Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disorder also known as Lou Gehrig’s disease. It affects nerve cells in the brain and spinal cord. Motor neurons responsible for controlling voluntary muscles are affected by this disease. The brain loses its ability to initiate and control muscle movements due to the death and degeneration of these neurons, leading to severe physical disabilities. Causes of Amyotrophic Lateral Sclerosis (ALS) include a combination of genetic and environmental factors, although the exact mechanisms remain unclear. Generally, ALS begins with muscle weakness or stiffness but can rapidly develop into more serious muscle atrophy, paralysis, and ultimately, respiratory failure.

Though the exact causes of ALS are still unknown, certain genetic and environmental factors are considered risk factors. There is no complete cure for ALS. The average life expectancy following diagnosis is about 2 to 5 years. However, some patients live much longer by managing symptoms, focusing on treatment, and maintaining a healthy quality of life.

Symptoms

ALS symptoms differ from person to person, both in terms of the initial signs and the rate of progression. Its symptoms start with localized muscle weakness or stiffness that spreads gradually throughout the body.

• Muscle Weakness: Muscle weakness causes loss of strength in the arms or legs. It makes daily tasks and movement difficult. This weakness gradually worsens with time and affects the overall functionality of the body.
• Speech and Swallowing Issues: Difficulty in swallowing food and liquids and speaking badly affect nutrition and communication. It makes eating difficult and increases the risks of swallowing. 
• Muscle Twitching and Cramping: Involuntary contractions of hands and feet interfere with fine motor skills and can cause discomfort.
• Posture Problems: Difficulty in maintaining posture or holding up the head affects balance and stability. It increases the risk of falls and causes poor posture.
• Emotional Lability: Due to disturbance in emotional regulation, uncontrolled laughter or crying without an obvious cause, affects social interactions and emotional responses.
• Fatigue and Atrophy: Continuous tiredness and muscle wasting reduce physical strength and endurance. It causes difficulty in performing everyday activities.
• Breathing Difficulties: Weakening of the respiratory muscles disturbs breathing. It needs mechanical assistance to support respiration.

When to See a Doctor

If you or someone you know is facing continuous muscle weakness, or difficulty in speaking, swallowing, or breathing, it’s crucial to consult with a healthcare provider as soon as possible. Early diagnosis and prompt treatment can manage symptoms and improve the quality of life. Although ALS is a serious and progressive disease, early detection of symptoms can result in better management strategies.

At Fitwell Hub we have experienced doctors who can provide specialized care and support for individuals with ALS. For more information or to schedule a consultation.

Causes Amyotrophic Lateral Sclerosis

Researchers still do not know the exact causes of ALS, but they consider a combination of environmental and genetic factors as potential causes.

• SOD1 Mutations: Any gene mutations in the SOD1 gene can cause faulty superoxide dismutase enzyme function. This mutation contributes to neurodegenerative diseases like ALS. It causes oxidative damage to motor neurons.
• Environmental Toxins: Toxic substances such as lead or pesticides increase the risk of developing neurological conditions by incorporating harmful substances. These substances disturb cellular functions or cause neuronal damage. 
• Trauma and Injuries: Any physical trauma or injury to the head or spinal cord can stimulate neurodegenerative changes or increase the progression of diseases like ALS. It causes direct damage to motor neurons.
• Oxidative Stress: An imbalance between free radicals and antioxidants causes oxidative stress. This stress leads to cellular damage and contributes to neuronal degeneration in conditions such as ALS.
• Mitochondrial Dysfunction: Disruptive mitochondrial function affects energy production. It causes neuronal damage and cell death, by the development of neurodegenerative diseases.
• Protein Aggregation: Toxic protein aggregation or misfolded proteins disturb cellular processes, causing neuronal damage and disease development. It causes neuronal damage and disease development.
• Immune Abnormalities: The dysfunction of the immune system causes inappropriate inflammation or immune responses. It damages motor neurons and increases neurodegenerative conditions.

You can also learn about the causes of Alzheimer’s by Clicking HERE.

Risk Factors

Risk factors are behaviors or conditions that increase the risk of a disease or other health issue. Age, genetics, gender, smoking, environmental exposures, and lifestyle choices, like military service, are examples of risk factors for ALS. By understanding these risk factors it becomes easy to determine the vulnerability of individuals and assist in early treatments or preventative measures. Some common risk factors are :

• Age: Doctors most commonly diagnose ALS between the ages of 40 & 70. The risk of developing ALS increases with age, especially when people enter their late 50s and early 60s. However, it can occur at any age.
• Gender: Generally, men are slightly more vulnerable to developing ALS than women. However, this difference decreases with age, and rates become more equal as people get older.
• Family History: The presence of a close family member with ALS increases a person’s risk of developing the disease, corresponding to a genetic or inherited factor.
• Smoking: Smoking is a significant risk factor, especially in women. In women, it leads to earlier onset and faster progression of the disease.
• Environmental Toxins: Continuous exposure to certain chemicals increases the risk of developing  ALS. However, researchers need to conduct further studies to confirm the exact relationship.
• Military Service: Certain research shows that veterans, particularly those who fought in war, are more vulnerable to developing ALS.

