More than 12,000 people in the U.S. have a definite diagnosis of ALS, for a prevalence of 3.9 cases per 100,000 persons in the U.S. general population, according to a report on data from the National ALS Registry. ALS is one of the most common neuromuscular diseases worldwide, and people of all races and ethnic backgrounds are affected. ALS is more common among white males, non-Hispanics, and persons aged 60–69 years, but younger and older people also can develop the disease. Men are affected more often than women.
In 90 to 95 percent of all ALS cases, the disease occurs apparently at random with no clearly associated risk factors. Individuals with this sporadic form of the disease do not have a family history of ALS, and their family members are not considered to be at increased risk for developing it.
About 5 to 10 percent of all ALS cases are inherited. The familial form of ALS usually results from a pattern of inheritance that requires only one parent to carry the gene responsible for the disease. Mutations in more than a dozen genes have been found to cause familial ALS.
What are the symptoms?
The parts of the body showing early symptoms of ALS depend on which muscles in the body are affected. Many individuals first see the effects of the disease in a hand or arm as they experience difficulty with simple tasks requiring manual dexterity such as buttoning a shirt, writing, or turning a key in a lock. In other cases, symptoms initially affect one of the legs, and people experience awkwardness when walking or running or they notice that they are tripping or stumbling more often. When symptoms begin in the arms or legs, it is referred to as “limb onset” ALS. Other individuals first notice speech problems, termed “bulbar onset” ALS.
Regardless of the part of the body first affected by the disease, muscle weakness and atrophy spread to other parts of the body as the disease progresses. Individuals may develop problems with moving, swallowing (dysphagia), and speaking or forming words (dysarthria). Symptoms of upper motor neuron involvement include spasticity and exaggerated reflexes (hyperreflexia) including an overactive gag reflex. An abnormal reflex commonly called Babinski’s sign (the large toe extends upward as the sole of the foot is stimulated in a certain way) also indicates upper motor neuron damage.
To be diagnosed with ALS, people must have signs and symptoms of both upper and lower motor neuron damage that cannot be attributed to other causes.
Affected individuals also face an increased risk of pneumonia during later stages of ALS.
The ALS Ice Bucket Challenge has quickly gone from a fundraising campaign to a viral Internet sensation, raising $15.6 million so far for the ALS Association to research Lou Gehrig’s disease.
But how did a campaign that has drawn in celebrities from Oprah and LeBron James to Bill Gates and Mark Zuckerberg actually start?
Chris Kennedy, a golfer in Sarasota, Fla., was nominated by a friend to participate in the Ice Bucket Challenge, which at the time, had nothing to do with ALS. The campaign was not tied to any specific charity, and participants would select a charity of their choice for donations. Kennedy’s friend had selected a charity that benefits a young child with cancer in the area. Kennedy, passing the challenge along, then selected ALS because a relative is suffering from the disease. Kennedy nominated his wife’s cousin Jeanette Senerchia, whose husband Anthony is the one suffering from ALS. Kennedy posted this video on July 15—what appears to be the first instance in which the Ice Bucket Challenge and ALS were linked.
“What started out as a small gesture to put a smile on Anthony’s face and bring some awareness to this terrible disease has turned into a national phenomenon and it is something we never could have dreamed of,” Kennedy said.
http://www.alsa.org/about-als/what-is-als.html
http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail_als.htm
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