fatal disease that results in degeneration of the motor neurones, or nerves, in the brain and spinal cord.
GPs and others in the primary care team can make a great difference to the quality of life of people with MND, with timely care, support and symptom management.
NICE guideline on MND
For guidance on best practice in MND care, see NICE guidance 42:
Below is a selection of other resources that may be useful for GPs, covering the following topics:
This is not an exhaustive list and is intended for guidance. Please contact us if you have any suggestions for this section.
Management of MND
Orrell RW. GPs have key role in managing motor neurone disease. Practitioner. 2011 Sep; 255(1743):19-22.
Howard, R.S. et al. Management of motor neurone disease. Postgrad Med J. 2002. Vol 78. 736-41.
Leigh, P.N. et al. The management of motor neurone disease. J Neurol Neurosurg Psychiatry. 2003. Vol 74. (Supp iv). iv32-iv47.
Talbot, K. Motor neurone disease. Postgrad Med J. 2002. Vol 78. 513 –519.
Turner, M. The treatment of motor neurone disease. The Practitioner. 2001. Vol 245. 530-539.
Parton, M. J. et al. Motor neurone disease and its management. Journal of the RCP London. 1999. Vol 33 (3). 212-218.
Miller, R.G.et al. Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: Drug, nutritional and respiratory therapies (an evidence based review). Neurology 2009. Vol 73. 1218-1226.
Correction to above article printed in Neurology Vol 73 (24) Dec 15 2009 2134 and Neurology Vol 74 (9) Mar 2010 781.
Miller, R.G.et al. Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: Multidisciplinary care, symptom management and cognitive/behavioural impairment (an evidence based review). Neurology 2009. Vol 73. 1227-1233.
Corr, B. et al. Service provision for patients with ALS/MND: a cost effective multidisciplinary approach. Journal of the Neurological Sciences. 1998 Vol 160 (Supp 1) S141-S145.
Brown, J.B. User, carer and professional experiences of care in motor neurone disease.
Primary Health Care Research and Development. 2003. Vol 4. 207-217.
Skelton, J. Nursing role in the multidisciplinary management of motor neurone disease.
British Journal of Nursing. 2005. Vol 14. (1). 20-24.
Morrison, K.E. Managing motor neurone disease. The Practitioner. 2005. Vol 249. 612-620.
Bradley, W.G. et al. Changes in the management of ALS since the publication of the AAN ALS
practice parameter 1999. ALS and other motor neuron disorders. 2004. Vol 5. 240-244.
Bradley, W.G et al. Current management of ALS. Neurology. 2001. Vol 57. 500-504.
Turner-Stokes, L et al. From diagnosis to death: exploring the interface between neurology, rehabilitation and palliative care in managing people with long-term neurological conditions. Clinical Medicine. 2007. Vol 7 No 2 129-136.
Beghi E, et al. The heterogeneity of amyotrophic lateral sclerosis: a possible explanation of treatment failure.Curr Med Chem. 2007;14(30):3185-200. Review.
Valdmanis PN, et al. Genetics of familial amyotrophic lateral sclerosis. Neurology. 2008 Jan 8;70(2):144-52. Review.
Piepers, S et al. Evidence–based care in amyotrophic lateral sclerosis. Lancet Neurology. 2006 Feb (5) 2: 105-106.
Ray, R.A et al. Non-finite loss and emotional labour: family caregivers’ experiences of living with motor neurone disease. Journal of Clinical Nursing 2007 16 (3a) 35-43.
Hughes, R.A et al. Living with motor neurone disease: lives, experiences of services and suggestions for change. Health and Social Care in the Community 2005 Jan 13 (1), 64–74.
Brettschneider, J et al. Drug therapy for pain in amyotrophic lateral sclerosis or motor neuron disease. Cochrane Database of Systematic Reviews, 2013 Jun 5 (6).
Radunovic, A et al. Clinical care of patients with amyotrophic lateral sclerosis. Lancet Neurology 2007 6 913-925.
Talbot K. Motor neurone disease. Pract Neurol 2009 Oct 9 (5) 303-9.
Talbot K et al. Motor neurone disease: a practice manual. Oxford University Press 2010 (Book).
Talbot K et al. Motor neurone disease:the facts. Oxford University Press 2008 (Book).
Quality of life
Norquist, J.M. et al. Factors which predict physical and mental health status in patients with
amyotrophic lateral sclerosis over time. ALS and other motor neuron disorders. 2003. Vol 4. 112-117.
