Amyotrophic lateral sclerosis, commonly referred to as ALS or Lou Gehrig’s disease, is a type of progressive neurodegenerative disorder that causes continual degeneration of the nerve cells located within the brain and spinal cord. Due to decline in nourishment caused by this degeneration, motor neurons begin to die and thus, voluntary movements and muscle action are no longer possible. After the death of motor neurons, the areas that send these signals begin the process of sclerosis—the stiffening or hardening of tissue. To help you better understand some of the causes, symptoms, and treatment options available, here’s everything you need to know about amyotrophic lateral sclerosis (ALS).
What is Amyotrophic Lateral Sclerosis?
Amyotrophic lateral sclerosis is a rare type of progressive disease that attacks the nerve cells in the central nervous system. Specifically, it attacks the nerve cells that are responsible for voluntary muscle movement. The progressiveness of this disease eventually leads to complete loss of muscle control. It is a terminal disease, with a prognosis of about three to five years after symptoms begin. Although rare, there are a few people who have lived with ALS for 10 years or more. Amyotrophic lateral sclerosis is a type of motor neuron disease, in that your body begins losing control of the neurons that are responsible for communication between the brain and the body. Eventually, these motor neurons weaken, twitch, and atrophy.
Progression of ALS Symptoms Over Time
Many of the early symptoms of ALS are overlooked due to their original subtlety and similarity to other spinal cord injuries. Symptoms may be seen as simple side effects from daily life, exercises, or illness. However, as symptoms progress, atrophy becomes more obvious and harder to attribute to other causes. Therefore, symptoms of amyotrophic lateral sclerosis tend to be categorized depending on the progression of the disease.
Early Signs of Amyotrophic Lateral Sclerosis
Some of the early symptoms of amyotrophic lateral sclerosis include muscle twitching, cramps, stiffness, muscle weakness across the limbs, neck, or diaphragm, slurred speech, and difficulties with chewing or swallowing. During the original onset of ALS, some individuals tend to notice the first signs or symptoms in their hands or arms. This leads to problems with simple tasks such as writing or opening a door. This can also occur in one of the legs, creating a higher risk of stumbling, limping, or tripping. If symptoms begin in the limbs, it’s considered “limb onset” ALS; if symptoms first appear as speech or swallowing problems, it’s called “bulbar onset” ALS.
Late-Onset ALS Symptoms
As ALS progresses, the symptoms tend to intensify and spread to other areas of the body. This leads to problems with voluntary movements, especially in terms of walking, speaking, swallowing, or even breathing. While this progression can vary between individuals, the end-result is eventually the same—loss of voluntary muscle control. While someone may experience late-onset symptoms of ALS, mental functioning is not usually affected until later, and they are fully aware of what is occurring within their body. This can lead to high rates of depression and anxiety, until eventual cognitive decline. Late stages of ALS patients require the use of ventilators for breathing assistance and some individuals experience dementia or problems with decision-making.
Causes of ALS
While the exact cause of ALS is unknown, there are a few factors that researchers believe increase an individual’s risk. Glutamate build-up around the nerve cells in the brain may be a contributor to the development of ALS along with various immune system problems, mitochondria issues, and oxidative stress. However, the main type of ALS is considered sporadic.
Sporadic ALS
Sporadic ALS occurs when there is no clear cause for the development of the disorder. This accounts for up to 95% of the people diagnosed with ALS. This seemingly random development of such a serious disorder can be scary for those with certain risk factors, which is why it’s important to see your doctor regularly and discuss your concerns.
Familial ALS
The remaining 5% to 10% of ALS cases are considered familial or caused by inherited genes. If one parent has ALS, there’s a 50% chance that their offspring will inherit the gene and develop amyotrophic lateral sclerosis during their lifetime. Currently, there are over 12 genes linked to ALS. The most common of these include SOD1, C9ORF72, and SPTLC1 genes.
In addition to the genetic factors that damage motor neurons, environmental factors may also play a role. When an individual has been exposed to toxic or infectious substances, overexertion of physical activity, physical trauma, viruses, and poor diet, they may increase their risk of developing ALS. This was theorized based on the increase of ALS diagnosis in war veterans and certain athletes.
ALS most commonly occurs in Caucasian males between the ages of 40 and 65, however it can occur at any time in your life. Smoking has also been shown to increase the risk of ALS, along with geographic location (particularly in certain areas of Guam and Japan) and your occupation.
Diagnosing Amyotrophic Lateral Sclerosis
Like many degenerative diseases, diagnosing ALS can be difficult. This is because symptoms are very similar to other neurological diseases. To receive a proper diagnosis, many doctors will perform a variety of tests to rule out other, more easily testable conditions. Testing begins with a comprehensive medical exam, questionnaire, and examining your family medical history. An electromyogram (EMG) will be performed to evaluate the strength and activity of your muscles during both contraction and while they’re at rest. These results can be used later for more targeted treatments.
Your doctor may also perform a nerve conduction study to measure the efficacy of your motor neurons and their ability to communicate with different areas in your body. This can help classify the extent of nerve damage or disease. You may also need to undergo an MRI along with blood tests, urine tests, and a spinal tap.
Treatment Options for ALS
Unfortunately, there is currently no cure for ALS and once damage occurs, it cannot be reversed. However, with the proper lifestyle changes, medications, and therapy, it is possible to decrease the progression of ALS symptoms, thus reducing the risk and severity of complications early on in the disease.
Medications
There are currently two different drugs that have been approved by the Food and Drug Administration for treating ALS. These include Riluzole (Rilutek) and Edaravone (Radicava). Riluzole is an oral medication that can help increase life expectancy by up to six months while Edaravone is an intravenous infusion that can help decrease the decline in daily functioning. There are side effects with both, which should be discussed with your doctor. Other medications can help reduce the severity of symptoms such as pain, sleep problems, muscle spasms, and more.
Physical Therapy
Physical therapy should be done to help address voluntary movements required for your independence. These exercises should be low impact to avoid further motor neuron deterioration and are best done under the supervision of a physical therapist.
Communication Support
To strengthen ongoing communication, treatments will include a speech therapist to help you adapt new techniques for talking when muscle deterioration begins to affect your vocals. This can also include integrating technology to help synthesize speech and communicate more efficiently.
Breathing Support
Breathing support and care should be performed to help slow symptoms and extend the time before you need to use a ventilator. This can include breathing exercises, ongoing tests and measurements of oxygen content, and trying various breathing devices.
The National Institute of Neurological Disorders and Stroke (NINDS) provides a substantial amount of federal funding for research surrounding different brain and nervous system afflictions. NINDS supports research that surrounds understanding the development, progression, and ongoing treatment options for those suffering from ALS. While there is currently no cure for ALS, NINDS and other supporting institutions hope to make progress in understanding how to reduce the damage ALS has on motor neurons.
If you or a loved one has been diagnosed with amyotrophic lateral sclerosis, it’s important to allow yourself time to learn how to cope and create a strong support system. ALS is a terminal condition, so it’s okay to grieve. It’s something that will affect your entire family, so try to focus on positive things in your life. Some people live for 10 years or more following a diagnosis but making the most out of that time is important. To help you adjust with physical changes and limitations, Byram Healthcare offers a range of urology supplies and support, catheters, incontinence care, and educational resources. Browse our products and if you have any questions, reach out to one of our representatives today.
