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Lou Gehrig in his first appearance with the New York Yankees before a game, circa June 11, 1923.

ALS (Lou Gehrig's disease)

USA Health is proud to be the home of the Kelly Butler ALS Center, allowing patients to see providers from a variety of disciplines during a single appointment.

Learn about the Kelly Butler ALS Center

What is ALS?

Amyotrophic lateral sclerosis, or ALS, causes weakness and wasting of all the muscles used to move, swallow, speak and breathe. The illness is not with the muscle, but with the nerve cells, or motor neurons. ALS often is referred to as a motor neuron disease (MND).

ALS also is known as Lou Gehrig’s disease. Lou Gehrig, a Major League Baseball player in the 1920s and ’30s, noticed a progressive weakness, with no clear reason, while still playing baseball.

Quick Facts

There are two types of ALS:

Sporadic: Occurs with no known cause, approximately 90% of cases.

Familial: Inherited from family, approximately 10% of cases.

Diagnosing ALS

ALS is diagnosed when a person shows signs of loss of motor neurons in different parts of the body.

  • Upper motor neurons travel from the brain to the spinal cord to control movement. A loss of these neurons results in stiffness of the limbs (spasticity), brisk reflexes (hyperreflexia), and slow movements of the hands and legs (bradykinesia). It may appear as weakness and slow movements of muscles that produce speech, leading to strained, effortful speaking.
  • Lower motor neurons are nerves that run from the spinal cord to the muscle. Their loss causes weakness, muscle wasting (amyotrophy), and twitching (fasciculations).
     

ALS specialists divide the body into four regions based on the way the nerves that are attached to the muscles are wired.

  • Brainstem/bulbar: Face and tongue
  • Cervical: Arms
  • Thoracic: Trunk
  • Lumbar: Legs
     

Neurologists look for signs of upper and lower motor neuron loss in each one of these four regions of the body. During diagnosis, it is not unusual to seek a second opinion.

Symptoms of ALS

  • Muscle cramps or twitching
  • Fatigue
  • Trouble with stairs
  • Trouble getting up from a chair
  • Occasional falls
  • Difficulty lifting items or holding a pen
  • Choking on liquids or trouble swallowing certain foods
  • Drooling or trouble clearing phlegm
  • Poor sleep
  • Shortness of breath with activity or when lying flat
  • Changes in speech or getting tired when speaking
  • Trouble making decisions or forgetfulness
  • Tendency to cry or laugh too much (pseudobulbar affect)

Progression of ALS

No two presentations of ALS are the same. In some individuals, the disease progresses very slowly, and in others, it progresses very quickly. There is no way to predict how a person’s ALS will progress.

Symptoms typically do not begin all at once. It may feel this way, but the body often will compensate for a problem until it becomes too weak to keep up. Eventually you will need help with all movements: getting out of bed, moving to a chair, getting dressed, showering, etc., due to paralysis. Loss of muscle mass and weight will happen. Weight loss is also a result of swallowing difficulties and a decreased ability to eat.

Treating ALS

Two medications are FDA approved for the treatment of patients with ALS:

  • Riluzole (Rilutek®)
  • Edaravone (Radicava®)
     

Your ALS specialist will review the pros and cons of each medication.

Kelly Butler ALS Center Physicians

The multidisciplinary team of providers at the Kelly Butler ALS Center includes highly skilled neurologists with expertise in ALS and other neurological conditions.

Referring Physicians

These neuromuscular physicians at USA Health can evaluate patients for referral to the Kelly Butler ALS Center.

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