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ALS Fast Facts

In a given year, about 30,000 Americans suffer from amyotrophic lateral sclerosis (ALS). Approximately 5,600 new cases of the disease are diagnosed each year. ALS most commonly develops in patients between the ages of 40 and 65, and the risk increases with age.

Men under the age of 65 are more likely than women to develop ALS. After the age of 70, both men and women are equally likely to develop the disease.

About 10% of people who develop ALS have a genetically inherited form of the disease, and children of these patients have a 50% chance of developing the disease themselves.

More than half of those diagnosed with ALS will die within two to five years of the onset of symptoms. About 20% will survive for five years or more, and about 10% will survive for more than 10 years.

On average, it takes about 12 months from the initial onset of symptoms until the disease is diagnosed.

What is Amyotrophic Lateral Sclerosis (ALS)?

Amyotrophic lateral sclerosis, commonly referred to as ALS, is a disease that affects nerve cells in the brain and the spinal column. Specifically, the disease attacks motor neurons, the nerve cells that control the movement of muscles throughout the body. ALS causes the neurons to slowly die over time, and as they do, the patient loses the ability to control or use his or her muscles.

ALS is sometimes called Lou Gehrig’s Disease in reference to the famous baseball player who developed the disease in the 1930s. Unfortunately, there is no cure for the disease, and it is eventually fatal. However, some treatments may extend the life expectancy of some patients.

Symptoms

The early signs of ALS are often so subtle that they go unnoticed, which can lead to the disease going undiagnosed for months. The earliest symptoms are typically muscle weakness, stiffness, cramping, and twitching; these initial symptoms tend to affect the hands, feet, and limbs. As the disease progresses, the symptoms will spread to other parts of the body and affect other motor functions, such as chewing, swallowing, speaking, and breathing.

*The medical information we gather and publish is vetted and intended to be up to date, accurate and express a spectrum of recognized scientific and medical points of view. The information comes from a nucleus of informed scientists, medical doctors, peer-reviewed scientific journals and the National Institute of Health. Please note, differing points of view among scientists and physicians are common. Every effort is employed to ensure the accuracy of these different points of view. That notwithstanding, it is incumbent on persons using this information to consult with his/her physician before reaching any conclusions. Our medical information and publications are not intended to be a substitute for consultation with one’s physician.

What Causes Amyotrophic Lateral Sclerosis (ALS)?

The precise cause of ALS has not yet been determined. The disease occurs in more than one form, and it’s possible that different forms of the disease may have different causes. One common characteristic of different forms of the disease is abnormal changes (called mutations) in certain genes in the patient’s cells. In some cases, these changes are passed on from affected parents to their children. In other cases, the gene mutations may be triggered by external environmental factors.

Scientists don’t yet know exactly which genes are involved in the development of ALS, nor do they know for certain the ways in which gene mutations lead to the development of the disease.

  • Some mutations seem to interfere with the communication between nerve cells and muscles.
  • Other mutations may interfere with the function of the nerve cells themselves and cause them to die.
  • Still, other mutations may cause toxic substances to accumulate in nerve cells, leading to damage and cell death.

*The medical information we gather and publish is vetted and intended to be up to date, accurate and express a spectrum of recognized scientific and medical points of view. The information comes from a nucleus of informed scientists, medical doctors, peer-reviewed scientific journals and the National Institute of Health. Please note, differing points of view among scientists and physicians are common. Every effort is employed to ensure the accuracy of these different points of view. That notwithstanding, it is incumbent on persons using this information to consult with his/her physician before reaching any conclusions. Our medical information and publications are not intended to be a substitute for consultation with one’s physician.

Is Amyotrophic Lateral Sclerosis (ALS) Hereditary?

There are two broad types of ALS: one has a definite connection to family history; the other seems to be linked to non-inherited factors.

