Amylotrophic+Lateral+Sclerosis

Amyotrophic Lateral Sclerosis is a progressive and degenerative disease of the motor neurons. Otherwise known as Lou Gherig’s disease, for the American Baseball hero who made it famous. Gherig was diagnosed and simultaneously he retired in 1939 catching public attention on the horrors of this dreadful disease. First identified in 1869, by the “father of ,” Jean-Marie Charcot of France, his reports would describe characteristics that in 140 years, there is still have little understanding of what causes the disease or how it could be prevented. In this amount of time the pattern for it has remained relatively unchanged, a fatal list of symptoms that in two to five years result in certain death.
 * Amyotrophic Lateral Sclerosis**

The terminology of the disease itself is broken down and translates to amyotrophic referring to the muscle atrophy, weakness and fasciculation syndrome. Lateral Sclerosis paints the picture of autopsy specimens,specifically hardness observed on lateral portions of the spinal cord that are a sign of necrosis. The basic principle behind ALS is how the nervous system begins to break down from neuronal cell death. This will weaken skeletal muscles until the body is ultimately paralyzed. Early stages will effect limbs, which is known as limb-onset ALS. About 25% of cases are "bulbar onset" ALS. These patients first notice difficulty speaking clearly or swallowing. Speech may become slurred, nasal in character, or quieter. The most serious symptoms start to effect breathing and from the deterioration, patients must use respirators to breathe. However, even such mechanism to surely compromise one’s ability to live, mental affects and other special senses are not usually seen.

Motor neurons begin at the top of the brain and are connected to a series of pathways to the spinal cord and from then on to muscles throughout the body. It is from these neurons that we are able to control all movement, whether one running, breathing, even opening your mouth, eyes and even breathing. As ALS progresses rapidly these neurons cease to function and eventually die. Damage to upper motor neurons results in delayed reflexes and weakness. Lower motor neurons begin at the base of the brain and continue down the spinal cord. Damage to lower motor neurons result in atrophy and twitching of muscles, tingling sensations known as Hoffman’s Signs along with a series of other observed symptoms patients have developed. An abnormal reflex commonly called Babinski's sign (the big toe extends upward and other toes spread out) also indicates upper motor neuron damage. The disease is extremely hard on the individual mentally, which accounts for the noticeable “assisted suicide” rates. Special senses such as sight, smell, hearing, touch or taste remain intact. Also, since it only effects motor neurons, intelligence, memory and personality remain. In this way, it is not the person being altered, just the capsule, or body which loses all functionality. The disease is considered “painless” although the physical deterioration and mental instability is painful enough to see.

-Around 6,000 diagnoses each year -Annual incidence- 2-3 per 100,000 people -Anywhere from 15,000 to 30,000 Americans have the disease at any given time -risk for developing ALS for individuals aged 18 years has been estimated to be 1 in 350 for men and 1 in 420 for women -Most people who develop ALS are between the ages of 40 and 75, with the majority after age 60, although it can occur at a younger age. -The incidence of ALS is five times higher than Huntington’s disease and about half that of multiple sclerosis.
 * __ALS Statistics__**

Causes of ALS Diagnosis Etiology Treatment Medical Advances References SOD1 Gene