Thursday, September 4, 2014

Amyotrophic Lateral Sclerosis (ALS)

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Amyotrophic Lateral Sclerosis (ALS)
Amyotrophic lateral sclerosis (ALS)— Lou Gehrig's disease, and rarely Charcot disease—is a neurodegenerative disorder with various causes. The term motor neurone disease (MND) is sometimes used interchangeably with ALS, while others use it to refers to a group of similar conditions that include ALS. ALS is characterised by muscle spasticity, rapidly progressive weakness due to muscle wasting. This results in difficulty speaking, swallowing, and breathing. The disease usually starts around the age of 60, except in cases that are directly inherited when the usual age of onset is around 50.

About 5 to 10% of cases are directly inherited from a person's parents. ALS is the most common of the five motor neuron disease.

The average survival from onset to death is three to four years. Only 4% survive longer than 10 years, although rare cases survive 50 years or more. Most die from respiratory failure. In much of the world rates of ALS are unknown. In Europe the disease affects about 2.2 people per 100,000 per year. In the United States, more than 5,600 are diagnosed every year, and up to 30,000 Americans are currently affected. ALS is responsible for 2 deaths per 100,000 people per year.

Description of the disease date back to at least 1824 by Charles Bell. In 1869 the connection between the symptoms and the underlying neurological problems were first described by Jean-Martin Charcot who in 1974 began using the term amyotrophic lateral sclerosis. It became well known in the United States when it affected a famous baseball player by the name of Lou Gehrig.


Amyotrophic lateral sclerosis (ALS), often referred to as "Lou Gehrig's Disease," is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed.

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.

What Types of Nerves Make Your Body Work Properly?

The body has many kinds of nerves. There are those involved in the process of thinking, memory, and of detecting sensations (such as hot/cold, sharp/dull), and others for vision, hearing, and other bodily functions. The nerves that are affected when you have ALS are the motor neurons that provide voluntary movements and muscle power. Examples of voluntary movements are your making the effort to reach for the phone or step off a curb; these actions are controlled by the muscles in the arms and legs.

The heart and the digestive system are also made of muscle but a different kind, and their movements are not under voluntary control. When your heart beats or a meal is digested, it all happens automatically. Therefore, the heart and digestive system are not involved in ALS. Breathing also may seem to be involuntary. Remember, though, while you cannot stop your heart, you can hold your breath - so be aware that ALS may eventually have an impact on breathing.

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.
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 as well as several other drugs in clinical trials that hold promise.

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 journey or experiences.  There are medically documented cases of people in whom ALS ‘burns out,’ stops progressing or progresses at a very slow rate. No matter what your individual course or situation may be, The ALS Association and your medical team are here to help.

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