What is ALS? | ALS diagnosis | ALS treatment options | ALS medications | Best ALS medications | Side effects of ALS | ALS natural remedies | FAQ | Resources
You probably know ALS (amyotrophic lateral sclerosis) as Lou Gehrig’s disease. He was a major league baseball player for the New York Yankees who had been one of the greatest sluggers, with 493 home runs, before being forced to retire in 1939 after developing ALS. On Jul. 4, 1939, Gehrig announced to a crowd of 62,000 fans that he was leaving baseball because of the disease. He died less than 2 years later, on Jun. 2, 1941. He was 36 years old.
Most people had never heard of ALS at that point, and it became forever associated with Gehrig. Lou Gehrig isn’t the only famous person to develop ALS. Stephen Hawking, the renowned physicist; Jim “Catfish” Hunter, another professional baseball player; and Senator Jacob Javits, are just a few other notable people who had ALS.
Today, ALS affects as many as 30,000 people in the United States, and approximately 5,000 new cases are diagnosed each year. Although it can appear at any age, most diagnoses are made in people between the ages of 40 and 70 years old. It is most common in those older than the age of 60.
What is ALS?
ALS stands for amyotrophic lateral sclerosis, a progressive neurodegenerative disease that affects nerve cells in the brain and spinal cord, according to the ALS Association. Motor neurons start in the brain and are sent to the spinal cord, which sends them to muscles throughout the body. These motor neurons are responsible for voluntary muscle movement and muscle control.
In ALS, motor neurons progressively degenerate, or die, and as that happens the brain’s ability to send messages to the muscles via the spinal cord decreases until it eventually stops altogether. When the muscles stop receiving signals, they become weak and twitch. Eventually, they atrophy, or waste away. Voluntary muscle movement is used in speaking, chewing, running, writing, exercising, and most other activities requiring movement.
There are two main types of ALS:
- The most common type is called sporadic and it accounts for 90% to 95% of all cases. It can affect anyone, anywhere, at any time.
- Familial ALS accounts for 5% to 10% of all cases. This type is inherited. People with this type have a 50% chance of having a child with the gene mutation who could develop the disease.
A third type is found on the island of Guam, where it is known as lytigo-bodigo. Symptoms start when people are in their 40s and 50s. As the disease progresses, usually when the people are in their 60s, they develop shaking and lack of coordination, as is often seen in Parkinson’s disease. Finally, cognitive problems associated with dementia occur.
How is ALS diagnosed?
“ALS is typically diagnosed by neuromuscular neurologists,” according to Anthony Geraci, MD, Director of Neuromuscular Medicine at Northwell Health. “Early signs are very broad and include subtle weakness of a limb such as a mild foot drop that causes you to occasionally trip, or decreased dexterity of the fingers, for example when typing on a keyboard. Weakness can also start in larger muscle groups and the patient could first notice trouble getting out of a chair or raising their arms to brush their hair. Some patients have a first symptom of slightly slurred speech or coughing when swallowing liquids. Muscle twitches and cramps can also be an early sign.”
Everyone does not experience early ALS in the same way. Some people might have symptoms first in their arm or leg, called limb onset. Others might have initial symptoms in the face and throat muscles and have difficulty with speech or swallowing. When it appears in the facial muscles it is called bulbar onset. The early symptoms depend on which nerves and muscles are affected first. As the disease progresses, additional muscle groups are involved until all muscles in the body are affected.
For some people, the first sign of ALS is uncontrollable weeping or laughing—called the pseudobulbar affect. This symptom may continue throughout the progression of the disease.
Although anyone can develop ALS, there are a few risk factors:
- Age: The disease typically affects people between the ages of 40 and 70
- Gender: Men are slightly more likely to develop ALS, however as age increases, this difference lessens
- Ethnicity: People who are Caucasian and non-Hispanic are more likely to get the disease than other ethnicities.
Veterans are twice as likely to develop ALS than non-veterans. The reasons for this aren’t fully understood but it could possibly be because of exposure to lead, pesticides, and other environmental toxins. It is recognized as a service-connected disease by the U.S. Department of Veteran’s Affairs.
There is no single laboratory test to diagnose ALS. It is often diagnosed based on a detailed history of symptoms, observation by a physician, and by exclusion (ruling out other conditions). Your doctor will ask the following questions if ALS is suspected, according to Dr. Geraci:
- Do you trip when you walk?
