What Are the Signs & Symptoms of Lou Gehrig's Disease?

Lou Gehrig's disease---also known as amyotrophic lateral sclerosis---is a progressive and serious neurological disorder that causes muscle deterioration, immobilizes its victims and is nearly always fatal. It affects the spinal cord and the brain's nerve cells. Motor neurons travel from the brain to the spinal cord to the body's muscles. The disease causes these neurons to deteriorate and die, resulting in the brain becoming powerless in controlling muscle movements. Late-state sufferers often become paralyzed.

History

Amyotrophic lateral sclerosis started being referred to as Lou Gehrig's disease in 1939 after the New York Yankees' Hall of Fame first baseman contracted the disease and was forced to retire from the sport. Gehrig died of ALS in 1941 at the age of 37. Nearly 30,000 Americans suffer with ALS and another 5,000 receive an ALS diagnosis each year. Generally, ALS strikes individuals in midlife, seems to favor men over women and chooses its victims randomly, often those with no family history of the disease.

Types

Lou Gehrig's disease is either sporadic or genetic. Sporadic ALS occurs in nearly 90 percent of all reported cases and has no definitive cause. The immune system, however, may be a contributing factor. Sporadic ALS sufferers start experiencing symptoms around age 56 with death occurring several years later. Genetic ALS affects multiple family members (or generations of families). This type produces similar symptoms as early as age 46 and may progress at a slower rate. Individuals inflicted with genetic ALS have a 50-50 chance of passing the disease to their children.

Signs and Symptoms

Signs and symptoms of Lou Gehrig's disease vary among sufferers and oftentimes are subtle. The condition first appears as muscle twitching, cramping or stiffness, weakness in the extremities or as slurred or garbled speech. One-quarter of afflicted individuals begin with vocal or swallowing issues while more than one-half experience difficulties in their arms and legs. Other common symptoms include tripping or stumbling, problems chewing, an overactive gag reflex, difficulty performing simple tasks, such as writing, turning a key or buttoning a blouse--even breathing. No matter what part of the body initially is impaired by ALS, all the muscles weaken and disintegrate as the disease advances. Ultimately sufferers lose their mobility, their ability get in and out of bed or employ their hands and arms. A person's cognitive abilities do not seem to be altered by the disease.

Risk Factors

As with many diseases, certain risk factors are inherent in Lou Gehrig's disease. A genetic defect on chromosome 21 is attributed to roughly 10 percent of all familial ALS. This chromosome "codes" for the superoxide dismutase enzyme that shields motor neurons from free radical damage. Other risk factors include: age---those 40 to 60 years old are more susceptible; gender---more men than women under 65 develop ALS; military background---individuals serving in the armed forces have an increased likelihood of being diagnosed; and geographic location---residents of Japan, Guam and West New Guinea have a higher incidence of ALS than people in other parts of the world.

Warning

Have a second physician review your case. Various other diseases or conditions, such as lead or mercury poisoning, hyperthyroidism and Lyme disease--many of which are treatable--share symptoms with ALS. Consulting with a neurologist specializing in Lou Gehrig's disease is recommended.

References

Article reviewed by Anita Crone Last updated on: Jul 2, 2009

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