This progressive nervous system disease, also known as Lou Gehrig's disease, destroys nerve cells.
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.
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.
Signs and symptoms of ALS vary greatly from person to person, depending on which neurons are affected. It generally begins with muscle weakness that spreads and gets worse over time. 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.
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.
Established risk factors for ALS include:
Environmental factors, such as the following, might trigger ALS.
As the disease progresses, ALS causes complications, such as:
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 a bilevel positive airway pressure (BiPAP) device to help with your breathing at night. This type of device supports your breathing through a mask worn over your nose, your mouth or both.
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.is respiratory failure. On average, death occurs within 3 to 5 years after symptoms begin. However, some people with ALS live 10 or more years.
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.
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.
Some people with ALS have problems with memory and decision-making, and some are eventually diagnosed with a form of dementia called frontotemporal dementia.
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.
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.
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:
Breathing care. You'll eventually have more difficulty breathing as your muscles weaken. Doctors might test your breathing regularly and provide you with devices to assist your breathing at night.
You can choose mechanical ventilation to help you breathe. Doctors insert a tube in a surgically created hole at the front of your neck leading to your windpipe (tracheostomy) that connects to a respirator.
Physical therapy. A physical therapist can address pain, walking, mobility, bracing and equipment needs that help you stay independent. Practicing low-impact exercises can help maintain your cardiovascular fitness, muscle strength and range of motion for as long as possible.
Regular exercise can also help improve your sense of well-being. Appropriate stretching can help prevent pain and help your muscles function at their best.
A physical therapist can also help you adjust to a brace, walker or wheelchair and might suggest devices such as ramps that make it easier for you to get around.
Occupational therapy. An occupational therapist can help you find ways to remain independent despite hand and arm weakness. Adaptive equipment can help you perform activities such as dressing, grooming, eating and bathing.
An occupational therapist can also help you modify your home to allow accessibility if you have trouble walking safely.
Speech therapy. A speech therapist can teach you adaptive techniques to make your speech more understandable. Speech therapists can also help you explore other methods of communication, such as an alphabet board or pen and paper.
Ask your therapist about the possibility of borrowing or renting devices such as tablet computers with text-to-speech applications or computer-based equipment with synthesized speech that can help you communicate.
Clinical studies on promising medications and treatments are occurring for ALS all the time.
Learning you have ALS can be devastating. The following tips may help you and your family cope:
Make decisions now about your future medical care. Planning for the future allows you to be in control of decisions about your life and your care. With the help of your doctor, hospice nurse or social worker, you can decide whether you want certain life-extending procedures.
You can also decide where you want to spend your final days. You might consider hospice care options. Planning for the future can help you and your loved ones calm anxieties.
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.
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.
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:
October 28th, 2021
