Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis (ALS) is a group of progressive diseases that affect the nerve cells in the brain and spinal cord that control muscle movement.
As the disease progresses, people with ALS gradually lose the ability to use their muscles.
Many experts refer to ALS as a single disease. However, it is an umbrella term for a group of diseases.
Collectively, ALS is the most common type of motor neuron disease.
Sometimes experts call this disease Lou Gehrig’s disease.
The name refers to the name of a famous American baseball player who had this condition.
Symptoms of Amyotrophic Lateral Sclerosis (ALS)
Progressive muscle weakness occurs in all cases of ALS, but it may not be the first indication of the condition.
Because, in the early stages of this disease, signs and symptoms may be barely visible.
However, ALS symptoms usually begin to appear over time.
But the development of symptoms will vary for each sufferer. Here are some common symptoms of ALS, namely:
- Difficulty with daily activities, including walking.
- Increased “awkwardness”.
- Weakness in the legs, arms, feet, and ankles.
- The appearance of cramps and twitching in the arms, shoulders, or tongue.
- Difficulty maintaining good posture and holding the head up.
- People with ALS may experience emotional lability (sudden changes in emotions), such as suddenly laughing out loud or crying uncontrollably.
- Changes in thinking or memory.
- Slurred speech and difficulty with voice projection
- Feeling pain in certain areas of the body.
- Fatigue.
- In later stages, ALS can cause sufferers to have difficulty breathing and swallowing.
Early symptoms often include clumsiness, unusual limb fatigue, muscle cramps and twitches, and slurred speech.
A person experiences symptoms throughout the body as the disease progresses.
Some people with ALS have problems with decision-making and memory.
This condition eventually leads to a form of dementia called frontotemporal dementia.
Meanwhile, emotional lability can cause fluctuations in mood and emotional responses.
Causes of Amyotrophic Lateral Sclerosis (ALS)
To date, experts do not know the specific cause of ALS.
However, 5 to 10 percent of cases of this disease are related to hereditary factors.
Apart from hereditary factors, several studies show that ALS is also linked to the following conditions:
- Cell damage due to exposure to free radicals.
- imbalance in glutamate levels, a chemical that acts as a messenger to and from the brain and nerves.
- abnormalities in body proteins.
- There is inflammation in the body’s nerves.
Some studies suggest that smoking may also be a risk factor for ALS.
Research into other possible environmental triggers is ongoing.
Amyotrophic Lateral Sclerosis Risk Factors
There are a number of factors that can increase a person’s risk of developing ALS, namely:
- Gender. ALS is slightly more common in men than in women before age 65. This difference disappears after age 70.
- Age. ALS symptoms most often appear in the late 50s or early 60s but can develop at any age.
Diagnosis of Amyotrophic Lateral Sclerosis
A doctor may consider a diagnosis of ALS if a person has nerve and muscle problems that worsen over time.
A doctor will look for increasing problems with symptoms such as:
- Muscle weakness.
- Muscle atrophy.
- Twitching in certain areas of the body such as the hands.
- Cramps.
- The appearance of stiff tissue or contracture.
Unfortunately, some of these symptoms can also be symptoms of other neurological diseases. This can make ALS difficult to diagnose early.
Therefore, doctors need to carry out several supporting examinations to confirm the diagnosis, such as:
- Electromyogram (EMG). The doctor will insert needle electrodes through the skin into various muscles. This test is intended to evaluate the electrical activity of the muscles as they contract and when they are at rest. Muscle abnormalities seen on an EMG can help the doctor diagnose or rule out ALS.
- Nerve conduction studies. These studies measure the ability of nerves to send impulses to muscles in different areas of the body. This test can determine whether a person has nerve damage or certain muscle diseases.
- MRI. Using radio waves and a strong magnetic field, an MRI produces detailed images of the brain and spinal cord. An MRI can reveal spinal cord tumors, herniated disks in the neck or other conditions that may be causing symptoms.
- Blood and urine tests. Analyzing blood and urine samples can help your doctor rule out other possible causes of ALS signs and symptoms.
- Spinal tap (lumbar puncture). This test involves taking a sample of spinal fluid for laboratory testing using a small needle inserted between two vertebrae in the lower back.
- Muscle biopsy. If your doctor believes you may have a muscle disease other than ALS, he or she may order a muscle biopsy. This procedure involves taking a sample of muscle for your doctor to analyze in a lab.
- Genetic testing. In addition to the above tests, genetic testing may also be helpful for people with a family history of ALS.
Amyotrophic Lateral Sclerosis (ALS) Treatment
ALS treatment aims to slow the progression of the disease and prevent complications.
The following are treatment methods that ALS sufferers can undergo, including:
1. Use of Assistive Devices
Assistive devices such as braces, mattresses, and wheelchairs can reduce pain by supporting the body in a more comfortable position.
Some people with ALS may also need help meeting their body’s nutritional needs.
Therefore, sometimes people with ALS may use a feeding tube for enteral feeding.
When speaking becomes more difficult, communication tools provide another way to express thoughts and needs.
Options include communication boards and electronic assistive communication devices.
2. Use of Drugs
Doctors can prescribe two drugs, riluzole and edaravone, to ALS sufferers.
The drug riluzole appears to reduce a certain type of nerve damage called glutamate-induced excitotoxicity.
In addition, this drug can slow the progression of respiratory symptoms and prolong life by several months.
Meanwhile, the drug Edaravone is useful in reducing ALS symptoms by reducing oxidative stress.
This can slow the progression of ALS, especially for those in the early stages of the condition.
In addition, doctors may also recommend other medications to treat ALS symptoms, including:
- Mexiletine and baclofen, for muscle cramps and spasms.
- Nonsteroidal anti-inflammatory drugs (NSAIDs) and morphine, for pain management.
- Dextromethorphan/quinidine (Nuedexta), for emotional lability.
3. Non-medical treatment (therapy)
With a doctor’s recommendation, ALS sufferers can also undergo therapy to relieve symptoms and help with daily activities.
Here are some types of therapy that ALS sufferers can do:
- Physical therapy ( physiotherapy ). This therapy aims to help people with this disease move, and maintain physical fitness. In addition, physical therapy is also useful in maintaining heart health and muscle strength in ALS sufferers.
- Speech therapy procedures. Aim to help people with ALS communicate better.
- Respiratory therapy. This type of therapy is useful for helping ALS sufferers who have difficulty breathing due to weakened respiratory muscles.
- Regulation of nutritional intake. by providing food that is easy to swallow but still meets the nutritional needs of sufferers of this disease.
Amyotrophic Lateral Sclerosis Complications
ALS specifically affects the motor neurons of the brain and spinal cord.
Additionally, other body systems that depend on these neurons will be affected as the disease progresses.
As the ability to control muscles decreases, other body functions will also be affected.
Here are some complications that can occur:
1. Weakened Respiratory System
ALS causes the muscles that control breathing to weaken over time. Breathing tends to become more labored.
As the respiratory system weakens, the risk of pneumonia increases.
2. Decreased ability to speak
Muscles in the mouth, jaw, and throat tend to lose strength and mobility.
Because of this, ALS sufferers will have difficulty making themselves understood when speaking.
In severe cases, some people lose the ability to speak.
3. Difficulty Eating
ALS usually affects the sufferer’s mouth muscles that function to chew and swallow.
This condition ultimately makes sufferers of this disease have difficulty eating.
Based on these conditions, choking is a possible complication.
4. Weight Loss and Malnutrition
People with ALS may have difficulty eating. In addition, the disease can cause sufferers to burn calories faster.
As a result of both, people with ALS usually experience rapid weight loss and malnutrition.
5. Decreased Ability to Move
Standing and walking generally become more difficult over time. Some people will have difficulty moving their arms.
These changes will occur differently for each person.
But in general, more muscles will be affected, and the loss of function will become more severe as ALS progresses.
6. Dementia
In most cases, ALS can cause cognitive changes. These changes can affect language and executive function. As a result, dementia is one of the serious complications that can occur.
Prevention of Amyotrophic Lateral Sclerosis (ALS)
Until now, experts have not found a definite method to prevent ALS. Because until now the cause of ALS is still unclear. However, one can participate in regular health check-ups, especially if you have a family member with ALS, or you have impaired mobility. This aims to detect this disease early.
When to See a Doctor?
Immediately check your health condition if you experience symptoms of ALS. Remember that early treatment can minimize the risk of serious complications lurking.