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), known as ALS, is a nervous system disease that affects nerve cells in the brain and spinal cord. ALS causes loss of muscle control. The disease gets worse over time.
ALS is often called Lou Gehrig's disease after the baseball player who was diagnosed with it. The exact cause of the disease is still not known. A small number of cases are inherited.
ALS often begins with muscle twitching and weakness in an arm or leg, trouble swallowing 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 of ALS vary from person to person. Symptoms depend on which nerve cells are affected. ALS generally begins with muscle weakness that spreads and gets worse over time. Symptoms might include:
ALS often starts in the hands, feet, arms or legs. Then it spreads to other parts of the body. Muscles get weaker as more nerve cells die. This eventually affects chewing, swallowing, speaking and breathing.
There's generally no pain in the early stages of ALS. Pain also is not common in the later stages. ALS doesn't usually affect bladder control. It also usually doesn't affect the senses, including the ability to taste, smell, touch and hear.
ALS affects the nerve cells that control voluntary muscle movements such as walking and talking. These nerve cells are called motor neurons. There are two groups of motor neurons. The first group extends from the brain to the spinal cord to muscles throughout the body. They're referred to as upper motor neurons. The second group extends from the spinal cord to muscles throughout the body. They're referred to as lower motor neurons.
ALS causes both groups of motor neurons to gradually deteriorate and then die. When motor neurons are damaged, they stop sending messages to the muscles. As a result, the muscles can't function.
For about 10% of people with ALS, a genetic cause can be identified. For the rest, the cause is not known.
Researchers continue to study possible causes of ALS. Most theories center on a complex interaction between genes and factors in the environment.
Established risk factors for ALS include:
Environmental factors, such as the following, have been associated with an increased risk of ALS.
As the disease progresses, ALS causes complications, such as:
Over time, ALS leads to weakness of the muscles used to breathe. People with ALS might need a device such as a mask ventilator to help them breathe at night. The device is similar to what someone with sleep apnea might wear. This type of device supports the person's breathing through a mask worn over the nose, the mouth or both.
Some people with advanced ALS choose to have a tracheostomy. This is a surgically created hole at the front of the neck leading to the windpipe. A ventilator may work better on a tracheostomy than on a mask.
The most common cause of death for people with ALS is breathing failure. Half of people with ALS die within 14 to 18 months of diagnosis. However, some people with ALS live 10 years or longer.
Most people with ALS develop weakness of the muscles used to form speech. This usually starts with slower speech and occasional slurring of words. It then becomes harder to speak clearly. This can progress to the point that others can't understand the person's speech. Other forms of communication and technology are used to communicate.
People with ALS can develop weakness of the muscles involved with swallowing. This can lead to malnutrition and dehydration. 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 language and decision-making. Some are eventually diagnosed with a form of dementia called frontotemporal dementia.
Amyotrophic lateral sclerosis, known as ALS, can be hard to diagnose early because it can have symptoms similar to other diseases. Tests to rule out other conditions or help diagnose ALS might include:
Treatments can't reverse the damage of ALS, but they can slow the progression of symptoms. They also can help prevent complications and make you more comfortable and independent.
You might need a team of health care providers and doctors trained in many areas to provide your care. The team works together to prolong your survival and improve your quality of life.
Your team works to 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 three medicines for treating ALS:
Your health care provider also might prescribe treatments for relief of other symptoms, including:
When ALS affects your ability to breathe, speak and move, therapies and other forms of support can help.
Breathing care. Most people with ALS eventually have more trouble breathing as muscles weaken. Your health care provider might test your breathing regularly and provide devices known as mechanical ventilation to assist your breathing at night.
You might choose to use a ventilator with a mask that can easily be applied and removed. This is known as noninvasive ventilation. Some people eventually have surgery that creates a hole at the front of the neck leading to their windpipe. This is called a tracheostomy. A tube inserted into the hole connects to a respirator to help them breathe. Sometimes people with ALS who have a tracheostomy also have a type of surgery called a laryngectomy. This surgery prevents food from entering the lungs.
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 also can help improve your sense of well-being. Appropriate stretching can help prevent pain and help your muscles function at their best.
A physical therapist also can help you overcome weakness by using a brace, walker or wheelchair. The therapist 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 also can 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 also can help you find other ways to communicate. These may include using a smart phone app, alphabet board, or pen and paper.
Ask your therapist about the possibility of recording your own voice to be used by a text-to-speech application.
Based on the current understanding of ALS, researchers are conducting clinical studies on promising medicines and treatments.
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. It also lessens the burden for your loved ones. With the help of your health care provider, hospice nurse or social worker, you can decide whether you want certain life-extending procedures.
You also can 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.
Your primary care provider may be the first to recognize the symptoms of ALS. Your provider will likely refer you to a doctor trained in nervous system conditions, known as a neurologist, to establish a diagnosis.
You might need many tests to diagnose your condition. The diagnosis process can be stressful and frustrating. These strategies might give you a greater sense of control.
Your primary care provider will likely review your family's medical history and your symptoms. Your neurologist and your primary care provider might conduct a physical and neurological exam. This might include testing your: