AAN Practice parameter guidance
20091,2 / EFNS/EALSC Working Group Good practice points3 / MND Association evidence-based recommendations4,5
Enteral nutrition
Enteral nutrition via PEG for maintenance of nutrition / Enteral nutrition via PEG should be considered to stabilize body weight / PEG is the standard procedure for enteral nutrition in ALS / PEG helps to stabilize weight, combat malnutrition and is a suitable means for long-term nutritional maintenance
Timing of gastrostomy / Insufficient data exists to support or refute specific timing of PEG insertion in patients with ALS / An individual approach should be utilized taking into account bulbar symptoms, malnutrition (weight loss >10%), respiratory function and the patient's general condition. Early operation is highly recommended / An individualized approach to the timing of PEG is advocated. The patient should be informed that PEG might not be applicable later on in the disease progression
Alternatives to PEG in patients with poor respiratory function / Palliative NGT is recommended for feeding of high-risk patients (FVC <30%) and patients who have declined PEG / · RIG is a suitable alternative when PEG is deemed hazardous
· NGT can be used for short-term feeding and when PEG or RIG is not suitable / · RIG should be used when patients have poor respiratory function
· NGT can be considered for short-term feeding and when PEG or RIG is not suitable
PEG and prolonging survival / PEG should be considered for prolonging survival in patients with ALS / There is no convincing evidence that PEG prolongs survival / Randomized controlled trials are recommended
PEG and QOL / Insufficient data exists to support or refute PEG for improving QOL in patients with ALS / There is no convincing evidence that PEG improves QOL / Randomized controlled trials are recommended
Respiratory management
Optimal pulmonary tests to detect respiratory insufficiency / · Nocturnal oximetry should be considered for monitoring hypoventilation (regardless of the FVC)
· Supine FVC and MIP could be useful in routine respiratory monitoring, in addition to the erect FVC
· SNP should be considered to detect hypercapnia and nocturnal hypoxemia / · VC should be considered for respiratory monitoring on a regular basis (if possible measured both standing/sitting and lying)
· SNP should be considered for monitoring of inspiratory muscle strength, particularly in bulbar patients who cannot perform VC accurately
· Nocturnal oximetry is recommended for patients with symptoms suggestive of nocturnal hypoventilation / · Check for symptoms and signs suggestive of respiratory insufficiency
· Spirometry (especially SNIP) is useful in detecting early respiratory deficiency and predicting the course of respiratory failure
· For patients with severe bulbar disease who cannot perform respiratory function tests, use signs of respiratory insufficiency and measure arterial blood gases (including bicarbonate)
· Measure arterial blood gases and perform overnight oximetry in any patient with symptoms suggestive of nocturnal hypoventilation
Criteria for initiating NIV treatment / Orthopnea or SNP <40cm H20 or MIP <-60 cm H2O or abnormal nocturnal oximetry or FVC <50% / At least one of the following: (a) dyspnoea, (b) orthopnea, (c) disturbed sleep not due to pain, (d) morning headache, (e) poor concentration, (f) loss of appetite
(g) excessive daytime sleepiness (ESS>9) and signs of respiratory muscle weakness (FVC <80% or SNP <40cm H2O) and evidence of either: significant nocturnal desaturation on overnight oximetry, or morning blood-gas PCO2 >6.5 Kpa / FVC <50% (or even <70%) or serum chloride <98 mEq/1 or signs of diaphragmatic weakness on CXR. For patients with moderate or severe bulbar symptoms, invasive rather than noninvasive ventilation should be considered
NIV, respiratory function and survival / NIV should be considered to treat respiratory insufficiency in ALS, both to prolong survival and to slow the rate of FVC decline / · NIV and TIV can prolong survival for many months and can improve patients' QOL
· NIV should be considered before TIV in patients with symptoms of respiratory insufficiency / Use noninvasive ventilation or invasive ventilation for alleviating breathlessness
Use NIPPV or bi-level positive airway pressure ventilators to provide noninvasive ventilation, depending upon local availability and expertise
Assisted ventilation and QOL / · NIV can be considered to enhance QOL in patients with ALS who have respiratory insufficiency
