Table 1.
Guideline recommendations and recent developments.
| EFNS | AAN | NICE | Recent developments (level of evidence) |
|---|---|---|---|
| Initiation of NIV | |||
| Clinical signs or FVC < 80% or SNIP < 40 cm H2O or MIP max < 60 mm H2O or nocturnal desaturation or pCO2 > 45 mmHg |
Orthopnea or FVC < 50% or SNIP < 40 cm H2O or MIP < −60 cm or nocturnal desaturation |
FVC < 50% or FVC < 80% + orthopnea or SNIP/MIP < 40 cm H2O or SNIP/MIP < 65 cm H2O (men), <40 cm H2O (women) + orthopnea orSNIP/MIP −10 cm H2O over 3 months |
Consider NIV in patients with FVC > 80% and in asymptomatic patients (Ib) Use of capnometry/polygraphy to detect early sleep disturbance (III): (1) nocturnal hypercapnia (2) nocturnal desaturation (3) sleep apnea |
| NIV in patients with severe bulbar involvement and frontotemporal symptoms | |||
| Patients with bulbar palsy are less compliant with NIV, due in part to increased secretions NIV improves quality of life and prolongs survival in patients presenting with respiratory insufficiency, although this has not been confirmed in patients with bulbar-onset disease. |
Bulbar involvement and executive dysfunction: possibly lower compliance with NIV Noncompliance was seen in 75% of patients with ALS and frontotemporal dysfunction No survival benefit was seen in patients with poor bulbar function |
Before a decision is made on the use of NIV for a person with a diagnosis of FTD, the multidisciplinary team, together with the respiratory ventilation service, should carry out an assessment that includes the person’s capacity to make decisions and to give consent, the severity of dementia and cognitive problems, whether the person is likely to accept treatment, whether the person is likely to achieve improvements in sleep-related symptoms or behavioral improvements, a discussion with the person’s family or carers (with the person’s consent if they have the capacity to give it) | Bulbar involvement possibly lowers compliance, but consequent symptomatic treatment of secretions can improve NIV acceptance (IV) Several retrospective studies with large numbers of subjects showed a survival benefit for bulbar patients (although less pronounced than in patients without bulbar involvement) (III) Neurobehavioral deficits in patients who do not fulfill the criteria for FTD do not significantly influence the patients’ decisions regarding life-prolonging measures (III) |
| Management of hypersalivation | |||
| Amitriptyline 10 mg 3 times a day Atropine drops 0.5–1% 3–4 ×/d Glycopyrrolate Transdermal scopolamine 1.5 mg every third day Botulinum toxin A/B injected into salivary glands Irradiation of salivary glands |
– | Advice on swallowing, diet, posture, positioning, oral care and suctioning Antimuscarinic medicine Glycopyrrolate Botulinum toxin A (second line) |
– |
| Management of tenacious bronchial secretions | |||
| N-acetylcysteine 200–400 mg/d*
Beta-receptor antagonists + nebulizer (saline, anticholinergic bronchodilators, mucolytic, furosemide)* Manual-assisted cough MI-E Portable home suction device Room humidifier |
MI-E possibly effective HFCWO unproven |
Humidification, nebulizers, carbocisteine* Manual-assisted cough Breath stacking Volume recruitment bag MI-E device |
Growing evidence for MI-E, but still no high-class evidence studies (IV) HFCWO probably ineffective (IV) |
| PEG and respiratory function/NIV | |||
| To minimize risks, PEG should be performed before vital capacity falls below 50% of predicted NIV during PEG procedure may be feasible in patients with respiratory impairment |
FVC > 50%: low risk FVC 30–50%: moderate risk FVC < 30%: high risk The risk of PEG placement increased when the FVC declined below 50% of predicted |
– | PEG insertion under NIV is safe, even in patients with FVC < 50% (III) Establish NIV prior to PEG, use NIV during PEG placement, use minimal sedation (IV) |
| Diaphragm pacing | |||
| The use of diaphragmatic pacing or respiratory exercises in ALS is not established | – | – | Diaphragm pacing is contraindicated in ALS (Ib) |
| Indication criteria of IV/transition from NIV to IV | |||
| Severe bulbar weakness or NIV intolerance or declines NIV: propose invasive mechanical ventilation IV has a major impact upon caregivers and should be initiated only after informed discussion Unplanned (emergency) IV should be avoided through an early discussion of end-of-life issues, coordination with palliative care teams and appropriate advance directives |
NIV not tolerated: further education regarding documented benefits; evaluate reasons for noncompliance; reintroduce NIV; if not successful: hospice referral for palliative care or IV Unable to maintain pO2 > 90%, pCO2 < 50 mmHg or unable to manage secretions: IV IV may be considered to preserve quality of life in patients with ALS who want long-term ventilatory support |
– | – |
| Termination of NIV/IV, end-of-life management | |||
| Discuss the options for respiratory support and end-of-life issues if the patient has dyspnea, other symptoms of hypoventilation or an FVC < 50% Rediscuss the patient’s preferences for life-sustaining treatment every 6 months Treatment of dyspnea: opioids alone or in combination with benzodiazepines if anxiety is present Use oxygen only if hypoxia is present |
– | If a person on continuous NIV wishes to stop treatment, seek advice from healthcare professionals who have knowledge and experience of stopping NIV | If termination of IV is legal in the patient’s country, the advance directive should include whether ventilation should be terminated under certain circumstances, since the patient will eventually lose the ability to communicate (IV) |
*
In our experience, mucolytic drugs are usually ineffective and may even be harmful if not combined with MI-E.
AAN, American Academy of Neurology; ALS, amyotrophic lateral sclerosis; EFNS, European Federation of Neurological Societies; FTD, frontotemporal dementia; FVC, forced vital capacity; HFCWO, high-frequency chest-wall oscillation; IV, invasive ventilation; (IV), class IV evidence; MI-E, mechanical insufflation–exsufflation; MIP, maximal inspiratory pressure; NICE, National Institute for Health and Care Excellence; NIV, non-invasive ventilation, pCO2, carbon dioxide partial pressure; pO2, oxygen partial pressure; PEG, percutaneous endoscopic gastrostomy; SNIP, sniff nasal inspiratory pressure.