ALS – Amyotrophic lateral sclerosis – Lou Gehrig’s disease

Amyotrophic lateral sclerosis, or ALS, is a disease of the nerve cells in the brain and spinal cord that control voluntary muscle movement.  Also known as Lou Gehrig’s disease, ALS is caused by a genetic defect in about 10% of cases, the rest having unknown causes.

ALS causes nerve cells, or neurons, to waste away or die, leaving them unable to send messages to muscles the patient wants to control.  This leads to muscle weakness, twitching and losing the ability to move arms, legs and body.  As the disease progresses and worsens, the muscles in the chest area stop working and it becomes difficult or impossible to breathe on ones’ own. 

ALS worldwide affects approximately 5 out of every 100,000 people. 

Early symptoms typically do not reveal themselves until after age 50, however they can start in younger people as well.  Patients with ALS lose muscle strength and coordination that eventually gets worse and makes routine tasks impossible, such as ascending a staircase, getting up from a chair or swallowing.

ALS does not affect the physical senses of sight, smell, taste, hearing and touch.  It only rarely affects bladder or bowel function, or a person’s ability to think and reason. 

Early symptoms include:

  • Hard time breathing or swallowing
    • Choking easily
    • Drooling
    • Gagging
  • Head drooping due to weakness in neck muscles
  • Muscle cramps, contractions and progressive weakness, commonly in one part of the body initially (e.g. arm or hand)
  • Paralysis

ALS is a very difficult disease to diagnose.  There is no one test or procedure to definitively establish the presence of ALS.  It is only through physical examination and a series of diagnostic tests, usually to rule out other diseases, that an ALS diagnosis can be established.  Most, if not all of the following procedures are typically conducted:

  • Electrodiagnostic tests including electromyography (EMG) and nerve conduction velocity (NCV)
  • Blood and urine studies including high resolution serum protein electrophoresis, thyroid and parathyroid hormone levels and 24 hour urine collection for heavy metals
  • Spinal tap
  • X-rays, including MRI
  • Myelogram of cervical spine
  • Muscle and/or nerve biopsy
  • Thorough neurological examination

Important Facts:

  1. the onset of ALS is insidious with muscle weakness or stiffness as early symptoms.  Progression of weakness, wasting and paralysis of the muscles of the limbs and trunk as well as those that control vital functions such as speech, swallowing and later breathing generally follows.
  2. ALS is not contagious.
  3. It is estimated that ALS is responsible for approximately 2 deaths per 100,000 population annually.
  4. Although the life expectancy of an ALS patient averages about 2-5 years from the time of diagnosis, this disease is variable and many people live with quality for 5 years or more.  More than half of all patients live more than 3 years after diagnosis.
  5. About 20% of people with ALS live 5 years or more and up to 10% will survive more than 10 years and 5% will live 20 years.  There are people in whom ALS has stopped progressing and a small number of people in whom the symptoms of ALS reversed.
  6. ALS occurs throughout the world with no racial, ethnic or socioeconomic boundaries.
  7. ALS can strike anyone.

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