Kaub-Wittemer, D et al. Quality of life and psychosocial issues in ventilated patients with amyotrophic
lateral sclerosis and their caregivers. Journal of Pain and Symptom Management. 2003. Vol 26. 890-896.
Bourke, S.C. et al. Non-invasive ventilation in ALS. Indications and effect on quality of life. Neurology. 2003. Vol 61. 171-177.
Goldstein, L.H. et al. Correlates of quality of life in people with motor neurone disease. (MND). ALS and other motor neuron disorders. 2002. Vol 3. 123-129.
Atkins, L. et al. Correlates of distress in caregivers of people with motor neurone disease.
British Psychological Society, proceedings. 2002. Vol 10. 53
Aboussouan, L.S. et al. Objective measures of the efficacy of non-invasive positive pressure ventilation in amyotrophic lateral sclerosis. Muscle & Nerve. 2001. Vol 24. 403-409.
Bolmsjo, I. Existential issues in palliative care: interview of patients with amyotrophic lateral sclerosis. Journal of Palliative Medicine. 2001. Vol 4 499-505.
Lee, J.N. et al. Quality of life issues in motor neurone disease: the development and validation of a coping strategies questionnaire, the MND Coping Scale. Journal of the Neurological Sciences. 2001. Vol 191. 79-85.
Bromberg M.B. Quality of life in amyotrophic lateral sclerosis. Phys Med Rehabil Clin N Am. 2008 Aug;19(3):591-605. Review.
Wasner, M. et al. Sexuality in patients with amyotrophic lateral sclerosis and their partners. Journal of Neurol. 2004. Vol 251. 445-448.
Goldstein, L.H. et al. The psychological impact of MND on patients and carers. Journal of the Neurological Sciences. 1998. Vol 160 (Supp1) S106-S113).
Moore, M.J. et al. Mood disturbances in motor neurone disease. Journal of the Neurological Sciences. 1998. Vol 160 (Supp1) S53-S56.
Hogg, K. et al. The psychological impact of motor neurone disease. Psychological Medicine. 1994. Vol 24. 625 632.
Worthington, A. Psychological aspects of motor neurone disease. Clinical Rehabilitation.1996. Vol 10 185-194.
Mockford, Carole et al. (A Review) Carers, MND and service provision. ALS 2006 7 132-141.
Mcleod JE, et al. A review of psychosocial aspects of motor neurone disease. J Neurol Sci. 2007 Jul 15;258(1-2):4-10. Review.
Skelton, J. Dysphagia in motor neurone disease. Nursing Standard. 1996. Vol 10 (33). 49-56.
Kirker, F.J. et al. The development and implementation of a standardized policy for the management of dysphagia in motor neurone disease. Palliative Medicine. 2003 Vol 17. 322-326.
Hadjikoutis, S. et al. Coughing and choking in motor neurone disease. Journal Neurol Neurosurg Psychiarty. 2000. Vol 68. 601-604.
Worwood, A.M. et al. Indicators and prevalence of malnutrition in motor neurone disease. Eur Neurol. 1998. Vol 40 (3).159-63.
Heffernan, C. et al. Nutritional management in MND/ALS patients: an evidence based review. ALS and other motor neuron disorders. 2004. Vol 5. 72-83.
Chio, A. et al. Percutaneous radiological gastrostomy: a safe and effective method of nutritional tube placement in advanced ALS. J Neurol Neurosurg Psychiarty. 2004. Vol 75. 645-647.
Hussain, A. et al. The team approach to percutaneous endoscopic gastrostomy feeding. British Journal of Hospital Medicine. 1995 Vol 54 (7) 308-312.
Rosenfeld J. et al. Nutrition and dietary supplements in motor neuron disease. Phys Med Rehabil Clin N Am. 2008 Aug;19(3):573-89. Review.
Misra,V.P. Botulinum toxin as a treatment for drooling of saliva. ACNR. 2002. Vol 2 (5). 11-12.
Pal, P.K. et al. Botulinum toxin A as treatment for drooling saliva in PD. Neurology. 2000. Vol 54. 244-247.
Harriman, M et al. Use of radiotherapy for control of sialorrhea in patients with amyotrophic lateral sclerosis. The Journal of Otolaryngology. 2001 Vol 30. 242-245.
