The 7 Stages of ALS: What You Need to Know

Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s disease, is a progressive neurological condition that kills motor neurons in the body that are responsible for essential muscle movements, such as talking, walking, breathing, drinking, and eating. This results in a partial or total loss of muscle control. In this article, we will discuss the 7 stages of ALS so that you can be better informed about this devastating disease.

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Risk Factors for ALS / Who Can Get ALS?

ALS impacts individuals of all ethnic backgrounds and races, with Caucasians and non-Hispanics being most likely to develop the condition. While the onset of ALS can occur at any age, symptoms most typically appear between the ages of 55 and 75, with the risk of ALS increasing with age. Overall, people assigned male at birth have a moderately greater likelihood to have ALS than people assigned female at birth, with this gap often closing as age increases. The majority of ALS cases are not hereditary, with only around 5-10% being genetic. The offspring of those with familial ALS having a fifty-percent chance of developing the condition. 

Symptoms of ALS

Considered to be a rare ailment, ALS often begins with muscle weakness that spreads throughout the body, worsening over time. The symptoms of ALS may vary on a case-by-case, depending on which motor neurons are impacted. Unlike some other neurological conditions, those with ALS are aware of the impact of the disease on their bodies, as they don’t lose their reasoning and mental abilities. Some common symptoms of ALS include:

• Muscle twitching, particularly in the tongue, shoulders, or arms
• Cognitive or behavioral changes
• Dysphagia (difficulty swallowing)
• Muscle cramps
• Muscle weakness in the hands, feet, legs, or ankles
• Incoherent speech or stuttering
• Frequent falls
• Difficulty with or inability to perform daily tasks
• Misplaced or inappropriate laughing, crying, or yawning

7 Stages of ALS

ALS patients gradually progress through seven stages of the disease, as their motor neurons increasingly deteriorate. Throughout these stages, a person’s ability to walk, write, move, speak, and breathe decreases. The experience of each stage is dependent upon the individual.

1. Early stage of ALS

The early stage of ALS often occurs before doctors provide a diagnosis. Muscular symptoms may only occur in a single area of the body, with any of the area able to be affected, including the muscles controlling respiration and swallowing. These early signs may occur as young as 50 or 60 years of age, with older adults impacted by ALS gradually experiencing poor balance, a weakened grip, and difficulty with regular communication. These difficulties may begin to impact their ability to carry out their usual activities of daily living. If ALS symptoms begin in the legs or arms, it’s considered to be ‘limb onset ALS’, while ALS that initially impacts the speech is referred to as ‘bulbar onset ALS’. 

Specific symptoms often found in the early stage of ALS include:

• Muscle weakness, tightness, cramping, and twitching
• Weakness when gripping
• Difficulty with balance
• Falling or tripping
• Fatigue

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2. Diagnosis stage of ALS

The second stage of ALS is referred to as the Diagnosis Stage, wherein the signs of ALS are notable as weakness spreads to various areas throughout the body and ALS is diagnosed. There is not a single test used in the diagnosis of ALS. Rather, doctors base the diagnosis of ALS on physical examination, medical history, and symptom history. While ALS is often apparent once a person reaches the diagnosis stage, ALS can sometimes mimic other neurological conditions, making diagnosis sometimes difficult. As such, doctors typically perform thorough testing to verify an ALS diagnosis and rule out other diagnoses. These tests may include:

• MRI scan
• Urine test
• Blood test
• Nerve conduction study
• Electromyography (EMG)
• Muscle biopsy
• Thyroid and parathyroid hormone panel tests
• Neurological evaluations

3. Middle Stage of ALS: Involvement of The Second Region

During the middle stage of ALS involving the second region, the majority of the voluntary muscles are paralyzed, including the lungs. This typically leads the lungs to become significantly damaged, resulting in difficulty breathing. The muscular paralysis and weakness during the second region middle stage of ALS can also cause difficulties with swallowing and eating, leading to increased risk of choking. Additionally, muscles become permanently shorter during this stage, otherwise known as contracture. Contracture impacts joints, preventing them from working properly, such as allowing knees and elbows to completely straighten. Some individuals with bulbar onset ALS may also experience what’s referred to as the pseudobulbar affect (PBA) during this stage. PBA may cause individuals to inappropriately cry or laugh with no real explanation.

