Amyotrophic lateral sclerosis (ALS)

Learning objectives

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

Symptoms with the progression of the disease:

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

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)

Preoperative and perioperative management

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