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Johns Hopkins Symptoms and Remedies

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Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease)

What is it?

Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, is a slowly progressive degeneration of the motor nerve cells of the spinal cord and lower brain stem, resulting in gradual loss of voluntary muscle control. (Sensory nerves and mental function are unaffected.) The disease is eventually fatal as muscles governing breathing, swallowing, and other crucial body functions are affected. Patients live an average of three to five years after the onset of symptoms; most die from respiratory insufficiency or from lung infections that tend to occur when breathing is impaired for long periods of time. ALS generally strikes between the ages of 50 and 70, and affects men slightly more often than women.

What Causes It?

  • The cause of ALS is unknown; however, about 5 to 10 percent of cases appear to be inherited.
  • Current theories suggest that the neurotransmitter glutamate (which is involved in the sending of nerve signals), free-radicals (unstable oxygen molecules that can damage cells), and the mineral calcium may all play roles in the death of motor nerve cells.

Prevention

  • There is no known way to prevent ALS.

Diagnosis

  • No specific tests confirm the diagnosis; patient history and physical examination are needed. Diagnosis is based on clinical manifestations of the disease.
  • Special neurological tests such as EMG (electromyography) or MRI (magnetic resonance imaging) may be performed to rule out other possible causes of symptoms.

How to Treat It

  • Although there is no cure, several drug treatments are either available or under study. Riluzole is the only Food and Drug Administration-approved drug for ALS; it has a modest effect in prolonging survival. Treatments that show promise include the anti-convulsant drug gabapentin, insulin-like growth factor I (Myotrophin), and certain antioxidants.
  • Mechanical devices such as dressing aids and special utensils for eating are available to facilitate various everyday tasks.
  • A cane or walker may be helpful for patients with gait impairment.
  • Patients should carefully consider the option of using a mechanical respirator in the event that they become unable to breathe unassisted. Artificial ventilation can help some patients survive for years; however, many patients choose not to be kept alive in a state of total paralysis, unable to communicate except with eye movements. It is important to discuss this issue with doctors and family early on, so that decisions about emergency resuscitation may be made in the event of a respiratory crisis.
  • Emotional support is crucial. Beyond the help that friends and family provide, a qualified counselor or psychotherapist can be a valuable asset. For assistance, contact the ALS Association or the Muscular Dystrophy Association.

When to Call a Doctor

  • See your doctor at the earliest signs of unexplained muscle weakness or difficulty controlling movement, particularly if speaking, breathing, or swallowing seems to be affected.