The aim of the management of ALS is to maximize quality of life and minimize morbidity
Guidelines for symptomatic care are becoming more evidenced-based
Management by a specialist, clinic-based multidisciplinary team is associated with improved survival
In patients with respiratory failure, noninvasive ventilation prolongs survival and improves quality of life
Nutritional status is an independent predictor of survival and, if patients opt for enteral feeding, early gastrostomy insertion is recommended before significant weight loss takes place
Cognitive impairment is common in ALS and is associated with a worse prognosis; the optimal management of this problem is unclear
The main aims in the care of individuals with amyotrophic lateral sclerosis (ALS) are to minimize morbidity and maximize quality of life. Although no cure exists for ALS, supportive and symptomatic care provided by a specialist multidisciplinary team can improve survival. The basis for supportive management is shifting from expert consensus guidelines towards an evidence-based approach, which encourages the use of effective treatments and could reduce the risk of harm caused by ineffective or unsafe interventions. For example, respiratory support using noninvasive ventilation has been demonstrated to improve survival and quality of life, whereas evidence supporting other respiratory interventions is insufficient. Increasing evidence implicates a causal role for metabolic dysfunction in ALS, suggesting that optimizing nutrition could improve quality of life and survival. The high incidence of cognitive dysfunction and its impact on prognosis is increasingly recognized, although evidence for effective treatments is lacking. A variety of strategies are used to manage the other physical and psychological symptoms, the majority of which have yet to be thoroughly evaluated. The need for specialist palliative care throughout the disease is increasingly recognized. This Review describes the current approaches to symptomatic and supportive care in ALS and outlines the current guidance and evidence for these strategies.
Subscribe to Journal
Get full journal access for 1 year
only $4.92 per issue
All prices are NET prices.
VAT will be added later in the checkout.
Tax calculation will be finalised during checkout.
Rent or Buy article
Get time limited or full article access on ReadCube.
All prices are NET prices.
Alonso, A., Logroscino, G., Jick, S. S. & Hernán, M. A. Incidence and lifetime risk of motor neuron disease in the United Kingdom: a population-based study. Eur. J. Neurol. 16, 745–751 (2009).
Roche, J. C. et al. A proposed staging system for amyotrophic lateral sclerosis. Brain 135, 847–852 (2012).
Balendra, R. et al. Use of clinical staging in amyotrophic lateral sclerosis for phase 3 clinical trials. J. Neurol. Neurosurg. Psychiatry 86, 45–49 (2015).
Del Aguila, M. A., Longstreth, W. T., McGuire, V., Koepsell, T. D. & van Belle, G. Prognosis in amyotrophic lateral sclerosis. A population-based study. Neurology 60, 813–819 (2003).
Bensimon, G., Lacomblez, L. & Meininger, V. A controlled trial of riluzole in amyotrophic lateral sclerosis. ALS/Riluzole Study Group. N. Engl. J. Med. 330, 585–591 (1994).
Miller, R. G., Mitchell, J. D. & Moore, D. H. Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). Cochrane Database Syst. Rev. Issue 3 Art No.: CD001447 (2013).
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 73, 1218–2134 (2009).
Miller, R. G. et al. Practice Parameter update: The care of the patient with amyotrophic lateral sclerosis: Multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review). Neurology 73, 1227–1233 (2009).
Centre for Clinical Practice at NICE (UK). Motor neurone disease: the use of non-invasive ventilation. [online] https://www.nice.org.uk/guidance/cg105 (2010).
The EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis: et al. EFNS guidelines on the Clinical Management of Amyotrophic Lateral Sclerosis (MALS) - revised report of an EFNS task force. Eur. J. Neurol. 3, 360–375 (2011).
The Association for Palliative Medicine of Great Britain and Ireland. APM position on Withdrawal of Ventilatory Support for Respiratory Failure at the Request of an Adult Patient. [online] http://apmonline.org/wp-content/uploads/2015/05/Withdrawal-of-Ventilatory-Suport-for-Respiratory-Failure-1307.pdf (2013).