4. Middle Stage of ALS: Involvement of third region

The fourth stage of ALS is the involvement of the third region. In this stage, ALS symptoms increase in severity and become more widespread. The mobility of individuals in this stage becomes highly restricted, often leading them to require assistance for activities of daily living, such as toileting and bathing. Breathing issues and the risk for respiratory failure, as well as challenges with eating and drinking also generally become more apparent during this stage. Some individuals may also begin to experience pseudobulbar palsy during this middle stage. Other symptoms often noted in those in this stage include:

• Fatigue
• Chronic headaches
• Speech disorientation
• Increased susceptibility to pneumonia

5. Mandatory gastrostomy stage of ALS

Individuals in the fifth stage of ALS typically experience an increase in dysphagia, or the paralysis of their mouth and throat muscles, leading to increased swallowing and breathing difficulties. This makes it extremely difficult for them to gain nourishment orally, and often results in significant weight loss. As such, doctors generally recommend a gastrostomy (G-tube) procedure during this stage. This is a surgical procedure in which a feeding tube is placed through the abdomen and into the stomach. It can improve the patient survival rate and quality of life, as it delivers needed hydration and nutrients.

6. Late stage ALS

The sixth stage of ALS is commonly referred to as the late stage or end stage. In this stage, muscle paralysis impacts the majority of a patient’s voluntary muscles. This results in extremely limited mobility, and assistance is required for even the smallest personal needs. The muscle groups responsible for breathing become compromised, and the body is unable to get enough oxygen, and ventilators are introduced to assist in breathing. Difficulties with breathing and muscle paralysis during this stage can result in:

• Headaches
• Fatigue
• Unclear thinking
• A loss of speech

7. Death Stage of ALS

In the final or death stage of ALS, people generally require a ventilator for breathing as the muscles in the diaphragm responsible for breathing weaken and breathing becomes increasingly difficult. A ventilator may be relied upon for longer periods of time as these muscles weaken. The majority of ALS deaths are the result of respiratory failure that slowly progresses over months. Respiratory failure leads to an accumulation of carbon dioxide in the body, resulting in lessened consciousness and patients sleeping up to 22 hours per day. During this time, fear, anxiety, and discomfort may be present, and comfort measures can be addressed by a doctor. Other, less common, causes of death in ALS include:

• Pulmonary embolism, a blockage in an artery in the lungs
• Pneumonia, resulting in aspiration or the presence of food or fluid in the lungs
• Cardiac arrhythmia, irregular heartbeat pacing
• Malnutrition, due to issues with swallowing

Life Expectancy With ALS

There is currently no known cure for ALS, with each case resulting in eventual death. The majority of those diagnosed with ALS live for 2-5 years following diagnosis. Very rarely, those with ALS can live for decades following diagnosis.

Treatment for ALS

While ALS has no cure, treatments exist for symptom management. These treatments are aimed at providing comfort, preventing unnecessary complications, and improving the quality of life for those with ALS. Some treatments may also aid in prolonging life for ALS patients. As part of a treatment plan, doctors may offer patients prescription medications to reduce the symptoms of ALS and control muscle cramping, spasms, and pain. While a few medications exist to slow the progression of ALS symptoms, their efficacy remains unclear. Other forms of treatment in ALS may include:

• Occupational and physical therapy
• Nutritional support, such as feeding tubes and assistive eating tools (dysphagia cups, specialized utensils and plates, etc.)
• Supportive braces and other devices
• Breathing support, such as ventilators
• Assistive communication support, such as AAC devices

In the final stages of ALS, hospice care is recommended as a supportive treatment to provide comfort and quality of life measures. In addition to physical comfort, hospice care also provides individuals and their loved ones with social, emotional, and spiritual support.

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ALS is a devastating, progressive disease responsible for the deterioration of motor functions in the body. If you or your loved one think you may be showing signs of ALS, it’s important to consult with your doctor to receive the proper diagnostic support.