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Amyotrophic lateral sclerosis

What's new

Updated 2023 ACCP guidelines for respiratory management in patients with neuromuscular disorders.

Background

Overview

Definition
ALS is a progressive neurological disorder characterized by degeneration of upper motor and lower motor neurons.
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Pathophysiology
ALS is familial (10%) and sporadic (90%) in origin. The pathological hallmark of ALS is the presence of inclusion bodies (aggregation of cytoplasmic proteins) in motor neurons.
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Epidemiology
In the US, the estimated prevalence of ALS is 5.2 persons per 100,000 persons.
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Disease course
Spinal motor neuron degeneration results in muscular paralysis, including respiratory muscle paralysis. Motor neuron degeneration can also occur in the frontal cortex, causing frontotemporal degeneration and dementia.
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Prognosis and risk of recurrence
The overall survival of patients with ALS is approximately 3 years after the time of diagnosis.
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Guidelines

Key sources

The following summarized guidelines for the evaluation and management of amyotrophic lateral sclerosis are prepared by our editorial team based on guidelines from the American College of Chest Physicians (ACCP 2023), the Infectious Diseases Society of America (IDSA/ACR/AAN 2021), the Amyotrophic Lateral Sclerosis Society of Canada (ALSSC 2020), the European Federation of Neurological Societies (EFNS 2012), and the American Academy ...
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Screening and diagnosis

Diagnosis
As per ALSSC 2020 guidelines:
Confirm the diagnosis of ALS by a neurologist or physical medicine and rehabilitation specialist with training and expertise in ALS.
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Refer patients with an initial diagnosis of ALS to an ALS specialty clinic for confirmation of diagnosis within 4 weeks.
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Revised El Escorial diagnostic criteria for amyotrophic lateral sclerosis
Presence of:
Evidence of lower motor neuron degeneration by clinical, electrophysiological, or neuropathologic examination
Evidence of upper motor neuron degeneration by clinical examination
Progressive spread of symptoms or signs within a region or to other regions, as determined by history or examination
Absence of:
Electrophysiological or pathological evidence of other disease processes that might explain the signs of lower motor neuron and/or upper motor neuron degeneration
Neuroimaging evidence of other disease processes that might explain the observed clinical and electrophysiological signs
Diagnostic criteria are not met
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  • Differential diagnosis

  • Communication of diagnosis

Diagnostic investigations

History and physical examination: as per EFNS 2012 guidelines, obtain prompt detailed clinical and paraclinical evaluations in all patients with suspected ALS. Consider obtaining additional investigations in selected patients, and repeating investigations if initial tests are equivocal.
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  • Evaluation of respiratory function

  • Cognitive evaluation

  • Genetic testing

  • Screening for venous thrombosis

Respiratory support

Supplemental oxygen: as per ALSSC 2020 guidelines, do not administer oxygen routinely for chronic respiratory insufficiency. Assess the etiology of the hypoxia and consider administering supplemental oxygen if hypoxemia remains after optimal noninvasive ventilation pressure is provided.
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  • Noninvasive ventilation

  • Invasive ventilation

  • Weaning from ventilatory support

Medical management

General principles: as per ALSSC 2020 guidelines, refer patients with ALS to specialized ALS multidisciplinary clinics for optimized healthcare delivery.
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  • Riluzole

  • Edaravone

  • Management of sialorrhea (pharmacotherapy)

  • Management of sialorrhea (oral suction)

  • Management of sialorrhea (botulinum toxin injection)

  • Management of sialorrhea (radiotherapy)

  • Management of sialorrhea (surgery)

  • Management of dyspnea

  • Management of excessive bronchial secretions (manually assisted cough)

  • Management of excessive bronchial secretions (lung volume recruitment)

  • Management of excessive bronchial secretions (mechanical insufflation-exsufflation)

  • Management of excessive bronchial secretions (high-frequency chest wall oscillation)

  • Management of excessive bronchial secretions (home suction device and room humidifier)

  • Management of excessive bronchial secretions (pharmacotherapy)

  • Management of pseudobulbar affect

  • Management of fasciculations

  • Management of spasticity

  • Management of muscle cramps

  • Management of dysarthria

  • Management of depression and anxiety

  • Management of insomnia and fatigue

  • Management of cognitive impairment

  • Management of venous thrombosis

  • Therapies with no evidence for benefit

  • Palliative care

  • End-of-life care

Nonpharmacologic interventions

Exercise: as per ALSSC 2020 guidelines, consider advising regular moderate-intensity exercise for improved function and QoL in the early phases of ALS.
C
consider offering a personalized exercise program, including strength with submaximal effort for resistance and aerobic training.
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  • Nutritional support

Patient education

General counseling: as per ALSSC 2020 guidelines, discuss treatment options and research opportunities in patients with ALS.
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Follow-up and surveillance

Indications for referral: as per EFNS 2012 guidelines, refer patients with suspected ALS to an experienced neurologist.
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  • Follow-up