Amyotrophic Lateral Sclerosis (ALS), sometimes referred to as "Lou Gehrig's Disease," is a progressive fatal neuromuscular disease that attacks nerve cells and pathways in the brain and spinal cord. Motor neurons, among the largest of all nerve cells, reach from the brain to the spinal cord and from the spinal cord to muscles throughout the body with connections to the brain. When they die, the ability of the brain to start and control muscle movement dies with them. With all voluntary muscle action affected, ALS patients in the later stages are totally paralyzed; through it all, however, their minds remain unaffected.

Amyotrophic comes from the Greek language. "A" means no or negative. "Myo" refers to muscle, and "Trophic" means nourishment--"No muscle nourishment." When a muscle has no nourishment, it "Atrophies" or wastes away. "Lateral" identifies the areas in a person's spinal cord where portions of the nerve cells that nourish the muscles are located. As this area degenerates it leads to scarring or hardening ("Sclerosis") in the region.

Over 5,000 Americans are diagnosed with ALS each year. There is great variation in the course of the disease. Symptoms usually appear in individuals between the ages of 40 - 70, though the disease has been reported in both younger and older persons. The average duration of ALS is two to five years, but some individuals remain active for years longer. In twenty percent of the cases, the disease plateaus for extended periods of time. In some cases, there is a remission associated with reaching a plateau. Most people are able to live productive and satisfying lives long after the disease has been diagnosed. Men seem to be affected more often than women. There appears to be some type of familial pattern in five to ten percent of all cases, but the majority of cases have no hereditary pattern.

Approximately one-third of patients become aware of the onset of the disease when their hands become clumsy, causing difficulty in the performance of fine tasks. Another one-third experience weakness in the legs and may trip because of mild foot drop. The remaining one-third notice slowing of speech or difficulty in swallowing. ALS may be present for some time before any symptoms are noticed. This lack of noticeable symptoms occurs because the lost or damaged nerve cells are compensated for by nerve cells that remain functioning. One early symptom is generalized fatigue. As muscle cells deteriorate, patients may experience stiffness or occasional jerking of the arms or legs resulting from spasticity (muscle tenseness). Often symptoms begin in the hands and feet, then travel inward toward the center of the body. One side is usually more affected. Paralysis eventually may be virtually complete, except for the muscles of the eyes. Anal and bladder muscles and function are not usually affected.

There is no specific clinical test that can identify ALS. Diagnosis is made by a neurologist through a physical examination, a thorough patient medical history, and neurological testing. Diagnostic testing often includes the electromyogram (EMG) to test muscle activity, CT Scan, MRI (Magnetic Resonance Imaging), muscle and/or nerve biopsy.

While it is true that there is no cure for ALS, much can be done to help the patient live with the disease. Treatment aimed at relieving symptoms can be very effective. Generally, patients should continue usual daily activities, stopping before they become fatigued. Patients should be encouraged to set their own limits of exertion, and to plan how they will use their energy and strength. The physician will probably suggest exercises, including breathing exercises, to strengthen unaffected or less-affected muscles. These exercises are not vigorous or tiring, but are intended to help maintain mobility and prevent joint stiffness and muscle contracture. Various devices such as foot-drop braces, hand splints, limb supports or wheelchairs enable the patient to remain as independent as possible. Good skin care, massage and knowledge of proper body positioning can prevent sores for those who are confined to bed for lengthy periods of time. If bowel and bladder function are affected by immobility, increasing daily fluid intake should improve the situation or the doctor may prescribe stool softeners, bulk formers or laxatives.

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