National Institute for Clinical Excellence, U. K. Motor neurone disease: assessment and management. NG521–47 [online] https://www.nice.org.uk/guidance/NG42 (2016).
Chio, A., Buffa, C., Mutani, R., Bora, G. & PARALS. Positive effects of tertiary centres for amyotrophic lateral sclerosis on outcome and use of hospital facilities. J. Neurol. Neurosurg. Psychiatry 77, 948–950 (2006).
Aridegbe, T. et al. The natural history of motor neuron disease: assessing the impact of specialist care. Amyotroph. Lateral Scler. Frontotemporal Degener. 14, 13–19 (2013).
Rooney, J. et al. A multidisciplinary clinic approach improves survival in ALS: a comparative study of ALS in Ireland and Northern Ireland. J. Neurol. Neurosurg. Psychiatry 86, 496–501 (2015).
Van den Berg, J. P. et al. Multidisciplinary ALS care improves quality of life in patients with ALS. Neurology 65, 1264–1267 (2005).
O'Brien, M., Jack, B. & Douglas Mitchell, J. Multidisciplinary team working in motor neurone disease: patient and family carer views. Br. J. Neurosci. Nurs. 7, 580–585 (2011).
Hogden, A., Greenfield, D., Nugus, P. & Kiernan, M. C. What influences patient decision-making in amyotrophic lateral sclerosis multidisciplinary care? A study of patient perspectives. Patient Prefer. Adherence 6, 829–838 (2012).
Hobson, E. V. et al. Using technology to improve access to specialist care in amyotrophic lateral sclerosis: A systematic review. Amyotroph. Lateral Scler. Frontotemporal Degener. 17, 313–324 (2016).
Baldinger, R., Katzberg, H. D. & Weber, M. Treatment cramps amyotroph. lateral sclerosis/motor neuron disease. Cochrane Database Syst. Rev. Issue 4 Art No.: CD004157 (2012).
Federal Register Online Drug Products Containing Quinine; Enforcement Action Dates. [online] http://www.fda.gov/OHRMS/DOCKETS/98fr/06-9713.htm (2006).
El-Tawil, S. et al. Quinine Muscle Cramps. Cochrane Database Syst. Rev. 4, CD005044 (2015).
Bedlack, R. S., Pastula, D. M., Hawes, J. & Heydt, D. Open-label pilot trial of levetiracetam for cramps and spasticity in patients with motor neuron disease. Amyotroph. Lateral Scler. 10, 210–215 (2009).
Weiss, M. D. et al. A randomized trial of mexiletine in ALS. Neurology 86, 1474–1481 (2016).
Ashworth, N. L., Satkunam, L. E. & Deforge, D. Treatment for spasticity in amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst. Rev. 2, CD004156 (2012).
Kheder, A. & Nair, K. P. S. Spasticity: pathophysiology, evaluation and management. Pract. Neurol. 12, 289–298 (2012).
Drory, V. E., Goltsman, E., Reznik, J. G., Mosek, A. & Korczyn, A. D. The value of muscle exercise in patients with amyotrophic lateral sclerosis. J. Neurol. Sci. 191, 133–137 (2001).
Marquardt, G. & Lorenz, R. Intrathecal baclofen for intractable spasticity in amyotrophic lateral sclerosis. J. Neurol. 246, 619–620 (1999).
Marquardt, G. & Seifert, V. Use of intrathecal baclofen for treatment of spasticity in amyotrophic lateral sclerosis. J. Neurol. Neurosurg. Psychiatry 72, 275–276 (2002).
McClelland, S. et al. Intrathecal baclofen for spasticity-related pain in amyotrophic lateral sclerosis: efficacy and factors associated with pain relief. Muscle Nerve 37, 396–398 (2008).
Chio, A. et al. Pain in amyotrophic lateral sclerosis: a population-based controlled study. Eur. J. Neurol. 19, 551–555 (2011).