  • Familial ALS. This form of the disease affects approximately 10% of patients and is clearly linked to genetic family history. In these cases, a mutation in a particular gene appears to substantially increase the risk of developing the disease, and a person with the mutation has a 50% chance of passing the mutation on to his or her child. In families where the mutation for ALS is present, multiple family members across generations are likely to have had ALS or a related disease called frontotemporal dementia (FTD).
    Familial ALS often develops earlier than sporadic ALS, with initial onset occurring in the patient’s late 40s or early 50s. In rare cases, the disease can occur in teenagers or children.
  • Sporadic ALS. This is the most common type of ALS, affecting about 90% of patients with the disease. This form of the disease doesn’t seem to be linked to family history and is instead probably caused by a combination of gene mutations and external environmental factors. The most common age range for the onset of sporadic ALS is between the late 50s and early 60s.

*The medical information we gather and publish is vetted and intended to be up to date, accurate and express a spectrum of recognized scientific and medical points of view. The information comes from a nucleus of informed scientists, medical doctors, peer-reviewed scientific journals and the National Institute of Health. Please note, differing points of view among scientists and physicians are common. Every effort is employed to ensure the accuracy of these different points of view. That notwithstanding, it is incumbent on persons using this information to consult with his/her physician before reaching any conclusions. Our medical information and publications are not intended to be a substitute for consultation with one’s physician.

How is Amyotrophic Lateral Sclerosis (ALS) Detected?

Doctors often miss the earliest signs of ALS, either failing to diagnose the disease entirely or mistaking the symptoms for another problem. The initial sign of ALS is muscle weakness, but the weakness is not always in the same part of the body. The symptoms might also look like those of different, less severe problems such as a pinched nerve or carpal syndrome. Consequently, ALS is difficult to spot early on, even for medical professionals. Early detection is even more unlikely because the disease is relatively uncommon, and most doctors don’t have experience diagnosing it.

Early signs of ALS include:

  • Muscle weakness. There is usually no pain associated with the weakness, and the location of the weakness varies from patient to patient. Common areas for the weakness to begin are the hands, feet, arms, and legs. Patients might also have trouble speaking or swallowing early on.
  • Muscle cramps
  • Uncontrollable muscle twitches
  • Falling or tripping
  • Difficulty grasping or holding objects
  • Fatigue in the extremities
  • Slurred speech
  • Difficulty swallowing
  • Uncontrollable laughing, crying or yawning

*The medical information we gather and publish is vetted and intended to be up to date, accurate and express a spectrum of recognized scientific and medical points of view. The information comes from a nucleus of informed scientists, medical doctors, peer-reviewed scientific journals and the National Institute of Health. Please note, differing points of view among scientists and physicians are common. Every effort is employed to ensure the accuracy of these different points of view. That notwithstanding, it is incumbent on persons using this information to consult with his/her physician before reaching any conclusions. Our medical information and publications are not intended to be a substitute for consultation with one’s physician.

How is Amyotrophic Lateral Sclerosis (ALS) Diagnosed?

When your doctor suspects that ALS might be the cause of early symptoms, he or she will conduct a variety of tests and exams. There is no single test or exam to detect ALS. Much of the diagnostic process is designed to rule out ALS by detecting other problems that could be causing symptoms, rather than directly diagnosing ALS itself.

  • Laboratory tests. Tests of your blood and urine will not necessarily confirm a diagnosis of ALS, but tests may be able to rule out other conditions that could be causing your symptoms.
  • Electromyogram (EMG). This test uses electrodes to measure the electrical activity in your muscles as they work. The test can be used to detect abnormalities in muscle function that support a diagnosis of ALS.
  • Imaging tests. Magnetic resonance imaging (MRI) scans can detect abnormalities in your brain, spinal column, or other parts of your body. These tests may be used to rule out ALS by revealing another condition that’s causing your symptoms.
  • Spinal tap. This procedure removes and tests a small amount of the fluid that protects your brain and spinal column. The test can often detect viral infections or inflammation in the brain.
  • Nerve conduction tests. These tests measure how well your nerves are able to communicate with your muscles. These tests may detect nerve damage or disorders other than ALS that could be causing symptoms.
  • Muscle biopsy. This test, a small amount of muscle tissue is removed and tested. The biopsy is typically used to detect other diseases and to rule out ALS.

