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Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder of the human motor system that can lead to muscle destruction. As ALS progresses, motor neurons in the primary motor cortex, cerebellum1, and spinal cord begin to degenerate.
The root cause of the rule is not yet known; However, researchers believe it is caused by various genetic mutations and environmental factors.
In this article, we will explore the 7 stages of ALS and the causes of the condition, diagnosis, and available treatment options.
1. Amyotrophic Lateral Sclerosis (ALS): What Is It All About?
Amyotrophic Lateral Sclerosis (ALS), also referred to as Lou Gehrig’s disease is a progressive neurological disease that causes the destruction of motor neurons in our body.
These motor neurons are responsible for muscle movement in our body, including talking, walking, chewing, and breathing.
ALS is not a common condition among people. A 2020 research study found that only 5.2 in every 1 lakh people were diagnosed with this condition.
ALS affects people at any stage in their lives, but the risk of ALS increases with age, and it is more likely to be diagnosed in middle-aged individuals.
2. Causes Of ALS
Though there is no definite set of factors that pinpoint what causes ALS, various research studies have found certain factors that make an individual more likely to get affected by the condition. Some of them include:
2.1. Genetic Factors
Genes can play a major role in a person’s affected by ALS. A 2017 research study confirmed that having a parent with Amyotrophic Lateral Sclerosis (ALS) increases their chances of getting affected by this condition.
About 5 to 10% per cent of individuals with ALS have this condition due to inherited genes from parents or other family members.
2.2. Environmental Factors
A 2017 research study identified that environmental factors were also responsible for this condition.
Some of the factors that make an individual more susceptible to this condition include:
- Smoking
- High levels of physical activity
- Earlier employment as military personnel
- Exposure to high levels of chemicals like pesticides and metals
- Exposure to electric shock
- Experiencing physical trauma that causes head injuries
2.3. No Specific Causes
When ALS occurs in an individual without any specific cause, it is known as Sporadic ALS. It is also one of the most common types of ALS that occurs in individuals.
About 90 to 95% of people ALS patients in the U.S. have Sporadic ALS.
3. Seven Stages Of Als
When a person is diagnosed with Amyotrophic Lateral Sclerosis (ALS), the motor neuron disease gradually progresses through the stages of ALS. Eventually, it takes away the individual’s ability to speak, write, walk and breathe, resulting in a shortened life span.
Some of the early symptoms of Amyotrophic Lateral Sclerosis (ALS) include:
- Weakness and stiffness in muscles
- Weakness in legs and ankles
- Tripping and falling
- Slurred speech.
All individuals affected by this condition may not go through the same symptoms as they vary from one person to another.
The average survival rate of ALS is around 3 years. Around 20% of people with this condition live five years, 10% of people survive ten years, and around 5% will live 20 years or even longer.
3.1. The Beginning Stage – Onset Of Symptoms
The early stages of ALS are indicated by the onset of symptoms:
- Muscle weakness
- Stiffness
- Tightness
- Cramping
These symptoms usually start when the individual is around 50 or 60 years of age.
The older person affected by ALS will slowly lose control, leading to poor balance, weak grip, and difficulty in talking.
These symptoms make it hard for them to perform their daily tasks like opening a bottle, buttoning clothes, and many other activities.
If the symptoms begin in the arms or legs, it is mostly referred to as ‘Limb onset ALS,’ but if the condition affects the speech first, it is called ‘Bulbar onset ALS.’
There is usually no pain in the earlier stages of ALS as they only gradually occur as the disease progresses.
In most cases, this stage happens even before the diagnosis is made. ALS also does not affect the bladder control of the individual or other senses.
3.2. Stage 2 – Diagnosis
Diagnosis is the second stage among the 7 stages of ALS. By this stage, the symptoms are evident, and the muscle weakness starts spreading gradually to other parts of the body.
To diagnose Amyotrophic Lateral Sclerosis (ALS), the doctor will usually look into the individual’s test results and previous medical history.
A 2018 research study emphasizes the difficulty of diagnosing ALS as most often, doctors and health experts rule out these conditions before actually officially diagnosing them with ALS.
Doctors usually make use of the following tests for ALS diagnosis:
- Blood tests
- Urine tests
- Electromyography tests
- Thyroid and parathyroid hormone level tests
- MRI scans
- Nerve biopsies
- Neurological check-ups
- Muscle biopsies
However, most individuals diagnosed with this condition retain high mental and reasoning abilities. They are very much aware of the loss of muscular function in their body as the stages of ALS progress.
3.3. Stage 3 – Involvement Of Second Region
The next stage includes the involvement of the second region. In this stage:
- Most of the voluntary muscle movement will be paralyzed.
- The muscles located in the lungs responsible for inhaling and exhaling will also be severely damaged and compromised.
