What You Need To Know About Amyotrophic Lateral Sclerosis
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What You Need To Know About Amyotrophic Lateral Sclerosis

There's no cure for ALS—but there is hope.

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that attacks the nerve cells controlling voluntary muscle movement in the brain and spinal cord. The ALS Association estimates that up to 30,000 Americans are affected. As ALS progresses, it kills the body’s nerve cells, resulting in weakened muscles, paralysis and ultimately, death. There is no cure.

The condition is sometimes called Lou Gehrig's disease, due to the widespread attention the baseball superstar brought to ALS after his 1939 diagnosis. Awareness of the disease became even greater in 2014, when Anthony Senerchia, a New Yorker diagnosed with ALS, inspired the Ice Bucket Challenge, a fundraising campaign that resulted in millions of dollars in donations for ALS research.

Symptoms and progression of ALS
Early symptoms occur in the muscles that are affected by ALS, but weakness and atrophy spread to the rest of the body as the disease progresses. Signs often begin with stiffness or weakness in one arm or leg, slurred speech or difficulty swallowing.

Patients diagnosed with ALS eventually lose the ability to walk, stand and use their extremities. As the disease progresses, patients require ventilator support to breathe. Due to weakening respiratory muscles, people affected by ALS often die of respiratory failure three to five years after the onset of the first symptom.

Despite this prognosis, about 10 percent of individuals diagnosed with ALS live for 10 or more years.

How ALS is diagnosed
There isn’t a single test that can diagnose ALS. Doctors begin by assessing a patient’s symptoms and reviewing their medical history. They’ll next run a series of tests, like electromyography, which detects electrical activity in the muscles, and nerve conduction study, which assesses the nerves' ability to send a signal. These tests are performed to eliminate the possibility of other diseases.

Other tests include regular neurologic exams, which can help determine whether muscle weakness, atrophy and other symptoms associated with ALS are getting progressively worse. This progression would be an indicator of ALS.

A diagnosis of ALS requires signs of both upper and lower motor neuron damage that cannot be attributed to another illness. Upper motor neurons in the brain send messages to lower motor neurons in the spinal cord, which control the muscles—including those in your arms, face and throat—that perform essential activities, like walking and chewing.

ALS and its early symptoms can resemble symptoms of other diseases, including multiple sclerosis, West Nile virus and HIV. Because of these similarities, it’s often recommended that patients seek multiple opinions.

Why do people develop ALS?
The cause of ALS is still unknown, but more than a dozen genetic mutations have been linked to the disease. It’s unclear how these mutations lead to motor neuron degeneration, but research suggests it’s caused by cellular defects.

Researchers are also studying factors such as exposure to toxic agents, physical trauma and chemical imbalances in the body. People with ALS have increased levels of glutamate, a chemical messenger in the brain that’s toxic to nerve cells.

Treatments for ALS
There is no cure for ALS, but the US Food and Drug Administration (FDA) has approved riluzole (Rilutek) for the treatment of the disease. The drug appears to slow the progression of ALS in some patients, but does not reverse any damage that has already occurred. It can prolong the life of some patients by a few months.

Other drugs and therapies may be prescribed to manage symptoms and keep patients functioning for as long as possible. These include speech and physical therapy, nutritional support and medications to manage pain, fatigue and muscle spasms.

New developments in research
When the ALS Ice Bucket Challenge dominated the internet in 2014, many criticized it as another example of “slacktivism.” But it seems the campaign inspired by Senerchia, who was diagnosed with ALS in 2003, worked. Before long, people all across the country took part. The rules were simple: post a video of yourself dumping ice water over your head for donations, and encourage others to do the same.

The 17 million people who accepted the challenge ultimately raised $115 million for the ALS Association. Donations have since funded multiple research projects, including Project MinE, in which scientists discovered a gene called NEK1. That discovery will help researchers develop new therapies to treat the disease.

Though Senerchia died in November 2017 of ALS, his legacy lives on; the ALS Association continues to use the Ice Bucket Challenge story to raise funds for research. "It's not what we take from life," he said, "but what we give back to others is what ultimately defines us."

This content was updated on November 30, 2017.

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