- Do you have trouble rising from a chair?
- Do you have trouble grasping a pen and writing?
- Has your handwriting changed?
- Have you or those close to you noticed slurred speech?
- Have you been experiencing muscle twitching or cramps lately?
Before making a diagnosis, your doctor might suggest tests to rule out HIV, human T-cell leukemia, West Nile virus, multiple sclerosis, post-polio syndrome, and Kennedy’s disease.
Some of the tests performed during the diagnostic process include:
- Electromyography (EMG) to detect electrical activity of muscle fibers
- Nerve conduction study (NCS) to measure electrical activity of nerves and muscles
- Magnetic resonance imaging (MRI) to rule out spinal cord tumor, herniated disk in the neck, syringomyelia, or cervical spondylosis
- Blood and laboratory tests might be used to rule out other diseases
As the disease progresses, your doctor should continue to perform neurological exams on a regular basis to note the progression of the disease and determine how quickly symptoms are worsening.
Most people with ALS die from respiratory failure, usually within one to five years of the onset of symptoms. About 10% of people survive for 10 years or more.
ALS treatment options
There is no cure for ALS and there are no effective treatments that can halt or reverse the progression of the disease.
There are two medications—Tiglutik and Rilutek (riluzole)—used to treat the main symptoms of ALS. Other treatments and medications might be used to manage muscle cramps, excess saliva and phlegm, involuntary episodes of crying or laughing, pain, depression, sleep disturbances, and constipation.
In addition to medications, other therapies can be helpful:
Physical therapy can help enhance independence and safety. Low-impact exercise, such as walking, swimming, stationary bicycling strengthens muscles, improves cardiovascular health, and helps fight fatigue and depression. Physical movement also improves range of motion, prevents spasticity and minimizes the shortening of muscles.
Occupational therapy might be used to help with practical issues such as ramps, braces, walkers, wheelchairs. Therapists might also help with other assistive devices and provide ways to better manage daily tasks, such as dressing and hygiene.
Speech therapy can sometimes help with communication difficulties. Therapists might also introduce computer-based speech synthesizers and teach caregivers ways to communicate with their loved ones.
Nutritional support might be helpful when chewing and swallowing become difficult. Nutritional therapists can help caregivers plan and prepare meals that provide calories, fiber, and fluids while decreasing the chances of choking.
In the advanced stages, some people might require ventilators that inflate and deflate the lungs.
ALS research for more effective treatments is ongoing, including through clinical trials. Here are some resources to find a clinical trial near you:
There are currently two medications approved by the U.S. Food and Drug Administration (FDA) for the treatment of ALS.
Tiglutik and Rilutek (riluzole) are in a class of medications called benzothiazoles. This medication works by changing the activity of certain natural substances in the body that affect nerves and muscles, and helps slow down the progression of the disease and prolong survival up to 12 months. Although the mechanism for improvement is not fully understood, this medication does sometimes provide improvement in bulbar and limb function during the early stages of the disease, according to a report published in 2018. It also might extend life during the late stage of the disease.
Radicava (edaravone injection) is in a class of medications called antioxidants. It might work to slow nerve damage associated with ALS symptoms. This medication can only be taken intravenously and must be administered by a medical professional.
A third medication, Nuedexta (dextromethorphan HBr and quinidine sulfate), is in a class of medications called antiarrhythmics. It does not work on the muscle symptoms of ALS but can treat the pseudobulbar affect, sudden and frequent outbursts of crying or laughing, which may be seen in people with ALS.
What is the best medication for ALS?
Only three medications have been approved by the FDA for the treatment of ALS symptoms. Your doctor will work with you to determine what combination of medications is best for you.
|Best medications for ALS
||50 mg every 12 hours
||Weakness, dizziness, dry mouth
||60 mg over 60 minutes for 14 days, then 14 day rest, then 10 days on, 14 off
||Headache, rash, bruise easily
|Nuedexta (dextromethorphan HBr and quinidine sulfate)
||1 capsule (20 mg dextromethorphan and 10 mg quinidine) daily for 7 days, then 1 capsule every 12 hours
||Gastrointestinal issues, dry eyes, cough
What are common side effects of ALS medications?
Side effects of Tiglutik and Rilutek include:
- Dry mouth
- Sleep disturbances
- Rapid heart rate
Side effects of Radicava are:
- Bruise easily
- Difficulty walking
Nuedexta side effects include:
- Gas stomach pains
- Dry mouth
- Muscle spasms
- Difficult or painful urination
This is not a complete list of side effects. Consult with your healthcare provider or pharmacist to learn more about each medication.