*The medical information we gather and publish is vetted and intended to be up to date, accurate and express a spectrum of recognized scientific and medical points of view. The information comes from a nucleus of informed scientists, medical doctors, peer-reviewed scientific journals and the National Institute of Health. Please note, differing points of view among scientists and physicians are common. Every effort is employed to ensure the accuracy of these different points of view. That notwithstanding, it is incumbent on persons using this information to consult with his/her physician before reaching any conclusions. Our medical information and publications are not intended to be a substitute for consultation with one’s physician.

How is Amyotrophic Lateral Sclerosis (ALS) Treated?

There is no cure for ALS, and no treatment can undo the damage that the disease does to the body’s nerve cells. Treatment plans, therefore, focus on slowing the progression of the disease, preventing complications associated with the disease, and easing discomfort caused by the disease’s symptoms.

  • Drug treatments. Two drugs have been approved for use in the treatment of ALS, and both drugs have shown success in slowing the progression of the disease. Riluzole is an oral medication that may increase life expectancy by three to six months. Edaravone is administered intravenously; it also may increase life expectancy, but studies have not yet shown by how long.
  • Other medications may be used to treat symptoms such as pain, depression, muscle cramps, fatigue, constipation, sleep disruptions, and uncontrollable crying or laughing.
  • Physical therapy can help patients to remain mobile and independent for a longer time as the disease progresses.
  • Occupational therapy can help a patient learn how to remain independent as muscle function deteriorates.
  • Speech therapy can help to maintain the ability to speak for as long as possible. Therapies can also help the patient learn how to use adaptive technologies to communicate as speech becomes more difficult.
  • Breathing therapies can help to ease the discomfort as breathing becomes more difficult.

*The medical information we gather and publish is vetted and intended to be up to date, accurate and express a spectrum of recognized scientific and medical points of view. The information comes from a nucleus of informed scientists, medical doctors, peer-reviewed scientific journals and the National Institute of Health. Please note, differing points of view among scientists and physicians are common. Every effort is employed to ensure the accuracy of these different points of view. That notwithstanding, it is incumbent on persons using this information to consult with his/her physician before reaching any conclusions. Our medical information and publications are not intended to be a substitute for consultation with one’s physician.

How Does Amyotrophic Lateral Sclerosis (ALS) Progress?

The muscle weakness that’s characteristic of early ALS will eventually spread to other parts of the body, resulting in weakness and paralysis. The sufferer will have increased difficulty moving, speaking, swallowing, and breathing. Usually, patients will eventually be unable to stand, walk, or use their arms and legs, and they will eventually require mechanical assistance to breathe.

Although there is some evidence that a form of dementia is present in some cases of ALS, the cognitive function does not usually decline as muscle deterioration progresses. The fact that the sufferer’s mental functions remain intact as their physical abilities decline often leads to depression and/or anxiety.

*The medical information we gather and publish is vetted and intended to be up to date, accurate and express a spectrum of recognized scientific and medical points of view. The information comes from a nucleus of informed scientists, medical doctors, peer-reviewed scientific journals and the National Institute of Health. Please note, differing points of view among scientists and physicians are common. Every effort is employed to ensure the accuracy of these different points of view. That notwithstanding, it is incumbent on persons using this information to consult with his/her physician before reaching any conclusions. Our medical information and publications are not intended to be a substitute for consultation with one’s physician.

How is Amyotrophic Lateral Sclerosis (ALS) Prevented?

The direct cause of ALS is not known, especially in cases of sporadic ALS in which heredity doesn’t seem to play a role. However, research has identified some risk factors that may increase the risk of developing the disease. The risk factors include:

  • Smoking. Some research has suggested a link between smoking and ALS, especially in post-menopausal women.
  • Military service. Veterans of the military are twice as likely to develop ALS as those who have not served. The increased risk isn’t connected with any type of service, a branch of service, combat experience, or location of service. Researchers have not yet been able to determine what aspect of service causes the increased risk.
  • Exposure to toxins. Studies have suggested links between ALS and exposure to some toxins, including lead, but a definite link between the disease and anyone toxin has not yet been established.

*The medical information we gather and publish is vetted and intended to be up to date, accurate and express a spectrum of recognized scientific and medical points of view. The information comes from a nucleus of informed scientists, medical doctors, peer-reviewed scientific journals and the National Institute of Health. Please note, differing points of view among scientists and physicians are common. Every effort is employed to ensure the accuracy of these different points of view. That notwithstanding, it is incumbent on persons using this information to consult with his/her physician before reaching any conclusions. Our medical information and publications are not intended to be a substitute for consultation with one’s physician.

