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  • Review Article
  • Published:

Supportive and symptomatic management of amyotrophic lateral sclerosis

Key Points

  • 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

Abstract

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.

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Figure 1: Recommended components of a multidisciplinary team for management of amyotrophic lateral sclerosis.
Figure 2: The Sheffield Support Snood.
Figure 3: MyNIV: An online guide for people with amyotrophic lateral sclerosis who use noninvasive ventilation.
Figure 4: A noninvasive ventilation mask.
Figure 5: A lung recruitment device.

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Acknowledgements

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.

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E.V.H. and C.J.M. were both involved in all aspects of manuscript preparation.

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Correspondence to Christopher J. McDermott.

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Competing interests

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).

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Glossary

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.

Euthanasia

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.

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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

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