About A.L.S

A.L.S. Amyotrophic Lateral Sclerosis is the most severe illness of a group of neurodegenerative diseases that damage motor neurons. A.L.S. is a rare and incurable disease. In America A.L.S is also known by the name Lou Gehrig's disease, after the famous baseball player who died of the disease in the 1930's; in England as M.N.D. Motor Neuron Disease; and in France as Maladie de Charcot for one of the first doctors to document the disease. The disease attacks the motor neurons, which the brain uses to control most of the body's voluntary muscles. As a result these neurons are destroyed and the patient can no longer use his muscles. This leads to muscle deterioration and eventually to complete paralysis. As the disease progresses the muscles that are used to speak, swallow, breathe and control the limbs, gradually stop working in no particular order. In most cases it does not effect intellect, memory or the senses.
Incidence and Prevalence: The impact on the community of A.L.S. is usually measured by the incidence and prevalence of the disease. Incidence is the number of new cases added in a defined period, usually over the course of a year. Prevalence is the number of cases existing at any point in time. The incidence of A.L.S. is 2 per 100,000 of total population, while prevalence is six per 100,000 of total population. Only 10% of cases are genetically inherited while the remaining 90% are sporadic. Research has found that the incidence is higher in people aged 45-65. Cases have been reported of people both older and younger contracting the disease but those cases occur infrequently.
Beginning Symptoms: The beginning symptoms of the disease are usually quite mild and non specific, which often delays an accurate diagnosis. The symptoms may be weakness in the upper or lower limb muscles and difficulty executing certain movements such as writing or lifting objects. Similarly climbing or walking distances become difficult. There may be muscle twitching and cramping (called fasciculation), as well as impaired speaking, swallowing and breathing. In some cases early symptoms include the head falls to one side as a result of weakened neck muscles. Diagnosis: the most important diagnostic test is an EMG, which records the electrical activity in the muscles. In A.L.S. patients, an EMG shows a lack of activity of the motor neurons, while, at the same time the sensory neurons continue to function normally.
The Disease's Progression: The onset of the disease and its advancement differs from patient to patient making it difficult to predict the path or rate of progression in any one individual. Usually there is a gradual weakening and eventual paralysis of the limb muscles. Likewise, the patient loses control of the muscles in charge of speaking, chewing, and swallowing and finally breathing which eventually is fatal for most of the patients. Most patients live 2-5 years from the onset of the disease. About 20% live 5-10 years and 10% survive for over 10 years. The Cause: The cause of the illness is unknown, which makes it difficult to find a cure.


Possible causes:
1. Environmental - the nerve cells are poisoned by met A.L.S., chemicA.L.S. or certain foods;
2. High levels of glutamates - Glutamate is a chemical that stimulates nerve activity. High levels of this chemical can over increase cell activity thus damaging the cells and eventually destroy them;
3. Lack of certain materiA.L.S. - certain materi A.L.S. that are responsible for the growth and well being of the cells are missing causing accumulation of cell material that harms the immune system;
4. Hereditary - Some of those patients that have inherited the illness lack a certain protein that neutralizes free radicA.L.S., which can cause the destruction of nerve cells.


None of these have been proven as the cause of A.L.S. but a connection has been found with the surplus of glutamates. It is possible that a combination of a few of these factors could be the cause of A.L.S.. Many different medicines have been tried for A.L.S. but without any real success. The only medicine that has been shown to slow the advancement of the disease is Riluzole. Unfortunately, the improvement in life expectancy is only a few months.
Critical to the well being of the patient is the care and support that he receives from his family. Additional important factors include physical therapy, medical equipment, speech therapy, nutritional support, breathing support and psychological counseling. Since this is a particularly difficult fatal illness and the patient is aware of his condition, it is advisable to discuss the use of artificial respiratory apparatus with the patient at the onset of the illness.