Amyotrophic lateral sclerosis (ALS) - NYSORA

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Amyotrophic lateral sclerosis (ALS)

Learning objectives

  • Describe the pathophysiology of Amyotrophic lateral sclerosis (ALS)
  • Signs and symptoms of ALS
  • Anesthetic management of ALS

Definition and mechanisms

  • Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s Disease, is a rare and progressive nervous system disease that affects nerve cells in the brain and spinal cord
  • Resulting in the progressive loss of motor neurons that control voluntary muscles
  • ALS often begins with muscle twitching and weakness in a limb, or slurred speech
  • Eventually, ALS affects the control of the muscles needed to move, speak, eat, and breathe
  • The most common cause of death for patients with ALS is respiratory failure
  • On average, patients die within 3-5 years after symptoms begin
  • There is no cure for this fatal disease
  • Most ALS cases are considered sporadic, about 5-10% of all ALS cases are familial

Signs and symptoms

  • Difficulty walking or doing normal daily activities
  • Tripping and falling
  • Weakness in the legs, feet, or ankles
  • Hand weakness or clumsiness
  • Slurred speech or trouble swallowing
  • Muscle cramps and twitching (fasciculations) in the arms, shoulders, and tongue
  • Inappropriate crying, laughing, or yawning
  • Cognitive and behavioral changes
  • Pulmonary/breathing problems:
    • Shortness of breath
    • Weak cough
    • Difficulty clearing the throat and lungs
    • Extra saliva
    • Inability to lie flat in bed
    • Repeated chest infections and pneumonia
    • Respiratory failure

Symptoms with the progression of the disease:

  • Not be able to stand or walk, get in or out of bed on their own
  • Dysphagia
  • Dysarthria
  • Dyspnea 
  • Weight loss
  • Malnourishment
  • Muscle cramps and neuropathy 
  • Anxiety and depression
  • Dementia

Risk factors

  • Age: increases with age and is most commonly between 40 and 70
  • Gender: males are more likely to develop ALS before the age of 65
  • Race and ethnicity: Caucasians and non-Hispanics are most likely to develop the disease
  • Heredity: in 5-10% of the cases
  • Smoking
  • Environmental toxin exposure: lead, pesticides such as Aldrin, Dieldrin, and DDT
  • Military service


  • Breathing problems requiring BiPAP or tracheostomy
  • Difficulty with speaking
  • Malnutrition and dehydration
  • Dementia


  • EMG
  • A nerve conduction study
  • MRI
  • Blood and urine tests
  • Muscle biopsy


The goal of treatment is to improve symptoms:

  • Supportive health care: 
    • Physical therapy
    • Nutritional counseling
    • Speech therapy
    • Assistive devices: splints, braces, grab bars,…
  • Non-invasive ventilation
  • Medications to manage symptoms including muscle cramps, stiffness, excess saliva, phlegm, unwanted episodes of crying and/or laughing, or other emotional displays
  • FDA-approved medications:
    • Riluzole (Rilutek)
    • Edaravone (Radicava)

Anesthetic management

Preoperative and perioperative management

difficult airway, tracheotomy, video laryngoscopy, fiberoptic scope, bulbar dysfunction, aspiration, ventilatory depression, rhabdomyolysis, succinylcholine, TIVA, propofol, fentanyl, hypovolemia, lung protective ventilation, depolarizing neuromuscular blocking agents, rocuronium, opioids, lignocaine

Postoperative management

  • Transfer patient to ICU
  • Administer paracetamol or NSAIDs postoperatively for pain management
    • Avoid opioids because of respiratory depression
  • Respiratory distress may require prolonged mechanical ventilation as well as re-intubation
    • Be aware that weaning is often prolonged and difficult
  • Consider that bulbar symptoms like dysphagia or dysarthria as well as cognitive impairment may lead to malnutrition and require intravenous or tube feeding 

Suggested reading

  • Gaik C, Wiesmann T. 2021Anaesthesia recommendations for Amyotrophic lateral sclerosis. Orphananesthesia.
  • Sarna R, Gupta A, Arora G. Amyotrophic lateral sclerosis and anaesthetic challenges: Perioperative lignocaine infusion-an aid. Indian J Anaesth. 2020;64(5):448-449. 
  • Thampi SM, David D, Chandy TT, Nandhakumar A. Anesthetic management of a patient with amyotrophic lateral sclerosis for transurethral resection of bladder tumor. Indian J Anaesth. 2013;57(2):197-199.
  • Marsh, S., Pittard, A., 2011. Neuromuscular disorders and anaesthesia. Part 2: specific neuromuscular disorders. Continuing Education in Anaesthesia Critical Care & Pain 11, 119–123.

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