What is ALS Disease?

ALS (Amyotrophic Lateral Sclerosis), also known as Lou Gehrig’s Disease, is an incurable fatal neuromuscular disease characterized by progressive muscle weakness, resulting in paralysis.Amyotrophic Lateral Sclerosis is a form of motor neuron disease. ALS, sometimes called Maladie de Charcot, is a progressive, neurodegenerative disease caused by the degeneration of motor neurons, the nerve cells in the central nervous system that control voluntary muscle movement.The disorder causes muscle weakness and atrophy throughout the body as both the upper and lower motor neurons degenerate, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, develop fasciculations ( muscle twitches) because of denervation ( loss of nerve supply ) and eventually atrophy ( is the partial or complete decrease in size or wasting away of a part of the body ) because of that denervation. The patient may ultimately lose the ability to initiate and control all voluntary movement; bladder and bowel sphincters and the muscles responsible for eye movement are usually spared.Over time, Lou Gehrig’s disease causes these motor neurons in the brain and spinal cord to shrink and disappear, so that the muscles no longer receive signals to move. As a result, the muscles become smaller and weaker, gradually the body becomes paralyzed
ALS often called Lou Gehrig’s disease is named after Lou Gehrig, a hall-of-fame baseball player for the New York Yankees who was diagnosed with ALS in the 1930s. People in England and Australia call ALS Motor Neurone Disease (MND). The French refer to it as Maladie de Charcot, after the French doctor Jean-Martin Charcot, who first wrote about ALS in 1869.ALS is one of the most common neuromuscular diseases worldwide, and people of all races and ethnic backgrounds are affected. One 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. “Familial ALS” accounts for approximately 5%–10% of all ALS cases and is caused by genetic factors.
Early Symptoms
A-myo-trophic 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 signal and control the muscles are located. As this area degenerates it leads to scarring or hardening (”sclerosis”) in the region.As motor neurons degenerate, they can no longer send impulses to the muscle fibers that normally result in muscle movement. Early symptoms of ALS often include increasing muscle weakness, especially involving the arms and legs, speech, swallowing or breathing. When muscles no longer receive the messages from the motor neurons that they require to function, the muscles begin to atrophy (become smaller). Limbs begin to look “thinner” as muscle tissue atrophies.As motor neurons degenerate, they can no longer send impulses to the muscle fibers that normally result in muscle movement.
How to confirm the disease?
Although the cause of ALS is not completely understood, the recent years have brought a wealth of new scientific understanding regarding the physiology of this disease. Electromyography (electrical studies of muscle) is often performed to confirm the disease. A biopsy (removal of a small piece of tissue) of muscle tissue may also been performed if the diagnosis is in doubt.

Cure/ Treatment

While there is not a cure or treatment today that halts or reverses ALS, there is one FDA approved drug, riluzole, that modestly slows the progression of ALS. Importantly, there are significant devices and therapies that can manage the symptoms of ALS that help people maintain as much independence as possible and prolong survival. It is important to remember that ALS is a quite variable disease; no two people will have the same experiences.

Recent Studies

Research studies are continually showing that there is a link between amyotrophic lateral sclerosis (ALS) — the disease that claimed the life of legendary Yankees first-base man, Lou Gehrig — and those who play sports on grass.

Careful study concludes that there is no correlation between physical activity and ALS. Indoor athletes, ice hockey players and swimmers, are not at risk for ALS. Outdoor athletes are.

A 10 year study is underway to help to understand what may have contributed to the deaths of the growing number of soccer players in Britain.It has been determined that a common environmental chemical may increase the risk for developing ALS. Chemicals, such as fertilizers and pesticides used in gardening and lawn care, may also cause ALS.-The Nature Lyceum

Currently, there’s no way to prevent or cure Lou Gehrig’s disease, but a number of treatments are available to people with the disease. Medicines can control symptoms, such as muscle cramping and difficulty swallowing, and other drugs can slow the development of the disease.
However,if a patient develops early warning signs, it is crucial to seek an immediate evaluation from a qualified neurology specialist.

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