Amyotrophic Lateral Sclerosis

GENERAL INFORMATION:

What is amyotrophic lateral sclerosis?

• Amyotrophic lateral sclerosis is also called ALS, or Lou Gehrig disease. ALS is a disease that affects the nervous system. The nervous system includes the brain, spinal cord, and nerves in the body. The main cells that make up the brain and spinal cord are called neurons. Neurons that control the muscles for moving, breathing, and swallowing are called motor neurons. Movement occurs when upper motor neurons (neurons in the brain) send messages to lower motor neurons (neurons in the spinal cord). The lower motor neurons relay these messages to the specific muscles that carry out the movement.
  
  
• In ALS, both upper and lower motor neurons are affected. The neurons die and stop sending messages to muscles. These muscles gradually weaken, waste away, and begin to twitch. Over time, ALS may lead to total paralysis (unable to move) of the muscles, including the muscles for breathing. ALS mostly affects men. It usually affects people between the ages of 40 and 70 years old.

• Autoimmune disease: The immune system is the part of your body that fights infection. When you have an autoimmune disease like ALS, your body attacks its own motor neurons.
  
  
• Chemical imbalance: Glutamate is a chemical in the brain that sends signals from one nerve to another. Too much glutamate may cause the motor neurons to die.
  
  
• Frequent exposure to toxic chemicals: Chemicals, such as fertilizers and pesticides used in gardening and lawn care, may also cause ALS.
  
  
• Genetics: Having a close family member with ALS increases your chances of having this disease.
  
  
• Infection: Germs, such as viruses, can damage the motor neurons.

• Familial (inherited) ALS: This is caused by an inherited (passed from parent to child) genetic defect in a protein enzyme that removes toxins (poisons) in your body. The absence of this enzyme may cause damage to the motor neurons.
  
  
• Sporadic (non-inherited) ALS: This is the most common type of ALS. The cause of sporadic ALS is unknown.

• Hand and arm symptoms:
  
  
  • Dropping things frequently.
    
    
  • Having trouble doing tasks with your hands, such as opening bottles or turning keys.
    
    
  • Having a weak hand grip.
  
  
• Feet and leg symptoms:
  
  
  • Dragging one leg, or tripping easily.
    
    
  • Your legs get tired easily when climbing stairs or walking.
  
  
• Other symptoms:
  
  
  • Difficulty breathing, eating, or swallowing, especially in later stages of illness.
    
    
  • Drooling of saliva (juices in mouth) or making too much phlegm (mucus).
    
    
  • Jerking of an arm or leg while resting.
    
    
  • Twitching of weakened muscles.
    
    
  • Unusual fatigue (feeling more tired than usual).
    
    
  • Voice and speech problems.

• Electromyography: This test is also called an EMG. Nerves that control your muscles are checked, and their electrical activity is measured.
  
  
• Imaging tests:
  
  
  • Computerized tomography scan: This test is also called a CT scan. A special x-ray machine uses a computer to take pictures of your head. You may be given dye before the pictures are taken. The dye is usually given in your IV. The dye may help your caregiver see the pictures better. People who are allergic to iodine or shellfish (lobster, crab, or shrimp) may be allergic to some dyes. Tell the caregiver if you are allergic to shellfish or have other allergies or medical conditions.
    
    
  • Magnetic resonance imaging: Another name for this test is an MRI. Radio waves are used to take computerized pictures of your brain.
  
  
• Lumbar puncture: Fluid is taken from your spine and sent to a lab for tests. This is also called an LP, or spinal tap.
  
  
• Muscle biopsy: A sample of your muscle may be taken and sent to the lab for tests.
  
  
• Nerve conduction velocity: This test measures the impulses (sudden force) traveling through nerves between the spinal cord and muscles.

• Medicines:
  
  
  • Central nervous system medicine: This is a type of medicine that works on the central nervous system of the body. It works to reduce the damage to the motor neurons by decreasing the release of glutamate. Glutamate is a chemical in the brain.
    
    
  • Other medicines: These may help ease muscle cramps, fatigue, and excessive saliva or phlegm caused by ALS.
  
  
• Rehabilitation program: This program may include physical, breathing, and speech exercises.

• National Institute of Neurological Disorders and Stroke
  P.O. Box 5801
  Bethesda, MD 20824
  Phone: 1-800-352-9424
  Web Address: http://www.ninds.nih.gov