Brettschneider, J., Kurent, J. & Ludolph, A. Drug therapy for pain in amyotrophic lateral sclerosis or motor neuron disease. Cochrane Database Syst. Rev. 6, CD005226 (2013).
World Health Organization. Cancer pain relief: with a guide to opioid availability. [online] http://apps.who.int/iris/bitstream/10665/37896/1/9241544821.pdf (WHO 1996).
Oliver, D. J. et al. Medication in the last days of life for motor neuron disease/amyotrophic lateral sclerosis. Amyotroph. Lateral Scler. 11, 562–564 (2010).
Connolly, S., Galvin, M. & Hardiman, O. End-of-life management in patients with amyotrophic lateral sclerosis. Lancet Neurol. 14, 435–442 (2015).
Renton, A. E. et al. A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron 72, 257–268 (2011).
DeJesus-Hernandez, M. et al. Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron 72, 245–256 (2011).
Cooper-Knock, J. et al. Clinico-pathological features in amyotrophic lateral sclerosis with expansions in C9ORF72. Brain 135, 751–764 (2012).
Majounie, E. et al. Frequency of the C9orfF72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study. Lancet Neurol. 11, 323–330 (2012).
Gordon, P. H. et al. A prospective cohort study of neuropsychological test performance in ALS. Amyotroph. Lateral Scler. 11, 312–320 (2010).
Elamin, M. et al. Executive dysfunction is a negative prognostic indicator in patients with ALS without dementia. Neurology 76, 1263–1269 (2011).
Woolley, S. C. & Strong, M. J. Frontotemporal dysfunction and dementia in amyotrophic lateral sclerosis. Neurol. Clin. 33 1–20 (2016).
Abrahams, S., Newton, J., Niven, E., Foley, J. & Bak, T. H. Screening for cognition and behaviour changes in ALS. Amyotroph. Lateral Scler. Frontotemporal Degener. 15, 9–14 (2014).
McCluskey, L. et al. ALS-Plus syndrome: non-pyramidal features in a large ALS cohort. J. Neurol. Sci. 345, 118–124 (2014).
Gallagher, J. P. Pathologic laughter and crying in ALS: a search for their origin. Acta Neurol. Scand. 80, 114–117 (1989).
Brooks, B. R. et al. Treatment of pseudobulbar affect in ALS with dextromethorphan/quinidine. Neurology 63, 1364–1370 (2004).
Pioro, E. P. et al. Dextromethorphan plus ultra low-dose quinidine reduces pseudobulbar affect. Ann. Neurol. 68, 693–702 (2010).
Iannaccone, S. & Ferini-Strambi, L. Pharmacologic treatment of emotional lability. Clin. Neuropharmacol. 19, 532–535 (1996).
Szczudlik, A., Słowik, A. & Tomik, B. [The effect of amitriptyline on the pathological crying and other pseudobulbar signs]. Neurol. Neurochir. Pol. 29, 663–674 (1995).
Kurt, A., Nijboer, F., Matuz, T. & Kübler, A. Depression and anxiety in individuals with amyotrophic lateral sclerosis: epidemiology and management. CNS Drugs 21, 279–291 (2007).
Pizzimenti, A., Aragona, M., Onesti, E. & Inghilleri, M. Depression, pain and quality of life in patients with amyotrophic lateral sclerosis: a cross-sectional study. Funct. Neurol. 28, 115 (2013).
Gauthier, A. et al. A longitudinal study on quality of life and depression in ALS patient-caregiver couples. Neurology 68, 923–926 (2007).
Rabkin, J. G. et al. Modafinil treatment of fatigue in patients with ALS: a placebo-controlled study. Muscle Nerve 39, 297–303 (2009).
Mackenzie, L. et al. Communications Technology and Motor Neuron Disease: An Australian Survey of People With Motor Neuron Disease. JMIR Rehabil. Assist Technol. 3, e2 (2016).