· TIV should be considered to preserve QOL in patients with ALS who want long-term ventilatory support / NIV and TIV can prolong survival for many months and can improve patients' QOL / · NIPPV and bi-level positive airway pressure ventilators have been shown to improve QOL and survival in MND/ALS patients
· Invasive ventilation improves QOL and survival for patients and should be considered for all appropriate patients, on the basis of a fully informed decision by the patient and carer
Improving compliance with NIV / NIV should be considered at the earliest sign of nocturnal hypoventilation or respiratory insufficiency / Not addressed / Not addressed
Targeted respiratory interventions for clearing secretions / · MIE should be considered to clear secretions in patients with ALS who have reduced peak cough flow (<270L/min)
· Insufficient data exists to support or refute HFCWO for clearing airway secretions in patients with ALS / · Teach the patient and carers manual assisted cough
· Provide a portable home suction device and a room humidifier
· Consider using a mucolytic, for example N-acetylcysteine, 200–400mg three times daily
· If these measures are insufficient, try a nebulizer with saline and a β-receptor antagonist and/or an anticholinergic bronchodilator and/or a mucolytic and/or furosemide in combination
· The use of a MIE might be helpful, particularly in the setting of an acute respiratory infection
· Cricopharyngeal myotomy might be helpful in rare cases when frequent episodes of cricopharyngeal spasm occur with severe bronchial secretions / Not addressed
Use of invasive ventilation via tracheostomy in patients with ALS / Consider invasive ventilation of patients on NIV who are unable to maintain pO2>90%, or pCO2 <50mmHg or who are unable to manage bronchial secretions / Consider using invasive ventilation via tracheostomy in patients with severe bulbar dysfunction who are not tolerant of NIV and whose respiratory symptoms decline on NIV (pCO2<50mmHg; pO2 = 65mmHg and FiO2 <30mmHg for at least 10 hours) / Consider invasive ventilation when noninvasive ventilation is not appropriate due to bulbar symptoms and when noninvasive ventilation is no longer effective due to progression of disease
Withdrawal of NIV or invasive ventilation in ALS / · Insufficient data exists to support or refute specific strategies for withdrawal of ventilation in ALS
· Protocols based on consensus for withdrawal of mechanical ventilation in intensive care units include counseling and symptom control with opioids, benzodiazepines and anticholinergic medications / · Parenteral diamorphine, a benzodiazepine and an antiemetic should be used when the patient decides that ventilatory support should be withdrawn
· For symptomatic treatment of dyspnea with opioids and/or oxygen, no controlled studies in ALS exist / · Advance directives are essential and should be reviewed periodically
· Since a mentally competent and informed patient has the right to refuse or discontinue any treatment including life support and mechanical ventilation, those responsible for care must not prolong ventilation against the patient's will
· If withdrawal is requested, appropriate treatment should be continued to ensure that symptoms such as anxiety, distress and discomfort are managed effectively
Abbreviations: AAN, American Academy of Neurology; ALS, amyotrophic lateral sclerosis; CXR, chest X-ray; EALSC, European ALS Consortium; EFNS, European Federation of Neurological Societies; ESS, Epworth sleepiness scale; FiO2, Fraction of inspired Oxygen; FVC, forced vital capacity; HFCWO, high frequency chest wall oscillation; MIE, mechanical insufflation–exsufflation; MIP, maximal inspiratory pressure; MND, motor neuron disease; NGT, nasogastric tube; NIPPV, noninvasive positive pressure ventilation; NIV, non-invasive ventilation; PEG, percutaneous endoscopic gastrostomy; QOL, quality of life; RIG, radiologically inserted gastrostomy; SNIP, sniff nasal inspiratory pressure; SNP, sniff nasal pressure; TIV, tracheostomy invasive ventilation.VC, vital capacity
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2. 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): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 73, 1227–1233 (2009).
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4. Heffernan, C. et al. Nutritional management in MND/ALS patients: an evidence based review. Amyotroph. Lateral Scler. Other Motor Neuron Disord. 5, 72–83 (2004).
5. Heffernan, C. et al. Management of respiration in MND/ALS patients: An evidence based review. Amyotrophic Lateral Sclerosis 7, 5–15 (2006).