AMYOTROPHIC LATERAL SCLEROSIS

AMYOTROPIC LATERAL SCOLEROSIS (ALS)
Definition:
Amyotrophic lateral sclerosis (a-my-o-TROE-fik LAT-ur-ul skluh-ROE-sis), or ALS, is a progressive nervous system disease that affects nerve cells in the brain and spinal cord, causing loss of muscle control.

Description:
ALS is often called Lou Gehrig's disease, after the baseball player who was diagnosed with it. Doctors usually don't know why ALS occurs. Some cases are inherited.
ALS often begins with muscle twitching and weakness in a limb, or slurred speech. Eventually, ALS affects control of the muscles needed to move, speak, eat and breathe. There is no cure for this fatal disease.
Symptoms
Signs and symptoms of ALS vary greatly from person to person, depending on which neurons are affected. Signs and symptoms might include:

ALS often starts in the hands, feet or limbs, and then spreads to other parts of your body. As the disease advances and nerve cells are destroyed, your muscles get weaker. This eventually affects chewing, swallowing, speaking and breathing.
There's generally no pain in the early stages of ALS, and pain is uncommon in the later stages. ALS doesn't usually affect your bladder control or your senses.
Causes
ALS affects the nerve cells that control voluntary muscle movements such as walking and talking (motor neurons). ALS causes the motor neurons to gradually deteriorate, and then die. Motor neurons extend from the brain to the spinal cord to muscles throughout the body. When motor neurons are damaged, they stop sending messages to the muscles, so the muscles can't function.
ALS is inherited in 5% to 10% of people. For the rest, the cause isn't known.
Researchers continue to study possible causes of ALS. Most theories center on a complex interaction between genetic and environmental factors.
Risk factors
Established risk factors for ALS include:

Environmental factors, such as the following, might trigger ALS.

Complications
As the disease progresses, ALS causes complications, such as:
Breathing problems
Over time, ALS paralyzes the muscles you use to breathe. You might need a device to help you breathe at night, similar to what someone with sleep apnea might wear. For example, you may be given continuous positive airway pressure (CPAP) or bilevel positive airway pressure (BiPAP) to assist with your breathing at night.
Some people with advanced ALS choose to have a tracheostomy — a surgically created hole at the front of the neck leading to the windpipe (trachea) — for full-time use of a respirator that inflates and deflates their lungs.
The most common cause of death for people with ALS is respiratory failure. On average, death occurs within three to five years after symptoms begin. However, some people with ALS live 10 or more years.
Speaking problems
Most people with ALS develop trouble speaking. This usually starts as occasional, mild slurring of words, but becomes more severe. Speech eventually becomes difficult for others to understand, and people with ALS often rely on other communication technologies to communicate.
Eating problems
People with ALS can develop malnutrition and dehydration from damage to the muscles that control swallowing. They are also at higher risk of getting food, liquids or saliva into the lungs, which can cause pneumonia. A feeding tube can reduce these risks and ensure proper hydration and nutrition.
Dementia
Some people with ALS have problems with memory and decision-making, and some are eventually diagnosed with a form of dementia called frontotemporal dementia.
Diagnosis
Amyotrophic lateral sclerosis is difficult to diagnose early because it can mimic other neurological diseases. Tests to rule out other conditions might include:Electromyogram (EMG). Your doctor inserts a needle electrode through your skin into various muscles. The test evaluates the electrical activity of your muscles when they contract and when they're at rest.
Abnormalities in muscles seen in an EMG can help doctors diagnose or rule out ALS. An EMG can also help guide your exercise therapy.

Treatment
Treatments can't reverse the damage of amyotrophic lateral sclerosis, but they can slow the progression of symptoms, prevent complications, and make you more comfortable and independent.
You might need an integrated team of doctors trained in many areas and other health care professionals to provide your care. This might prolong your survival and improve your quality of life.
Your team will help you select the right treatments for you. You have the right to choose or refuse any of the treatments suggested.
Medications
The Food and Drug Administration has approved two drugs for treating ALS:

Your doctor might also prescribe medications to provide relief from other symptoms, including:

Therapies

Potential future treatments
Clinical studies on promising medications and treatments are occurring for ALS all the time.
Coping and support
Learning you have ALS can be devastating. The following tips may help you and your family cope:

Preparing for your appointment
You might first consult your family doctor about signs and symptoms of ALS. Then your doctor will probably refer you to a doctor trained in nervous system conditions (neurologist) for further evaluation.
What you can do
You might need many tests to diagnose your condition, which can be stressful and frustrating. These strategies might give you a greater sense of control.

What to expect from your doctor
Your family doctor will review your family's medical history and your signs and symptoms. Your neurologist and your family doctor might conduct a physical and neurological examination, which might include testing the following:

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