Reed, H. et al. Head-Up; An interdisciplinary, participatory and co-design process informing the development of a novel head and neck support for people living with progressive neck muscle weakness. J. Med. Eng. Technol. 39, 404–410 (2014).
Baxter, S. et al. Evaluating a novel cervical orthosis, the Sheffield Support Snood, in patients with amyotrophic lateral sclerosis/motor neuron disease with neck weakness. Amyotroph. Lateral Scler. Frontotemporal Degener. 17, 436–442 (2016).
Fried-Oken, M. et al. Purposes of AAC device use for persons with ALS as reported by caregivers. Augment. Altern. Commun. 22, 209–221 (2006).
Hwang, C.-S., Weng, H.-H., Wang, L.-F., Tsai, C.-H. & Chang, H.-T. An eye-tracking assistive device improves the quality of life for ALS patients and reduces the caregivers' burden. J. Mot. Behav. 46, 233–238 (2014).
Mills, T., Bunnell, H. T. & Patel, R. Towards personalized speech synthesis for augmentative and alternative communication. Augment. Altern. Commun. 30, 226–236 (2014).
Marchetti, M. & Priftis, K. Brain-computer interfaces in amyotrophic lateral sclerosis: A metanalysis. Clin. Neurophysiol. 126, 1255–1263 (2015).
Desport, J. C. et al. Factors correlated with hypermetabolism in patients with amyotrophic lateral sclerosis. Am. J. Clin. Nutr. 74, 328–334 (2001).
Jawaid, A. et al. A decrease in body mass index is associated with faster progression of motor symptoms and shorter survival in ALS. Amyotroph. Lateral Scler. 11, 542–548 (2010).
Marin, B. et al. Alteration of nutritional status at diagnosis is a prognostic factor for survival of amyotrophic lateral sclerosis patients. J. Neurol. Neurosurg. Psychiatry 82, 628–634 (2011).
Paganoni, S., Deng, J., Jaffa, M., Cudkowicz, M. E. & Wills, A.-M. Body mass index, not dyslipidemia, is an independent predictor of survival in amyotrophic lateral sclerosis. Muscle Nerve 44, 20–24 (2011).
Genton, L., Viatte, V., Janssens, J. P., Héritier, A. C. & Pichard, C. Nutritional state, energy intakes and energy expenditure of amyotrophic lateral sclerosis (ALS) patients. Clin. Nutr. 30, 553–559 (2011).
Bouteloup, C. et al. Hypermetabolism in ALS patients: an early and persistent phenomenon. J. Neurol. 256, 1236–1242 (2009).
Funalot, B., Desport, J.-C., Sturtz, F., Camu, W. & Couratier, P. High metabolic level in patients with familial amyotrophic lateral sclerosis. Amyotroph. Lateral Scler. 10, 113–117 (2009).
Wills, A.-M. et al. Hypercaloric enteral nutrition in patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled phase 2 trial. Lancet 383, 2065–2072 (2014).
Plowman, E. K. Is There a Role for Exercise in the Management of Bulbar Dysfunction in Amyotrophic Lateral Sclerosis? J. Speech Lang. Hear. Res. 58, 1151–1166 (2015).
Plowman, E. K. et al. Impact of expiratory strength training in amyotrophic lateral sclerosis. Muscle Nerve 54, 48–53 (2016).
Plowman, E. K. et al. Autologous myoblasts attenuate atrophy and improve tongue force in a denervated tongue model: a pilot study. Laryngoscope 124, E20–E26 (2014).
Restivo, D. A. et al. ALS dysphagia pathophysiology: differential botulinum toxin response. Neurology 80, 616–620 (2013).
Stavroulakis, T., Walsh, T., Shaw, P. J. & McDermott, C. J., Progas Study. Gastrostomy use in motor neurone disease (MND): A review, meta-analysis and survey of current practice. Amyotroph. Lateral Scler. Frontotemporal Degener. 14, 96–104 (2013).
