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Amyotrophic Lateral Sclerosis (ALS) Early Symptoms, Causes, and Prognosis

ALS (amyotrophic lateral sclerosis) definition and facts*

*Amyotrophic lateral sclerosis facts medical author: William C. Shiel Jr., MD, FACP, FACR

  • Amyotrophic lateral sclerosis is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells responsible for controlling voluntary muscles.
  • Amyotrophic lateral sclerosis, or ALS, is sometimes called Lou Gehrig's disease.
  • As many as 20,000 to 30,000 people in the United States have ALS, and an estimated 5,000 people in the United States are diagnosed with the disease each year.
  • Only about 5 to 10 percent of all ALS cases are inherited in the family's genes.
  • Early symptoms of ALS are subtle and may include twitching, cramping, or stiffness of muscles; muscle weakness affecting an arm or a leg; slurred and nasal speech; or difficulty chewing or swallowing.
  • No one test can provide a definitive diagnosis of ALS, although the presence of upper and lower motor neuron signs in a single limb is strongly suggestive.
  • The cause of ALS is not known, and scientists do not yet know why ALS strikes some people and not others. Scientists have discovered that mutations in the gene that produces the SOD1 enzyme were associated with some cases of familial ALS.
  • No cure has yet been found for ALS. The first drug treatment for the disease -- riluzole (Rilutek) is believed to reduce damage to motor neurons by decreasing the release of glutamate. Clinical trials with ALS patients showed that riluzole prolongs survival by several months and extends the time before a patient needs ventilation support. Other treatments for ALS are designed to relieve symptoms and improve the quality of life for patients.

What is ALS (amyotrophic lateral sclerosis)?

Amyotrophic lateral sclerosis (ALS) is a rare group of neurological diseases that mainly involve the nerve cells (neurons) responsible for controlling voluntary muscle movement. Voluntary muscles produce movements like chewing, walking, breathing and talking. The disease is progressive, meaning the symptoms get worse over time. Currently, there is no cure for ALS and no effective treatment to halt, or reverse, the progression of the disease.

ALS belongs to a wider group of disorders known as motor neuron diseases, which are caused by gradual deterioration (degeneration) and death of motor neurons. Motor neurons are nerve cells that extend from the brain to the spinal cord and to muscles throughout the body. These motor neurons initiate and provide vital communication links between the brain and the voluntary muscles.

Messages from motor neurons in the brain (called upper motor neurons) are transmitted to motor neurons in the spinal cord and to motor nuclei of brain (called lower motor neurons) and from the spinal cord and motor nuclei of brain to a particular muscle or muscles.

In ALS, both the upper motor neurons and the lower motor neurons degenerate or die, and stop sending messages to the muscles. Unable to function, the muscles gradually weaken, start to twitch (called fasciculations), and waste away (atrophy). Eventually, the brain loses its ability to initiate and control voluntary movements.

Early symptoms of ALS usually include muscle weakness or stiffness. Gradually all muscles under voluntary control are affected, and individuals lose their strength and the ability to speak, eat, move, and even breathe.

Most people with ALS die from respiratory failure, usually within 3 to 5 years from when the symptoms first appear. However, about 10 percent of people with ALS survive for 10 or more years.

Medically Reviewed by a Doctor on 9/18/2017

Source: MedicineNet.com
https://www.medicinenet.com/amyotrophic_lateral_sclerosis/article.htm

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