What Are Symptoms and Treatment of ALS? - dummies

What Are Symptoms and Treatment of ALS?

By Scott Barnes

ALS stands for amyotrophic lateral sclerosis, a progressively debilitating disease that causes muscle spasms; weakness from wasting away of muscles; and ultimately, strained speaking, swallowing, and breathing. The disease goes by a few other names, including motor neurone disease (MND), Charcot disease, and Lou Gehrig’s disease, the latter of which refers to the legendary first baseman who died two years after his ALS diagnosis.

ALS is a neurodegenerative disease, affecting the nerve cells in the brain and spinal cord. Motor neurons reach from your brain to your spinal cord and run throughout your body. When affected by ALS, these motor neurons degenerate until they eventually die, at which point the brain can no longer control the muscles served by the neurons. As muscles stop receiving messages from motor neurons, tissues begin to shrink.

The cause of ALS is currently unknown. Between 5 and 10 percent of cases may be genetic, but all other cases occur randomly. Researchers are studying several potential triggers to this disease, the most likely of which are

  • Gene mutation

  • Chemical imbalance

  • Autoimmune disorder

Early symptoms of ALS

The first symptoms of ALS are usually weakness or noticeable shrinking of muscles, or cramping or stiffness of specific muscles or sets of muscles, in an arm or leg. Other ALS symptoms may include trouble with swallowing, and slurred speech or a change in speech tone. (Often, the voice develops a more nasal tone or becomes notably quieter.)

Those who first develop symptoms in a leg may notice that they stumble or trip more than usual, or that they feel awkward when they walk or run. Sometimes, people sense that one of their feet seems to have developed “a mind of its own,” dragging on the ground as they walk.

Symptoms that first show up in an arm often involve loss of dexterity performing once-simple tasks, such as buttoning a shirt, typing, turning a key, or using a fork.

A smaller portion of ALS patients experience symptoms in the respiratory system initially, most commonly, difficulty in breathing.

In rare cases, the symptoms of ALS remain confined to one limb for a long period of time, or even for the whole length of the illness. Most people with ALS experience a slow degeneration of the muscles throughout their bodies. Over time, ALS patients have more and more difficulty moving, swallowing, and speaking or forming words. The reflexes become exaggerated, which leads to involuntary muscle twitches or spasms, and the gag reflex may become hypersensitive. Some patients laugh, cry, or smile uncontrollably, as motor neurons continue to shut down.

Late stages of ALS

As ALS progresses, patients may lose the ability to walk or use their hands and arms, until they eventually become partially or totally paralyzed. Loss of the ability to speak and swallow food can prompt use of a feeding tube. Breathing difficulties often become so severe that patients require the use of a portable ventilator.

The muscles that control the bladder and bowel, as well as those that control eye movement, typically are not affected by ALS until the final stages of the disease. The brain is also spared, so although an ALS patient may have no control over her body, she can think clearly.

Most who suffer from ALS die from respiratory failure within three to five years of the first symptoms. Around 4 percent of ALS patients survive longer than ten years. In rare cases, ALS advances to a point where it seems to stabilize, or begins to progress at a very slow rate.

The two most notable long-term survivors of ALS are guitarist Jason Becker, who was diagnosed in 1989, and theoretical physicist Stephen Hawking, who was diagnosed in 1963 at age 21. As of summer 2014, both Becker and Hawking were alive and still working, with the help of specially designed communication systems.

For reasons unknown at this time, ALS progression tends to be slower in those who are younger than 40 when symptoms appear; who are mildly obese at the time of diagnosis; and whose first symptoms are what doctors call upper motor neuron symptoms, which include exaggerated reflexes, an increasingly sensitive gag reflex, or tight and stiff muscles.

Treatment of ALS

Currently, there is no cure or treatment plan that halts or reverses the progression of ALS. The U.S. Food and Drug Administration has approved one drug that slows the progression of the disease, and several new drugs are in clinical trials. Many medical devices and therapies can manage the symptoms of ALS and help those with the disease to live longer and to live as independently as possible.

If you are diagnosed with ALS, remember that this is a highly variable disease — no two cases progress exactly the same, and your healthcare providers will tailor a treatment program specifically for you.

In the summer of 2014, social media sparked a fundraising phenomenon, “The ALS Ice Bucket Challenge,” which helped raise almost $42 million (nearly 2,000% over the same one-month period in the previous year) in donations for the ALS Association. The rules of the challenge required participants to be filmed having a bucket of ice water poured on their heads within 24 hours of their nomination, or forfeit by making a $100 charitable donation to the ALS Association. Celebrities who contributed monies or videos of themselves being doused in ice cubes and chilly water include Bill Gates, Jennifer Lopez, LeBron James, Justin Timberlake, Drake, Martha Stewart, Lady Gaga, former president George W. Bush, Oprah Winfrey, Kaley Cuoco, and many more.