ALS

Amyotrophic Lateral Sclerosis (ALS) is a neurological disorder that can worsen over time, leading to paralysis. Initially, ALS is characterized by muscle twitching, muscle weakness, and speech problems.  

ALS or amyotrophic lateral sclerosis is a disease that became famous in 2014 through the ice bucket challenge, a challenge that involves pouring a bucket of cold water over your head. This challenge was created to raise funds for research on ALS or also known as Lou Gehrig’s disease.

Specifically, ALS attacks the brain and spinal cord that control muscle movement (motor nerves) so it is also called 
motor neuron disease. Over time, the nerves will be increasingly damaged. As a result, ALS sufferers lose muscle strength, the ability to speak, eat, and even breathe.

Causes of Amyotrophic Lateral Sclerosis

The cause of ALS is still unknown. However, about 5-10% of ALS cases are known to be hereditary.

Apart from hereditary factors, several studies have shown that ALS is thought to be linked to a number of conditions below:

• Glutamate excess Glutamate
  is a chemical that acts as a messenger to and from the brain and nerves. However, when it accumulates around nerve cells, glutamate can cause nerve damage.
• Immune system disorders
  In people with ALS, the immune system mistakenly attacks healthy nerve cells. As a result, these cells are damaged.
• Mitochondrial disorders
  Mitochondria are where energy is produced in cells. Disruptions in this energy production can damage nerve cells and accelerate the progression of ALS.
• Oxidative stress
  Excessive levels of free radicals will cause oxidative stress and cause damage to various body cells.

Risk factors for a myotrophic lateral sclerosis

There are a number of factors that can increase a person’s risk of developing ALS, namely:

• Aged between 40–70 years
• Having a parent with ALS
• Long-term exposure to lead chemicals
• Have a smoking habit

Symptoms of Amyotrophic Lateral Sclerosis

Early symptoms of ALS often begin in the legs and then spread to other parts of the body. As the disease progresses, symptoms worsen, nerve cells are damaged, and muscles continue to weaken.

The above conditions affect the sufferer’s ability to speak, chew, swallow and breathe.

Symptoms of ALS include:

• Muscle cramps or stiffness and twitching in the arms and tongue
• Arms feel weak and often drop things
• Weakened legs causing frequent falls or stumbling
• Difficulty holding head up and maintaining body position
• Difficulty walking and doing daily activities
• Speech disorders, such as slurred speech or speech that is too slow
• Difficulty swallowing, easy choking, and drooling from the mouth

Although it interferes with movement, ALS does not affect sensory function and the ability to control urination (BAK) or defecation (BAB). ALS sufferers can also still think well and interact with others.

When to see a doctor

See a doctor immediately if you feel changes in your arm and leg muscles, muscle cramps in your legs, and feel weak for several days or weeks. A doctor’s examination is also necessary if there are changes in the way you talk or walk.

ALS is a disease that will gradually worsen. If you are diagnosed with ALS, do regular check-ups with a neurologist, so that the development of this disease can be better monitored.

Diagnosis of Amyotrophic Lateral Sclerosis

To determine ALS, the doctor will ask in detail about the symptoms experienced by the patient and perform a physical examination. After that, to rule out the possibility of symptoms caused by other diseases, the doctor will perform the following examinations:

• Electromyography (EMG), to check the electrical activity of muscles
• MRI scan, to see the problem with the nervous system
• Blood and urine sample tests, to determine the patient’s general health condition and detect genetic abnormalities or other causative factors.
• Nerve conduction velocity test, to assess the function of the body’s motor nerves.
• Taking a tissue sample (biopsy) of muscle, to see abnormalities in the muscle.
• Lumbar puncture examination, to examine a sample of cerebrospinal fluid taken through the spine.

Amyotrophic Lateral Sclerosis Treatment

ALS treatment aims to slow the progression of the disease and prevent complications. Treatment methods that can be given include:

Drugs

To treat ALS, doctors can prescribe the following medications:

• Baclofen and diazepam, to relieve symptoms of muscle stiffness that interfere with daily activities.
• Trihexyphenidyl or amitriptyline, to help patients who have difficulty swallowing.
• Riluzole, to slow the progression of nerve damage that occurs in ALS.
• Edaravone , to protect brain cells and spinal cord from free radicals that can cause damage.

Therapy

Therapy for ALS is done to help muscle and respiratory function. Therapies that can be given are:

• Respiratory therapy, to help patients who have difficulty breathing due to weakened respiratory muscles.
• Physical therapy (physiotherapy), to help patients move, as well as maintain physical fitness, heart health, and muscle strength.
• Speech therapy, to help patients communicate better
• Occupational therapy, to help patients perform daily activities independently
• Regulation of nutritional intake, by providing food that is easy to swallow but still meets the patient’s nutritional needs.

ALS cannot be completely cured. However, the various treatments above can relieve symptoms and help patients perform daily activities.

Amyotrophic Lateral Sclerosis Complications

As ALS progresses, sufferers may experience a number of the following complications:

• Difficulty speaking
  The words spoken by ALS sufferers become slurred and difficult to understand.
• Difficulty breathing
  Difficulty breathing or hypoventilation can occur because the respiratory muscles begin to not work properly. This causes not enough oxygen to be taken in by the lungs. This condition can lead to respiratory failure, which is the leading cause of death in people with ALS.
• Difficulty eating
  This complication can cause ALS sufferers to become malnourished and dehydrated.
• Dementia 
  Dementia is a condition that causes a decline in memory and decision-making ability.

Prevention of Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis is difficult to prevent, because the cause is unknown. Get regular checkups, especially if you have a family member with ALS, or if you have impaired mobility.

Khosia, R. et al. (2021). Amyotrophic Lateral Sclerosis (ALS) Prediction Model Derived from Plasma and CSF Biomarkers. PLOS One, 16(2), pp. 1–8.
Mejzini, R. et al. (2019). ALS Genetics, Mechanisms, and Therapeutics: Where Are We Now? Frontiers in Neuroscience, 13(1310), pp. 1–27.
Kids Health (2017). For Kids. Lou Gehrig's Disease (ALS)
National Institute of Health (2022). National Institute of Neurological Disorders and Stroke. Amyotrophic Lateral Sclerosis (ALS) Fact Sheet.
National Institutes of Health (2021). MedlinePlus. Amyotrophic Lateral Sclerosis (ALS).
John Hopkins Medicine (2019). Neurology and Neurosurgery. ALS – Amyotrophic Lateral Sclerosis.
Mayo Clinic (2022). Diseases & Conditions. Amyotrophic Lateral Sclerosis (ALS).
Anderson, P. WebMD (2014). ALS Ice Bucket Challenge Blows Up Social Media.
Lights, V & Guthrie, C. Healthline (2022). ALS (Lou Gehrig's Disease).
Melinosky, C. WebMD (2021). What is ALS? What Are the Types and Causes?