Lou Gerigs Disease - ALS - Info and Resources

Lou Gerigs Disease

Amyotrophic lateral sclerosis (ALS, sometimes called Lou Gehrig's Disease, or Maladie de Charcot) is a progressive, usually fatal, neurodegenerative disease caused by the degeneration of motor neurons, the nerve cells in the central nervous system that control voluntary muscle movement.

As one of the motor neuron diseases, the disorder causes muscle weakness and atrophy throughout the body as both the upper and lower motor neurons degenerate and die, ceasing to send messages to muscles.

Unable to function, the muscles gradually weaken, develop fasciculations (twitches) because of denervation, and eventually atrophy due to that denervation. The patient may ultimately lose their ability to initiate and control all voluntary movement except of the eyes.

The onset of ALS may be so subtle that the symptoms are frequently overlooked. The earliest symptoms may include twitching, cramping, or stiffness of muscles; muscle weakness affecting an arm or a leg; and/or slurred and nasal speech. These general complaints then develop into more obvious weakness or atrophy that may cause a physician to suspect ALS.

The parts of the body affected by early symptoms of ALS depend on which motor neurons in the body are damaged first. About 75% of people experience "limb onset" ALS. In some of these cases, symptoms initially affect one of the legs, and patients experience awkwardness when walking or running or they notice that they are tripping or stumbling more often. Other limb onset patients first see the effects of the disease on 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.

Lou Gerigs Disease (ALS)

ALS is one of the most common neuromuscular diseases worldwide, and people of all races and ethnic backgrounds are affected. Between 1 to 2 people per 100,000 develop ALS each year . ALS most commonly strikes people between 40 and 60 years of age, but younger and older people can also develop the disease. Men are affected slightly more often than women.

Scientists have not found a definitive cause for ALS and the onset of the disease can be linked to a variety of risk factors. It is believed that one or more of the following factors are responsible for the majority of ALS cases. Researchers suspect a virus, exposure to neurotoxins or heavy metals, DNA defects, immune system abnormalities, and enzyme abnormalities as the leading causes of the disease. There is a hereditary factor in familial ALS (FALS) however there is no known hereditary component in the 90-95% cases diagnosed as sporadic ALS.

A few causative factors have been suggested for the increased incidence on the West Pacific. Prolonged exposure to a dietary neurotoxin is the suspected risk factor in Guam's increased incidence in ALS. The neurotoxin is a compound found in the seed of the cycad Cycas circinalis, a tropical plant found in Guam, which was used in the human food supply during the 1950s and early 1960s.

An inherited genetic defect linked to a defect on chromosome 21 is believed to cause approximately 40% of familial cases of ALS. This mutation is believed to be autosomal dominant. The children of those diagnosed with familial ALS have a higher risk factor for developing the disease, however those who have close family members diagnosed with sporadic ALS have no greater a risk factor than the general population .

According to The ALS Association, military veterans are at an increased risk of contracting ALS. In its report ALS in the Military, the group pointed to an almost 60% greater chance of the disease in military veterans than the general population, and latest statisics showing Gulf War vet's at twice the risk than the general population according to a study done by the Veteran's Affairs Administration and the DOD.