Stavroulakis, T. et al. The impact of gastrostomy in motor neurone disease: challenges and benefits from a patient and carer perspective. BMJ Support. Palliat. Care 6, 52–59 (2016).
Group, P. S. Gastrostomy in patients with amyotrophic lateral sclerosis (ProGas): a prospective cohort study. Lancet Neurol. 14, 702–700 (2015).
Stavroulakis, T. et al. Factors influencing decision-making in relation to timing of gastrostomy insertion in patients with motor neurone disease. BMJ Support. Palliat. Care 4, 57–63 (2013).
Johnson, J. et al. Eating-derived pleasure in amyotrophic lateral sclerosis as a predictor of non-oral feeding. Amyotroph. Lateral Scler. 13, 555–559 (2012).
National Institute for Clinical Excellence, UK. Dementia: supporting people with dementia and their carers in health and social care [online] https://www.nice.org.uk/guidance/cg42 (NICE 2014).
Bourke, S. 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 Neurol. 5, 140–147 (2006).
Shefner, J. M., Watson, M. L., Meng, L. & Wolff, A. A. The Neals/Cytokinetics STUDY Team. A study to evaluate safety and tolerability of repeated doses of tirasemtiv in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener Amyotroph. Lateral. Scler. Frontotemporal Degener. 14, 574–581 (2013).
Orion Corporation, Orion Pharma. Effects of ODM-109 on Respiratory Function in Patients With Amyotrophic Lateral Sclerosis (ALS). Clinicaltrials.gov [online] https://clinicaltrials.gov/ct2/show/NCT02487407.
Carratù, P. et al. Early treatment with noninvasive positive pressure ventilation prolongs survival in Amyotrophic Lateral Sclerosis patients with nocturnal respiratory insufficiency. Orphanet J. Rare Dis. 4, 10 (2009).
Terzano, C. & Romani, S. Early use of non invasive ventilation in patients with amyotrophic lateral sclerosis: what benefits? Eur. Rev. Med. Pharmacol. Sci. 19, 4304–4313 (2015).
O'Neill, C. L. et al. Non-invasive ventilation in motor neuron disease: an update of current UK practice. J. Neurol. Neurosurg. Psychiatry 83, 371–376 (2012).
Pinto, A., de Carvalho, M., Evangelista, T., Lopes, A. & Sales- Luís, L. Nocturnal pulse oximetry: a new approach to establish the appropriate time for non-invasive ventilation in ALS patients. Amyotroph. Lateral Scler. Other Motor Neuron. Disord. 4, 31–35 (2003).
Rafiq, M. K. et al. Using transcutaneous carbon dioxide monitor (TOSCA 500) to detect respiratory failure in patients with amyotrophic lateral sclerosis: A validation study. Amyotroph. Lateral Scler. 13, 528–532 (2012).
Baxter, S. K. et al. The initiation of non-invasive ventilation for patients with motor neuron disease: patient and carer perceptions of obstacles and outcomes. Amyotroph. Lateral Scler. Frontotemporal Degener. 14, 105–110 (2013).
Baxter, S. K. et al. The impact on the family carer of motor neurone disease and intervention with noninvasive ventilation. J. Palliat. Med. 16, 1602–1609 (2013).
Gonzalez-Bermejo, J. et al. Prognostic value of efficiently correcting nocturnal desaturations after one month of non-invasive ventilation in amyotrophic lateral sclerosis: a retrospective monocentre observational cohort study. Amyotroph. Lateral Scler. Frontotemporal Degener. 14, 373–379 (2013).
Berlowitz, D. J. et al. Identifying who will benefit from non-invasive ventilation in amyotrophic lateral sclerosis/motor neurone disease in a clinical cohort. J. Neurol. Neurosurg. Psychiatry 87, 280–286 (2016).
Hobson, E. V. et al. Management of sialorrhoea in motor neuron disease: a survey of current UK practice. Amyotroph. Lateral Scler. Frontotemporal Degener. 14, 521–527 (2013).
