Amyotrophic Lateral Sclerosis (ALS)

What is it?

• ALS is a“progressive degeneration of motor neurons on the brainstem, anterior horn cells of the spinal cord, and the motor cortex that eventually affects all voluntary muscles” (Hamby, 2017). The onset of ALS typically occurs between the ages of 40 and 70 (the average age is 55). It is 20% more common in men. However, as age increases the incidence equalizes between genders.

Types

• Sporadic: 90-95% of cases occur randomly without genetic cause or family history

• Familial: small amount of people are thought to inherit ALS

How is it diagnosed?

• Generally a diagnosis of exclusion. The patient must have both upper and lower motor neuron symptoms. Electromyograph and muscle/nerve biopsy are used.

Symptoms & Stages

• Onset of symptoms is generally gradual and initial symptoms can vary. During the early stage, patients experience muscle weakness and/or stiffness with progressive weakness and paralysis to follow. For each patient, the order of symptoms and rate of disease progression will vary.

  • Gradual onset of progressive muscle weakness is the most common first symptom. Other early signs include: tripping, dropping things, abnormal fatigue, slurred speech, muscle cramps, muscle twitching, and/or uncontrolled periods of laughing or crying.

  • Once breathing is affected, permanent ventilatory support to assist breathing will be required.

Treatment

• There is no cure for ALS and treatment is primarily focused on symptom management and supportive care

Life Expectancy

• The average life expectancy after diagnosis is 3 years, with overall life expectancy varying based on age and symptoms present at diagnosis. ~20% live for 5 years, 10% for 10 years, and 5% live for 20+ years.