- Some muscles get paralyzed while some other unused muscles become shortened permanently. These permanently shortened muscles are known as contractures, and they hinder joints like elbows from fully straightening.
- Muscle weakness can make eating and swallowing very hard, and there is a higher risk of choking.
- Some individuals with this condition suddenly burst into uncontrollable laughter or crying for no appropriate reason, a reaction known as the pseudobulbar effect.
3.4. Stage 4 – Involvement Of Third Region
The involvement of the third region is stage four. As the disease progresses, the mobility function of the individual becomes extremely limited. They may need a nurse or anyone else to take care of their personal needs.
In the middle stages, the patients may experience:
- Constant headaches
- Fatigue
- Vulnerability to pneumonia.
- There will be disorientation of speech in some cases, and it will become very hard to eat and drink through the mouth which will most often have to be done through the other available resources.
Respiratory inefficiency that occurs in the middle stages is one of the leading causes of death in this condition.
3.5. Stage 5 – Need For Gastrostomy
The fifth stage among the 7 stages of ALS calls for the need for gastrostomy as all the individual muscles are paralyzed, which includes even the mouth and throat muscles.
Dysphagia becomes a major problem for people with this condition, making it very hard for them to intake nutrition and other essential fluids orally.
People suffering from severe dysphagia have problems related to weight loss, swallowing, and breathing difficulties.
Gastrostomy feeding is the usually recommended method to provide adequate nutritional support for people experiencing severe dysphagia.
Gastrostomy can also, to a great extent, improve the survival rate, quality of life, and nutritional outcome of individuals affected by ALS. This treatment method also ensures proper hydration of the patient.
3.6. Stage 6 – End Stage
The sixth stage of the ALS is usually known as the end stage. Now that most voluntary muscles are paralyzed, even those involved in breathing become weaker.
The body becomes unable to bring in enough air for breathing. Therefore, breathing is now provided via ventilators in the final stages.
3.7. Stage 7 – Death
The last and final stage of the 7 stages of ALS 2is death. The patient eventually dies due to lack to lack of air and the inability to use lung muscles.
Respiratory failure is the major cause of individuals diagnosed with ALS, followed by pneumonia, cardiovascular problems, and pneumonia.
4. Treatment For ALS Patients
Though there is no known cure for ALS, there are many available treatment options that help bring relief to the symptoms caused by the condition.
Some of them include:
4.1. Physical Therapy
Physical therapy 3is an amazing way to toughen the muscles that are unaffected by ALS. This helps them to strengthen it further and to remain independent.
Individuals with ALS can indulge in low-impact exercises like swimming, walking, and stationary bicycling. Also, exercises keep our minds occupied from any negative thoughts and significantly enhance our mental health.
They also help in dealing with the fatigue caused by this condition.
4.2. Medication
According to the National Institute of Neurological Disorders and Stroke, medicines like riluzole or edaravone are prescribed by doctors to people who have ALS to reduce the damage and slow down the 7 stages.
4.3. Mental Therapy
Any person who is diagnosed with ALS experiences severe symptoms that can cause unbearable pain. During these tough times, many ALS patients may face many challenges regarding their mental health.
It is advisable to consult with a doctor or health expert to seek therapy or any other alternative treatments to help people going through this condition.
5. In the End
Amyotrophic Lateral Sclerosis (ALS) is a fatal condition, and unfortunately, there is no known cure for this progressive disease to date. Almost all cases of ALS are deadly. However, proper treatment options, guidance, and mental health assistance help people with ALS manage their lives.
Any individual experiencing ALS symptoms should seek the assistance of a doctor or a healthcare professional to develop an effective plan to help them during the 7 stages of ALS.
6. Frequently Asked Questions
1. What Are the Different Stages of ALS?
The different stages of ALS are: Early, Middle and Late.
2. How Long Do Stages of Als Last?
Most patients progress to the end stages of ALS within two to five years from diagnosis and the disease is eventually terminal.
3. What Is the Best Treatment for Als?
Although there is no known cure for ALS, the drug riluzole has been approved for treatment and may slow the progression of the disease. It is expensive, however, and appears modestly effective. Generally, treatment is designed to help control symptoms. Drugs such as baclofen or diazepam may help control spasticity.
- Van Overwalle, Frank, et al. “Consensus paper: cerebellum and social cognition.” The Cerebellum 19 (2020): 833-868. ↩︎
- Masrori, Pegah, and Philip Van Damme. “Amyotrophic lateral sclerosis: a clinical review.” European journal of neurology 27.10 (2020): 1918-1929. ↩︎
- Zhang, Qing, et al. “Signaling pathways and targeted therapy for myocardial infarction.” Signal transduction and targeted therapy 7.1 (2022): 78. ↩︎
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