Jackson, C. E. et al. Randomized double-blind study of botulinum toxin type B for sialorrhea in als patients. Muscle Nerve 39, 137–143 (2009).
Barbero, P. et al. Long-term follow-up of ultrasound-guided botulinum toxin-A injections for sialorrhea in neurological dysphagia. J. Neurol. 262, 2662–2667 (2015).
Dogu, O., Apaydin, D., Sevim, S., Talas, D. U. & Aral, M. Ultrasound-guided versus “blind” intraparotid injections of botulinum toxin-A for the treatment of sialorrhoea in patients with Parkinson's disease. Clin. Neurol. Neurosurg. 106, 93–96 (2004).
Meijer, J.-W. G., van Kuijk, A. A., Geurts, A. C. H., Schelhaas, H. J. & Zwarts, M. J. Acute deterioration of bulbar function after botulinum toxin treatment for sialorrhoea in amyotrophic lateral sclerosis. Am. J. Phys. Med. Rehabil. 87, 321–324 (2008).
Neppelberg, E., Haugen, D. F., Thorsen, L. & Tysnes, O.-B. Radiotherapy reduces sialorrhea in amyotrophic lateral sclerosis. Eur. J. Neurol. 14, 1373–1377 (2007).
Guy, N. et al. Comparison of radiotherapy types in the treatment of sialorrhea in amyotrophic lateral sclerosis. J. Palliat Med. 14, 391–395 (2011).
Suárez, A. A. et al. Peak flow and peak cough flow in the evaluation of expiratory muscle weakness and bulbar impairment in patients with neuromuscular disease. Am. J. Phys. Med. Rehabil. 81, 506–511 (2002).
Sancho, J., Servera, E., Díaz, J. & Marín, J. Predictors of ineffective cough during a chest infection in patients with stable amyotrophic lateral sclerosis. Am. J. Respir. Crit. Care Med. 175, 1266–1271 (2007).
Tzeng, A. C. & Bach, J. R. Prevention of pulmonary morbidity for patients with neuromuscular disease. Chest 118, 1390–1396 (2000).
Rafiq, M. K. et al. A preliminary randomized trial of the mechanical insufflator-exsufflator versus breath-stacking technique in patients with amyotrophic lateral sclerosis. Amyotroph. Lateral Scler. Frontotemporal Degener. 16, 448–455 (2015).
Lange, D. J. et al. High-frequency chest wall oscillation in ALS: an exploratory randomized, controlled trial. Neurology 67, 991–997 (2006).
DiPALS Writing Committee, on behalf of the DiPALS Study Group Collaborators. Safety and efficacy of diaphragm pacing in patients with respiratory insufficiency due to amyotrophic lateral sclerosis (DiPALS): a multicentre, open-label, randomised controlled trial. Lancet Neurol. 14, 883–892 (2015).
United States Food and Drug Administration. Safety and summary sheet of probably benefit. [online] http://www.accessdata.fda.gov/cdrh_docs/pdf10/H100006b.pdf
Assistance Publique - Hôpitaux de Paris. Early stage amyotrophic lateral sclerosis phrenic stimulation (RespiStimALS) Clinicaltrials.gov [online] https://clinicaltrials.gov/ct2/show/NCT01583088.
Barrow Neurological Institute. Diaphragm pacing system (DPS) in participants with amyotrophic lateral sclerosis (ALS) (DPS in ALS) Clinicaltrials.gov [online] https://clinicaltrials.gov/ct2/show/NCT01938495.
Synapse Biomedical. Humanitarian device exemption post-approval study of NeuRx diaphragm pacing system for amyotrophic lateral sclerosis. Clinicaltrials.gov [online] https://clinicaltrials.gov/ct2/show/NCT01605006
Whitehead, B., O'Brien, M. R., Jack, B. A. & Mitchell, D. Experiences of dying, death and bereavement in motor neurone disease: A qualitative study. Palliat. Med. 26, 368–378 (2012).
