Everything about Amyotrophic Lateral Sclerosis (ALS) – Causes, Symptom, and Treatment

Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s disease, is a progressive nerve disorder that causes muscles to lose control. It occurs when the nerve cells present in the brain and spinal cord start to degenerate.

ALS attacks motor neurons used in performing voluntary muscle activities. So as the disease progresses, these nerve cells begin to break down until there is no control over voluntary movements of respective body parts. Read on to learn about its type, symptoms, causes, and more!

There are two main types of Amyotrophic Lateral Sclerosis (ALS):

1. Familial ALS or FALS: It is a type that runs in the family and generally develops due to changes in a gene’s structure. Only 5% to 10% of all ALS cases include this disease type where the faulty gene is passed down by heredity. So, if a parent has a faulty gene, there is 50% likelihood that it will be transmitted to the children. 
2. Sporadic ALS: Sporadic ALS is the common type that affects around 95% of people with this disease. Moreover, sometimes it can even develop without a clear cause. Other types that physicians have classified according to the different symptoms patients suffer are:
3. Classical ALS: This type is categorised when a patient’s upper and lower motor neurons deteriorate.
4. Primary Lateral Sclerosis (PLS): In this progressive disease type, only the patients' upper motor neurons deteriorate. Moreover, there is usually no issue with the lower motor neuron for at least two years.
5. Progressive Bulbar Palsy (PBP): It is a case of Progressive Bulbar Palsy if the condition starts with difficulty in chewing, speaking, swallowing or other due to deterioration of lower motor neurons.
6. Progressive Muscular Atrophy (PMA): It is a PMA if only the lower motor neurons of patients deteriorate. Also, the upper motor neurons, in this case, remain unaffected for at least two years.

The signs and symptoms of Amyotrophic Lateral Sclerosis vary from one person to another, i.e., depending on the neurons affected. The common symptoms include:

• Weakening of leg, ankle and feet
• Falling and tripping
• Difficulty in walking and performing daily activity
• Muscle cramps and twitching of arms
• Trouble swallowing or slurred speech
• Change in cognitive movement and behaviour
• Inappropriate yawning, laughing or crying

The causes of Amyotrophic Lateral Sclerosis are as below:

• Transfer of faulty genes (if it is Familial ALS)
• Protein abnormalities, such as misfolding
• Nerve inflammation
• Incorrect balance of the chemical messenger glutamate

The risk factors for Amyotrophic Lateral Sclerosis are as below:

Demographic Factors

• Gender: According to studies, men are more likely to experience this disease than women. However, as one ages, the difference between gender risks decreases.
• Age: The likelihood of getting diagnosed with the disease increases with age. Generally, the symptoms of Amyotrophic Lateral Sclerosis develop between the age of 55 and 75. However, it can also occur early too.
• Race: Non-Hispanics and Caucasians have higher chances of developing ALS disease.

Other Factors

• Smoking: Smoking increases the risk of one developing ALS disease.
• Military Service: Studies show people who have served military are at a higher risk of developing ALS. The reason behind it is still unknown. However, it can be because of exposure to chemicals, metal, traumatic injuries, etc.
• Environmental Toxin Exposure: Some evidence has even linked exposure to lead and similar substances to ALS. However, no single chemical or agent has been associated with ALS.

Doctors use no definitive diagnosis process to cure amyotrophic lateral sclerosis. Instead, a series of muscle and imaging tests take place along with an analysis of detailed medical history. Also, the neurologists may ask you about different symptoms you may be experiencing. The tests are as follows:

1. Nerve Conduction Study: It measures the electrical activity of the muscles and nerves by analysing your nerve’s ability to send signals to another nerve or a muscle.
2. Electromyography (EMG): EMG involves a recording technique to detect the electrical activity of muscle fibres. This, in turn, help diagnose the disorder.
3. Magnetic Resonance Imaging (MRI): In this non-invasive process, doctors use magnetic fields and radio waves to bring out detailed images of your brain and spinal cord.
4. Muscle Biopsy: This test is carried out when the doctor suspects you have a muscle disease other than ALS. During the process, you will receive local anaesthesia. The physician then will remove a sample of your muscle by making a small cut and send it to the lab for examination.
5. Blood and Urine Tests: Doctors may also suggest blood and urine tests depending on your symptoms, findings, and test results. It helps in ruling out the possibilities of other diseases.

When discussing the treatment of Amyotrophic Lateral Sclerosis, it does not cure the disease but slows its progression. Also, these treatment methods aim to alleviate symptoms and prevent complications. These include:

• Physical Therapy: It reduces discomfort from cramps, stiff muscles and fluid retention. As a result, you can stay mobile.
• Speech Therapy: Doctors suggest these strategies for communication and safer swallowing training. So, you can maintain verbal communication for a longer time. In addition, you may also learn how to communicate nonverbally.
• Nutritional Counselling: It ensures you eat a balanced diet and live healthily. The nutritionist will also help you suggest a food alternative if an item is difficult to swallow.
• Special Equipment: You may get the assistance of special equipment like a wheelchair and electric beds to function independently.
• Assistive Devices: These include grab bars, braces, splints, and reach devices to help you work on your own. It makes eating, bathing and getting dressed easy.
• Medications: Doctors generally suggest drugs to help relieve muscle cramps, control extra saliva or other symptoms. The two common types are Edaravone and Riluzole. The first slows the rate of decline in the functioning of the body, as associated with ALS. The second reduces any further damage to motor neurons.

This, in turn, extends the rate of survival for a few more months. However, it cannot reverse the damage already done.

Half of the patients live for 3 or more years after getting diagnosed with ALS. On the other hand, 20% of them live for 5 or more years, and 10% of the people live for 10 or more years. However, only 5% of the patients live for 20 or more years, i.e., with the condition.

Here are a few strategies that can help in the better management of Amyotrophic Lateral Sclerosis:

1. Reach Out: You can connect to online support groups or other ALS patients in person to ask questions or provide insights through shared experiences.
2. Keep in Touch: It is common to lose social contact. So, make sure you keep in touch with friends and family. Also, keep up with social activities as much as possible.
3. Plan Ahead: There are certain limitations for ALS patients to do particular things. So, foreseeing things and planning ahead make it easier to perform.
4. Get Equipped: Prepare a bag containing hand wipes, tissue, and easy-to-hold cutlery to carry everywhere. Put up a disability sign in the car. Make important adjustments at home, like purchasing a device that lifts the toilet seat.
5. Hire a Caregiver: You may need the assistance of caregivers to make it easy to perform your day-to-day activities. Moreover, they are generally medically experienced, so they can look after your diet, medicine and things to avoid.
6. Arrange for Your Caregiver’s Time-off: Your caregiver may require a few days off to care for themselves and their family. So, for the days they do not come, arrange for a relative, friend, or another caregiver. It is generally for the weekend or other holidays.