Amyotrophic lateral sclerosis (a-my-o-TROE-fik LAT-ur-ul skluh-ROE-sis), or ALS, is a progressive nervous system (neurological) disease that destroys nerve cells and causes disability.
ALS is often called Lou Gehrig's disease, after the famous baseball player who was diagnosed with it. ALS is a type of motor neuron disease in which nerve cells gradually break down and die.
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 ALS, and eventually the disease is fatal.
Early signs and symptoms of ALS include:
Difficulty walking or doing your normal daily activities
Tripping and falling
Weakness in your leg, feet or ankles
Hand weakness or clumsiness
Slurred speech or trouble swallowing
Muscle cramps and twitching in your arms, shoulders and tongue
Difficulty holding your head up or keeping good posture
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 progressively weaken. This eventually affects chewing, swallowing, speaking and breathing.
ALS doesn't usually affect your bowel or bladder control, your senses or your thinking ability. It's possible to remain actively involved with your family and friends.
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 may need an integrated team of doctors trained in many areas and other health care professionals to provide your care. This may prolong your survival and improve your quality of life.
Your team will help you select the right treatments for you. You always have the right to choose or refuse any of the treatments suggested.
Two medications are currently approved by the Food and Drug Administration for the treatment of ALS.
Riluzole (Rilutek) — This drug appears to slow the disease's progression in some people, perhaps by reducing levels of a chemical messenger in the brain (glutamate) that's often present in higher levels in people with ALS. Riluzole is taken as a pill and may cause side effects such as dizziness, gastrointestinal conditions and liver function changes.
Edaravone (Radicava) — The FDA approved edaravone in 2017 based on six-month clinical trial that showed it reduced the decline in daily functioning associated with ALS. The drug is given via intravenous infusion (typically 10-14 days in a row, once a month), and side effects may include bruising, gait disturbance, hives, swelling and shortness of breath.
Edaravone contains sodium bisulfite, which may cause serious allergic reactions in people with sulfite sensitivity.
Your doctor may also prescribe medications to provide relief from other symptoms, including:
Muscle cramps and spasms
Uncontrolled outbursts of laughing or crying
Breathing care. You'll eventually have more difficulty breathing as your muscles become weaker. Doctors may test your breathing regularly and provide you with devices to assist your breathing at night.
You may 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), and the tube is connected 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 may help maintain your cardiovascular fitness, muscle strength and range of motion for as long as possible.
A physical therapist can also help you adjust to a brace, walker or wheelchair and may suggest devices such as ramps that make it easier for you to get around.
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.
Occupational therapy. An occupational therapist can help you find ways to remain independent despite hand and arm weakness. Adaptive equipment can help you perform daily 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.
Occupational therapists also have a good understanding of how assistive technology and computers can be used, even if your hands are weak.
Speech therapy. Because ALS affects the muscles you use to speak, communication is an issue in advanced ALS. A speech therapist can teach you adaptive techniques to make your speech more clearly understood. Speech therapists can also help you explore other methods of communication, such as an alphabet board or simple 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 may help you communicate.
Nutritional support. Your team will work with you and your family members to ensure you're eating foods that are easier to swallow and meet your nutritional needs. You may eventually need a feeding tube.
Psychological and social support. Your team may include a social worker to help with financial issues, insurance, and getting equipment and paying for devices you may need. Psychologists, social workers and others may provide emotional support for you and your family.
Potential future treatments
Clinical studies on promising medications and treatments are occurring in ALS all the time.
Whether you are eligible for a clinical study depends on many factors related to your ALS and also whether there are ongoing studies enrolling patients. While many of these studies are promising, they are still only studies, and it isn't yet clear if these treatments will help people with ALS. Talk to your doctor about what might be available for you.