Aoun, S. M., Connors, S. L., Priddis, L., Breen, L. J. & Colyer, S. Motor Neurone Disease family carers' experiences of caring, palliative care and bereavement: an exploratory qualitative study. Palliat. Med. 26, 842–850 (2012).
Baxter, S. K. et al. The use of non-invasive ventilation at end of life in patients with motor neurone disease: A qualitative exploration of family carer and health professional experiences. Palliat. Med. 27, 516–523 (2013).
Phelps, K., Regen, E., Oliver, D., McDermott, C. & Faull, C. Withdrawal of ventilation at the patient's request in MND: a retrospective exploration of the ethical and legal issues that have arisen for doctors in the UK. BMJ Support. Palliat. Care. http://dx.doi.org/10.1136/bmjspcare-2014-000826 (2015).
National Clinical Guideline Centre. Care of Dying Adults in the Last Days of Life. NG31 [online] www.nice.org.uk/guidance/ng31?unlid=8981581202016219221856 (2015).
Motor Neurone Disease Association. MND Just in Case kit - information for professionals [online] http://www.mndassociation.org/forprofessionals/mndmanagement/mnd-just-in-case-kit/ (2015).
Sancho, J., Servera, E., Díaz, J. L., Bañuls, P. & Marín, J. Home tracheotomy mechanical ventilation in patients with amyotrophic lateral sclerosis: causes, complications and 1-year survival. Thorax 66, 948–952 (2011).
Spataro, R., Bono, V., Marchese, S. & La Bella, V. Tracheostomy mechanical ventilation in patients with amyotrophic lateral sclerosis: clinical features and survival analysis. J. Neurol. Sci. 323, 66–70 (2012).
Heritier Barras, A.-C. et al. Is tracheostomy still an option in amyotrophic lateral sclerosis? Reflections of a multidisciplinary work group. Swiss Med. Wkly. 143, w13830 (2013).
Hayashi, H. & Oppenheimer, E. A. ALS patients on TPPV: totally locked-in state, neurologic findings and ethical implications. Neurology 61, 135–137 (2003).
Motor Neurone Disease Association. Pathway for Preparing to Withdraw Non-Invasive Ventilation (NIV) in Patients with MND) [online] http://www.mndassociation.org/wp-content/uploads/2015/02/leicestershire-and-rutland-pathway-and-guidelines-for-withdrawing-niv.pdf
Peters, M., Jenkinson, C., Doll, H., Playford, E. D. & Fitzpatrick, R. Carer quality of life and experiences of health services: a cross-sectional survey across three neurological conditions. Health Qual. Life Outcomes 11, 1–1 (2013).
Goldstein, L. H., Atkins, L., Landau, S., Brown, R. & Leigh, P. N. Predictors of psychological distress in carers of people with amyotrophic lateral sclerosis: a longitudinal study. Psychol. Med. 36, 865–875 (2006).
O'Brien, M. R., Whitehead, B., Jack, B. A. & Mitchell, J. D. The need for support services for family carers of people with motor neurone disease (MND): views of current and former family caregivers a qualitative study. Disabil. Rehabil. 34, 247–256 (2012).
Craig, P. et al. Developing and evaluating complex interventions: the new Medical Research Council guidance. BMJ 337, a1655–a1655 (2008).
Bourke, S. C., Williams, T. L., Bullock, R. E., Gibson, G. J. & Shaw, P. J. Non-invasive ventilation in motor neuron disease: current UK practice. Amyotroph. Lateral Scler. Other Motor Neuron Disord. 3, 145–149 (2002).
Chio, A. et al. Non-invasive ventilation in amyotrophic lateral sclerosis: a 10 year population based study. J. Neurol. Neurosurg. Psychiatry 83, 377–381 (2012).
I've got nothing to lose by trying it. Weighing up claims about cures and treatments for medical conditions. Sense about Science. [online] www.senseaboutscience.org/data/files/resources/136/Ive-got-nothing-to-lose_web.pdf (2013).