Tysnes O.B. Treatment of sialorrhea in amyotrophic lateral sclerosis. Acta Neurol Scand Suppl. 2008;188:77-81. Review.
Polkey, M.I et al. Ethical and clinical issues in the use of home non-invasive mechanical ventilation for the palliation of breathlessness in motor neurone disease. Thorax. 1999. Vol 54 367-371.
King’s MND Care and Research Centre. Non-invasive positive pressure ventilation – guidelines.
Bradley, M.D. et al. Outcome of ventilatory support for acute respiratory failure in motor neurone disease. J Neurol Neurosurg Psychiatry. 2002. Vol 72. 752-756.
Gelanis, D.F. Respiratory failure or impairment in amyotrophic lateral sclerosis. Current Treatment Options in Neurology. 2001. Vol 3. 133-138.
Lyall, R.A. et al. Respiratory muscle strength and ventilatory failure in amyotrophic lateral sclerosis. Brain. 2001. Vol 124. 2000-2013.
Oppenheimer, E.A. Treating respiratory failure in ALS: the details are becoming clearer. Journal of the Neurological Sciences. 2003. Vol 209.1-4.
Shee, C.D. et al. Non-invasive ventilation and palliation: experience in a district general hospital and a review. Palliative Medicine. 2003. Vol 17. 21-26.
Bourke, Stephen. C. et al. Effects of non invasive ventilation on survival and quality of life in patients with
amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurology. 2006. Vol 5 (2). 140-147.
Heffernan, C et al. Management of respiration in MND/ALS patients: An evidence based review. Amyotrophic Lateral Sclerosis 2006. Vol 7. 5-15.
Tripodoro, V.A, et al. Management of dyspnea in advanced motor neurone diseases. Curr Opin Support Palliat Care. 2008 Sep;2(3):173-9. Review.
Eng, D. Management guidelines for motor neurone disease patients on non-invasive ventilation at home. Palliat Med. 2006 Mar;20(2):69-79.
Radunovic A et al. Mechanical ventilation for amyotrophic lateral sclerosis/motor neurone disease Cochrane Database of Systematic Reviews 2009 (4).
OIiver, D. Palliative care for motor neurone disease. Practical Neurology. 2002. Vol 2. 68-79.
Neudert, C. et al. The course of the terminal phase in patients with amyotrophic lateral sclerosis. J Neurol. 2001. Vol 248. 612-616.
Oliver, D. et al. The involvement of specialist palliative care in the care of people with motor neurone disease. Palliative Medicine. 2000. Vol 14. 427-428.
Loftus, L.A. A collaborative nursing model for advanced non-malignant disease. International Journal of Palliative Nursing. 2000. Vol 6 (9). 454-458.
Shaw, P. Top 100: motor neurone disease. General Practitioner. 20 February 1998. 6062.
Barby, T.F.M. et al. Palliative care in motor neurone disease. International Journal of Palliative Nursing. 1995 Vol 1 (4). 183-188.
Gray, J. et al. Teamwork is the key in motor neurone disease care. MIMS Magazine. 1993. Vol 20 (34). 16-20.
Oliver, D. et al. Palliative care in amyotrophic lateral sclerosis. Oxford University Press 2000 (Book).
Oliver, D. et al. Palliative care in amyotrophic lateral sclerosis: from diagnosis to bereavement. Oxford University Press 2006 (Book).
Addington-Hall, J. et al. Specialist palliative care in non-malignant disease. Palliative Medicine.1998. Vol 12. 417-427
Hughes, R. et al. Assessing palliative care outcomes for people with motor neurone disease living at home. International Journal of Palliative Nursing. 2004. Vol 10. (9). 449-453.
McCluskey L. Amyotrophic Lateral Sclerosis: ethical issues from diagnosis to end of life. NeuroRehabilitation. 2007;22(6):463-72. Review.
End of life decisions
Ganzini, L. et al. The final month of life in patients with ALS. Neurology. 2002. Vol 59. 428-431.
Benditt, J.O. et al. Empowering the individual with ALS at the end of life. Disease specific advance care planning. Muscle & Nerve. 2001. Vol 24. 1706-1709.
Quill,T.E. Initiating end of life discussions with seriously ill patients – addressing the “Elephant in the room.” JAMA. 2000. Vol 284 (19). 2502-2507.
Griffith, R. Living wills, duty of care and the right to treatment. British Journal of Community Nursing. 2004. Vol 9. (11). 488-91.
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