Amyotrophic Lateral Sclerosis (ALS) Caregiver Tips

A diagnosis of ALS is devastating both for the patient and for their loved ones and caregivers. The disease is fatal, and coping with that fact can make dealing with its symptoms and complications even more difficult than they would be otherwise.

  • Make room for grief. Therapies and treatments can make the grieving process take a back seat to the daily routine. But giving the grieving process the time and space it deserves is an important part of coping with the disease.
  • Plan ahead. The progression of the disease will make communication and decision-making difficult in the years ahead. Make decisions about medical plans, end-of-life considerations, and financial issues now.
  • Recognize the positive. ALS does not usually cause mental decline, and many sufferers have happy, joy-filled experiences for years following their diagnosis. Don’t let the physical symptoms rule out the possibilities.
  • Get help. Find a support group for ALS caregivers to help you maintain your mental and physical health as you deal with the demands of caregiving.

*The medical information we gather and publish is vetted and intended to be up to date, accurate and express a spectrum of recognized scientific and medical points of view. The information comes from a nucleus of informed scientists, medical doctors, peer-reviewed scientific journals and the National Institute of Health. Please note, differing points of view among scientists and physicians are common. Every effort is employed to ensure the accuracy of these different points of view. That notwithstanding, it is incumbent on persons using this information to consult with his/her physician before reaching any conclusions. Our medical information and publications are not intended to be a substitute for consultation with one’s physician.

Amyotrophic Lateral Sclerosis (ALS) Brain Science

Researchers are looking for the causes of ALS, for an understanding of how the disease affects the brain, and for effective treatments for the disease. Current studies include:

  • Researchers suspect that a brain-cell protein called membralin might play a role in the development of ALS. Scientists don’t know exactly what membralin does in the brain’s nerve cells, but they have found evidence that a deficiency of protein may be a cause of ALS and other degenerative nerve diseases. Their studies suggest that gene therapies that increase levels of membralin may have potential as an ALS treatment.
  • One clinical study is currently testing a drug that takes a new approach to treat ALS. The drug aims to improve muscle function rather than improving the communication between nerves and muscles. The hope is that the drug will help muscles to work more efficiently to make up for the weakness caused by ALS nerve damage. The drug seems to be particularly effective at helping the muscles that control breathing, and patients may be able to breathe better for longer as a result.

*The medical information we gather and publish is vetted and intended to be up to date, accurate and express a spectrum of recognized scientific and medical points of view. The information comes from a nucleus of informed scientists, medical doctors, peer-reviewed scientific journals and the National Institute of Health. Please note, differing points of view among scientists and physicians are common. Every effort is employed to ensure the accuracy of these different points of view. That notwithstanding, it is incumbent on persons using this information to consult with his/her physician before reaching any conclusions. Our medical information and publications are not intended to be a substitute for consultation with one’s physician.

Amyotrophic Lateral Sclerosis (ALS) Research

Scientists are working on several research projects to expand on what is known about Amyotrophic Lateral Sclerosis (ALS). The research will improve knowledge about the factors that increase the risk for ALS, as well as the causes, and best treatments and cures, and will aid people living with ALS and their caregivers.

We are currently gathering the information required to support projects such as Imaging and BioFluid Biomarkers in Amyotrophic Lateral Sclerosis (TRACK-ALS), Investigating Complex Neurodegenerative Disorders Related to Amyotrophic Lateral Sclerosis and Frontotemporal Dementia, and Cervical Electrical Stimulation for ALS.

*The medical information we gather and publish is vetted and intended to be up to date, accurate and express a spectrum of recognized scientific and medical points of view. The information comes from a nucleus of informed scientists, medical doctors, peer-reviewed scientific journals and the National Institute of Health. Please note, differing points of view among scientists and physicians are common. Every effort is employed to ensure the accuracy of these different points of view. That notwithstanding, it is incumbent on persons using this information to consult with his/her physician before reaching any conclusions. Our medical information and publications are not intended to be a substitute for consultation with one’s physician.

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