Zettler, P. J. & Greely, H. T. The strange allure of state 'right-to-try' laws. JAMA Intern. Med. 174, 1885–1886 (2014).
Medical Innovation Bill [HL] 2014–2015. UK Parliament website. [online] http://services.parliament.uk/bills/2014-15/medicalinnovation.html
Wicks, P. et al. Subjects no more: what happens when trial participants realize they hold the power? BMJ 348, g368–g368 (2014).
Wicks, P. et al. Sharing Health Data for Better Outcomes on PatientsLikeMe. J. Med. Internet Res. 12, e19 (2010).
Stiggelbout, A. M. et al. Shared decision making: really putting patients at the centre of healthcare. BMJ 344, e256 (2012).
E.V.H. is funded by a National Institute for Health Research (NIHR) Doctoral Research Fellowship. C.J.M. receives funding from the NIHR, the Motor Neuron Disease Association and EU Joint Programme – Neurodegenerative Disease Research (JPND). The views expressed are those of the authors and not necessarily those of the NHS, the NIHR or the Department of Health.
E.V.H. and C.J.M. are investigators in a number of clinical studies of symptomatic treatments, interventions and drugs in amyotrophic lateral sclerosis. These include remote monitoring of noninvasive ventilation (receiving equipment from Phillips), and telehealth (funded by the National Institute for Health Research (NIHR), UK, and the Motor Neuron Disease Association, UK, in collaboration with Mylan) and a clinical trial of levosimendan (funded by Orion Pharma). C.J.M. has also been an investigator on clinical trials including diaphragmatic pacing (funded by the NIHR), the Sheffield Support Snood (funded by the NIHR), cough augmentation (funded by the Motor Neuron Disease Association UK), tirasemtiv (funded by Cytokinetics) and dexpramipexole (funded by Biogen Idec).
- Noninvasive ventilation (NIV)
The provision of bilevel inspiratory and experiatory ventilatory support through the patient's upper airway using a mask.
- Advanced planning
A formal decision-making process that aims to help patients establish decisions about future care
- Behavioural variant of FTD
The predominant symptoms of this disorder are executive dysfunction and behavioural changes.
- Environmental controls
Electronic assistive technology that enables people with significant disabilities to independently access equipment in their environment, such as home or hospital.
- Eye-gaze grids
A basic form of communication aid where a patient points to or looks at letters on an alphabet board in order to spell out words.
- Eye-gaze software
Computer software that tracks eye movements allowing the patient to navigate and control a computer by tracking where they are looking.
- Voice banking
A software records multiple examples of the patients voice to synthesize a voice similar to the patients' own, which can be used as a replacement when using communication software.
- Type 2 respiratory failure
Inadequate ventilation that causes hypercapnoea (as opposed to type 1 respiratory failure, which causes hypoxaemia) and, if severe, hypoxia; in ALS, respiratory failure is caused by neuromuscular weakness of respiratory muscles.
- Breath stacking
A technique to improve lung recruitment and cough: multiple inspiratory breaths are taken in succession and held (rather than exhaled) until full lung capacity is reached and exhalation is then allowed; the effectiveness of the process can be improved by using lung-recruitment bags.
With the patient's consent, a doctor acts directly to end the patient's life, for examply by administering a lethal injection.
- Refusal or withdrawal of treatment
At the patient's request, treatment can be declined or withdrawn, even if the lack of treatment has the potential to hasten the patient's death.
- Physician-assisted suicide
A doctor knowingly and intentionally provides a person with the knowledge, means, or both required to commit suicide, for example by counselling about lethal doses of drugs, prescribing such lethal doses or supplying the drugs.
About this article
Cite this article
Hobson, E., McDermott, C. Supportive and symptomatic management of amyotrophic lateral sclerosis. Nat Rev Neurol 12, 526–538 (2016). https://doi.org/10.1038/nrneurol.2016.111
BMC Medicine (2021)
Ageing Research Reviews (2021)
Neural Regeneration Research (2021)
Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration (2021)
Nature Reviews Neurology (2021)