Understanding ALS. What to Know About the Disease.

Amyotrophic Lateral Sclerosis (ALS) is a difficult diagnosis, often accompanied by fear and uncertainty. For patients and caregivers, understanding this complex condition is crucial for effectively managing its challenges.

What is ALS?

ALS is a progressive neurodegenerative disease that affects nerve cells in the brain and spinal cord, called neurons, which control voluntary muscle movements. As these neurons break down, the brain struggles to initiate or regulate movements, leading to muscle weakness and atrophy.

Normal Motor Neuron

Neurons in the spinal cord transmit signals from the brain to the muscles, enabling muscle contraction and movement.

Motor Neuron of ALS

In ALS, the affected neurons can no longer transmit signals from the brain to the muscles, leading to muscle atrophy.

ALS was discovered in 1869 by the French neurologist Jean-Martin Charcot. It became widely known in 1939 when it ended the career of one of baseball’s most beloved players, Lou Gehrig. For many years, ALS was commonly called “Lou Gehrig’s Disease.” Although rare,¹ ALS has a significant impact on those who have it, often causing disability and reducing lifespan.

The exact cause of ALS remains unknown, but research points to genetic and environmental factors. Grasping these mechanisms is vital for developing effective treatments and, hopefully, a cure.

Who Gets ALS?

About 30,000 people are affected by ALS in the United States, with roughly 5,000 new cases diagnosed each year. Most individuals who develop ALS are between 40 and 70 years old, with an average age of 55 at diagnosis. However, cases also occur in people in their twenties and thirties.²

ALS is 20% more common in men than women. As age increases, the incidence of ALS becomes more equal between men and women.³

About 90% of ALS cases occur without any known family history or genetic cause. The remaining 5–10% of ALS cases — known as familial ALS (FALS) — are inherited through a mutated gene with a known connection to the disease.³

Common Symptoms of ALS

Early ALS signs are often overlooked as they resemble less serious conditions. Recognizing these symptoms early enables quicker diagnosis and better disease management.

• Muscle Weakness: Muscle weakness, especially in arms and legs, is often the first sign of ALS, causing trouble lifting objects, climbing stairs, or doing daily tasks.
• Twitching and Cramping: Frequent muscle twitching and cramping are common in ALS patients. These involuntary movements can be uncomfortable and may worsen over time.
• Speech and Swallowing Difficulties: Speech and swallowing difficulties in ALS include slurred speech, trouble articulating, and swallowing issues as the disease progresses.

How is ALS Diagnosed?

Diagnosing ALS can be difficult due to its similarity to other neurological disorders. A comprehensive approach is required, involving several steps and tests.

• Medical History and Physical Examination: The diagnostic process begins with a detailed medical history and physical exam. Doctors check for muscle weakness, twitching, atrophy, and ask about family history and symptom development.
• Electromyography (EMG): EMG is a key diagnostic tool for ALS, measuring muscle electrical activity at rest and during contraction to detect abnormalities.
• Nerve Conduction Studies (NCS): NCS evaluates nerve signal speed and strength, helping distinguish ALS from other nerve conditions muscles.

What Are the Emerging Treatment Options for ALS?

Although there is no cure for ALS, treatments can manage symptoms, improve quality of life, and possibly slow progression.

• Medications: Riluzole and edaravone are the only FDA-approved medications for ALS. Riluzole can slow progression in some patients, while edaravone may reduce daily decline function.
• Physical Therapy: Physical therapy is key for maintaining muscle strength and flexibility. Customized exercises help patients stay mobile and lower risks like joint contractures, which tighten tissues, causing pain and limited movement.
• Assistive Devices: Assistive devices like wheelchairs, communication aids, and feeding tubes greatly enhance life quality for ALS patients. They help manage muscle loss and support independence.

ALS progression timing varies, but patients usually have better outcomes with multidisciplinary care. Impact on quality of life depends on age, body composition, and health. Early treatment, staying active, and a positive attitude are crucial. Family support is essential.

What is the life expectancy of ALS?

On average, life expectancy after an ALS diagnosis is three to five years. About 30% of people live five years or more, and 10% to 20% survive at least 10 years. These statistics can differ, and a neurologist can help estimate a person’s likely outcome.

Factors Influencing Prognosis

Multiple factors influence ALS progression, such as age of onset, symptom development rate, and initial location. Early intervention and support improve outcomes.

New Treatments on the Horizon

Research on ALS continues, with several promising treatments being studied. These new therapies provide hope for slowing disease progression and enhancing the lives of individuals affected by ALS.

• Gene Therapy: Gene therapy targets ALS-related genetic mutations by correcting or silencing them, potentially stopping or reversing disease progression.
• Stem Cell Therapy: Stem cell therapy transplants healthy stem cells to replace damaged neurons. Early trials show promise, and research continues to improve this method.
• Neuroprotective Agents: Researchers are investigating neuroprotective agents that can shield neurons from damage, potentially slowing ALS progression and maintaining muscle function.

Quality of Life

Despite challenges, many ALS patients stay positive and find ways to enjoy life. Support from family, friends, and healthcare professionals is crucial in managing emotional and physical effects disease.

Understanding ALS is the first step to managing its effects on patients and caregivers. Despite challenges, research progress offers hope. Staying informed and seeking support helps those affected face this journey with resilience determination.

Cooper and Inspira Neuroscience

If you or a loved one has ALS or is experiencing symptoms, Cooper and Inspira Neuroscience provides advanced diagnosis, treatment, and support.

Our experts use a team approach, working with you and your primary care physician to create a tailored treatment plan. Our team includes the following members:

• Neurologists
• Physical medicine and rehabilitation specialists
• Genetic counselors
• Behavioral medicine
• Psychology / Neuropsychology
• Orthopedic surgeons
• Social workers
• Pulmonologists
• Nutritionists
• Physical and Occupational Therapists
• Speech and Language Therapists

For an appointment with a Cooper and Inspira Neuroscience neuromuscular disease specialist, click here.

References

1. Woody, Kristina. February 2023. Is ALS Really that Rare? ALS Association. https://www.als.org/blog/is-als-really-that-rare[↩]
2. January 2025. Understanding ALS. ALS Association. https://www.als.org/understanding-als[↩]
3. January 2025. ALS Disease Statistics: Insights from Latest Research. Target ALS. https://www.targetals.org/2025/01/30/als-statistics/[↩][↩]