What are the natural cures for ALS?
There are no cures, natural or medicinal, for ALS. “Lifestyle changes, such as diet and exercise have not been shown by rigorous scientific study to impact the course of ALS, however, patients with ALS often feel better by ‘taking control; of factors, such as diet, in a situation where they otherwise have no control. Alternative treatments, such as acupuncture, have been shown to increase an overall sense of well-being in patients with ALS and techniques, such as meditation, can help with anxiety. Tonic water can occasionally be effective in mitigating the muscle cramps patients can get with ALS, but prescription medications often work better,” according to Dr. Geraci.
There are also some aids that can help you maintain your independence as long as possible according to the Muscular Dystrophy Association.
Here are a few examples:
- Buttons: Use Velcro or snaps to close shirts. Use oversize buttons with loops instead of buttonholes. Use a button hook.
- Zipper pull: A hook that can be inserted into the zipper pull; it has a string with a handle to help in pulling the zipper up or down.
- Cylindrical foam: Improves grip on pens, pencils, razors, toothbrushes, and silverware.
- Rocker knives: Specially shaped knives allow you to cut foods with a rocking motion instead of a sawing motion
- Risers: Place under table legs to raise it to fit wheelchairs to sit close to the table.
- Wrist braces: Can support your wrist and stabilize your hand.
- Lift chairs: Have a rising seat to help you go from sitting to standing.
The ALS Association also suggests using electronic communication devices. These can be stand-alone or might work in conjunction with a computer, tablet, or cell phone.
Frequently asked questions about ALS
What are the very first signs of ALS?
Early symptoms of this motor neuron disease are often subtle and can easily be overlooked. As the disease progresses, symptoms become more pronounced and include:
- Muscle twitches (fasciculations) in arm, leg, shoulder, or tongue
- Muscle cramps
- Tight and stiff muscles
- Muscle weakness affected an arm, leg, neck or diaphragm
- Slurred and nasal speech
- Difficulty chewing or swallowing
- Difficulty breathing
Some people first notice difficulty with simple, daily tasks, such as buttoning a shirt, writing, or turning a key in a lock.
Symptoms do not always follow the same pattern. They might appear in the limbs for some people and others might have initial symptoms in the face and throat. As the disease progresses, additional muscle groups are involved until all muscles in the body are affected.
For some people, the first sign of ALS is the pseudobulbar affect, uncontrollable weeping or laughing.
How does a person get ALS? / What triggers ALS disease?
The causes of ALS are not completely understood; however, research indicates that there might be multiple factors that could contribute to ALS according to the Hospital for Special Surgery (HSS). These include damage or dysfunction on the cellular level including defective glutamate metabolism, mitochondrial dysfunction, an abundance of free radicals, cytoskeletal protect defects, and an accumulation of protein aggregates. Other factors can include:
- Autoimmune and inflammatory reactions
- Viral infections
- Gene mutations
What is the new drug for ALS?
The most recent FDA approval for the treatment of ALS was Tiglutik in 2018. This is a newer formulation of rilutek, which was first approved by the FDA in 1995.
The FDA also approved the medicine Radicava in 2017. At the time, this marked the first new treatment in 22 years.
What are the three types of ALS?
The three types of ALS are:
- Sporadic which accounts for 90% to 95% of all cases.
- Familial (hereditary) ALS accounts for 5% to 10% of all cases.
- Guamanian (also known as lytigo-bodigo) the initial symptoms affect the muscles, like ALS. As the disease progresses, people develop shaking and lack of coordination, and then cognitive problems associated with dementia occur.
What does Rilutek do for ALS?
Rilutek slows the progression of ALS and prolongs survival.
Can you exercise with ALS?
Some exercise might be beneficial for ALS patients. A study completed by the ALS Association and Johns Hopkins University found that exercises for stretching, endurance, and resistance were all safe and tolerated by those with ALS, although exercise was not found to significantly change disease progression. Exercise programs should be developed in conjunction with physical therapy.
Is ALS fatal? / Can you reverse ALS?
Most people with ALS die from respiratory failure, usually within one to five years of the onset of symptoms. About 10% of people survive for 10 or more years, according to the National Institutes of Neurological Disorders and Stroke.
Related resources for ALS