Amyotrophic lateral sclerosis (ALS) - also known as Lou Gehrig's disease - is a rapidly progressing neurological disorder in which most motor neurons in the spinal cord and brain degenerate, typically over four to six years. Currently, ALS is a fatal disease, but scientists are hopeful that a cure is forthcoming.
- Is an adult-onset disease that usually begins between the ages of 40 and 70.
- Has no known cause.
- Impacts men and women of all ethnic and racial groups equally.
- Has no known cause in 90% of cases. About 10% of cases run in families and the ALS patients have defined genetic defects that are known to trigger the disease.
- Has disease symptoms that are similar to other neurological disorders. And ALS has no single diagnostic test, which is why the diagnosis is often delayed.
- Causes motor nerve cells to degenerate, meaning many types of movement are impaired and ultimately lost.
- Initially causes muscle weakness, muscle twitching, cramping and stiffness of muscles, unusual fatigue and clumsiness, or difficulty swallowing and speaking.
Although the sequence of emerging symptoms and the progression rate for the disease differ from person to person, most people with ALS will have their muscles weaken and eventually become paralyzed. Many functions other than muscle movement - such as the ability to think - remain intact.
Scientists are closely examining several factors about ALS that seem to play a role in motor neuron degeneration, including:
- High levels of the neurotransmitter glutamate.
- Exposure to adverse environmental factors such as infections or poisons.
- Insufficient energy generation by brain cells.
- Inflammation in the spinal cord.
- Slowing of the transport of substances in long neuron processes known as axons.
There is currently no cure for ALS, but researchers are dedicated to discovering new treatments. A single drug called riluzole has been approved for use in ALS, but at best it modestly slows the disease. Several other experimental drugs are in trials and development in ALS research laboratories.