Complications

  ALS progression leads to various complications. These complications affect body functions.

• Respiratory Failure: Respiratory muscles weaken with time in ALS patients, causing difficulty in breathing. This decline generally results in respiratory failure, a leading cause of death, usually within 3 to 5 years of symptom beginning.
• Difficulty Swallowing: ALS affects the swallowing muscles, and causes malnutrition and dehydration. As a result, many patients require feeding tubes to maintain proper nutrition and hydration.
• Speech Difficulties: Patients with ALS frequently suffer from loss of speech clarity due to damage to the speech muscles. They have to rely on other methods of communication.
• Immobility: Progression of ALS increases muscle weakness and paralysis spread. Patients become dependent on others for their daily care and mobility assistance.
• Cognitive Changes: Though ALS mainly affects motor neurons, some patients also suffer cognitive and behavioral changes. Sometimes it causes frontotemporal dementia, which affects their personality and decision-making powers.

Prevention

For the prevention of ALS, there are no well-known preventive measures, by following certain lifestyle modifications the risk of developing ALS can be reduced. 

1- Avoid Exposure to Environmental Toxins

Certain chemicals like pesticides and heavy metals are poisonous substances. Limiting their exposure can reduce the risk of developing ALS. These chemicals cause nerve injury therefore limiting the exposure to these poisons can be an effective step in the prevention. 

2- Maintain a Healthy Lifestyle

Taking regular exercise, eating a balanced diet, and avoiding bad habits like smoking helps to improve overall health. By following these practices the risk of developing ALS is reduced. It improves nerve function and reduces inflammation.

3- Genetic Counselling

Genetic counseling is helpful for individuals who have a family history of ALS. It provides insight into the genetic factors linked to ALS, helps in determining individual risk, and guides how to plan a family and lead a healthy lifestyle.

At Fitwell Hub, we offer a Community Care Program aimed at improving your overall well-being and preventing diseases. For more information, visit Fitwell Hub’s Community Care Program.

Diagnosis

The Diagnosis of ALS becomes challenging due to its similarity to other neurological disorders. Early diagnosis depends on a combination of clinical assessments, patient history, and a series of tests to exclude other conditions.

Tests

The following tests are performed to identify ALS.

• Electromyography (EMG): It is used to measure the electrical activity of muscles. It confirms the diagnosis by identifying motor neuron abnormalities.
• Nerve conduction study: This study is used to evaluate the speed and strength of signals traveling along the nerves to identify nerve damage.
• Magnetic Resonance Imaging (MRI): MRI is used to exclude other conditions like tumors or spinal cord diseases.
• Blood and urine tests: These tests are used to exclude other condition’s symptoms that are similar to ALS, like infections, metabolic disorders, or vitamin deficiencies. By evaluating blood and urine samples, doctors can identify other possible causes of symptoms, and confirm an ALS diagnosis.
• Lumbar puncture (spinal tap): This test is used to evaluate the cerebrospinal fluid for the detection of abnormalities.

Fitwell Hub’s lab offers comprehensive diagnostic services, including these essential tests. For more details, visit FitwellHub Lab.

Treatment

Though there is no perfect cure for ALS, by following certain treatment options symptoms can be managed effectively and quality of life is improved.

1. Disease-Slowing Medications: Disease-slowing medications like Riluzole and Edaravone are used to slow the progression of ALS. These medications target mechanisms involved in motor neuron damage. These medications do not cure the disease but extend the survival rate and improve overall quality of life.
2. Physical Therapy: This therapy is used to maintain muscle strength, flexibility, and mobility. Personalized exercise plans are used to manage symptoms and improve physical function. They reduce the risk of contractures and maintain independence.
3. Speech Therapy: This therapy is used to assist individuals with communication difficulties. It develops strategies and tools to improve speech clarity and effectiveness. This therapy uses augmentative communication devices as the disease progresses.
4. Occupational Therapy: This therapy is used for individuals to adapt to daily life activities and maintain their independence. Therapists provide different strategies and tools for easier performance of tasks, like using assistive devices and modifying the home environment.

Medications

The following medications are used to treat ALS.

• Riluzole: It slows down the progression of disease and reduces damage to motor neurons. This extends survival rate and improves overall quality of life.
• Edaravone: It slows down the decline in daily functioning and neutralizes oxidative stress. It also protects motor neurons from further damage.
• Antispasmodic Drugs: These drugs reduce muscle spasms and cramps by providing relief from involuntary muscle contractions and improving comfort and mobility.
• NSAIDs: These are used to manage pain linked with ALS by reducing inflammation and relieving discomfort. It improves the overall quality of life.

Our Fitwell Hub pharmacy provides access to these medications and other important treatments. For more information.

Frequently Ask Question’s (FAQ’s)